Natural Healing for Low Platelet Count

Conquer Low Platelets

Alternative And Natural Therapies For Itp (idiopathic Thrombocytopenia Purpura). Live Free From Itp. Complete Program To Increase Platelets. This Is What You Will Learn With this Guide: The Two Herbs That can help bring up your platelets. The Two Vitamins needed to keep those platelets from dropping. What foods may cause your platelets to drop. How science has confirmed the benefits of these herbs in their use with low platelets. Why your doctor may not know about these natural alternatives and how you can assist him in helping you. Different tests that naturopathic doctors do to determine your real state of health that may reverse the course of your body drastically. Understand some of the reasons why people develop low platelets. Discover how your digestive tract may be the culprit to your low platelet level problems. How you can prevent the most drastic step a splenectomy. How you can restore your health so that you dont need any more dangerous drugs. Get your life back and stop ending up in the hospital all the time. Learn why your immune system is attacking your platelets and how to calm it down. Learn what over the counter medications to stay away from if you have low platelets Continue reading...

Conquer Low Platelets Overview


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Thrombopenia Absent Radii


This infant has the thrombocytopenia-absent radius (TAR) syndrome. There is absence of the radius bilaterally. Note that the absence of the radius of the right forearm has resulted in a club hand. In the TAR syndrome the thumb is always present. Figure 1.40. This infant has the thrombocytopenia-absent radius (TAR) syndrome. There is absence of the radius bilaterally. Note that the absence of the radius of the right forearm has resulted in a club hand. In the TAR syndrome the thumb is always present. Figure 1.42. This infant has Fanconi's syndrome. Note the congenital absence of the right radius and right thumb. In Fanconi's syndrome the thumb may occasionally be present. Note the club hand with absence of the radius and the thumb. This may be unilateral or bilateral. In Fanconi's syndrome there is pancytopenia (anemia, neutropenia, and thrombocytopenia) in addition to the hypoplastic or absent thumbs and hypoplastic or absent radius. Figure 1.44. Another example of...

Preface to the First Edition

Thyroiditis, pemphigus vulgaris and bullous pemphigoid. Noteworthy, some of these disorders are exclusively mediated by circulating autoantibodies such as the hemolytic anemias, thrombocytopenia, pemphigus, and pemphigoid while others, such as allergic autoimmune encephalomyelitis and autoimmune thyroiditis require the transfer of immunocompetent cells in addition to auto-antibodies.

Bayesian Methods for Cancer Phase I Clinical Trials

Where the value chosen for the target probability 0 would depend on the nature of the dose limiting toxicity it would be set relatively high when the DLT is a transient, correctable, or nonfatal condition, and low when it is lethal or life threatening (O'Quigley et al., 1990). Participants in cancer phase I trials are usually late stage patients for whom most or all alternative therapies have failed. For such patients, toxicity may be severe before it is considered an intolerable burden (Whitehead, 1997). Thus, in cancer phase I trials, dose limiting toxicity is often severe or potentially life threatening and the target probability of toxic response is correspondingly low, generally less than or equal to 1 3. As an example, in a phase I trial evaluating 5-fluorouracil (5-FU) in combination with leucovorin and topotecan (see Sec. 1.4.1), dose limiting toxicity was defined as any treatment attributable occurrence of (1) a nonhematologic toxicity (e.g., neurotoxicity) whose severity...

Acute Fatty Liver of Pregnancy

Serum aminotransferases are generally less than 1,000 IU L and do not reflect severity of liver dysfunction. Hyperbilirubinemia averages 10 to 15 mg dL, but levels up to 30 to 40 mg dL have been reported. In the setting of eclampsia and preeclampsia, hyperbilirubinemia is predominantly unconjugated and hemolysis is present. Increases in alkaline phosphatase are difficult to interpret because they overlap the normal values seen late in pregnancy. A left-shifted leukocytosis and some degree of thrombocytopenia are nearly universal. Clinical and laboratory findings suggest the diagnosis of AFLP. The differential diagnosis includes acute viral hepatitis, acute toxic or drug-induced hepatitis, preeclampsia-related liver disease (including hemolysis, elevated liver enzymes, and low platelets syndrome HELLP ), and biliary tract disorders. Imaging studies are useful in assessing the biliary tree. Virologic markers and history can help to rule out viral and toxic hepatitis. Upper...

Metabolic Miscellaneous Etiologies

Included in this group are FHF secondary to Wilson's disease (see Chapter 124, Management of Wilson's Disease), autoimmune hepatitis, Reye's syndrome, and pregnancy-related ALF from acute fatty liver of pregnancy, hemolysis, abnormal liver enzymes and low platelets(HELLP) syndrome, or hepatic rupture (see Chapter 133, Biliary Strictures and Neoplasms). FHF may be the first clinical manifestation of Wilson's disease. Indeed, Wilson's disease should be considered in any young patient with unexplained FHF, particularly when

HIV and haematological problems

Normochromic, normocytic anaemia of unknown origin are often seen but usually have no adverse effect on HIV-infected individuals. Severe anaemia or neutropenia should be investigated for other underlying causes. Thrombocytopenia is common in HIV disease and, only if persistent, causing bleeding and less than 20 X 109 litre warrants treatment with antiretrovirals which is usually effective. Many therapies used to treat HIV may be toxic to bone marrow.

Diagnosis and Initial Management

In all patients with lower GI bleeding, fluid resuscitation and correction of coagulopathy or thrombocytopenia take precedence over diagnostic or therapeutic procedures. Nasogastric (NG) lavage is performed looking for fresh blood, clots, or coffee grounds suggestive of an upper GI source. The absence of blood on NG lavage does not exclude upper GI bleeding, unless bile is obtained. Evaluation of the anorectum should include digital rectal exam and anoscopy. The latter will allow identification of internal hemorrhoids, fissures, or fistulas. After this point, the decision between further diagnostic studies will depend on the severity of bleeding, overall condition of the patient and comorbid diseases, and the availability of each diagnostic tool in a specific hospital. Available options include colonoscopy, tagged red blood cell (RBC) scan, and

Problemsspecial considerations Preexisting disease

In terms of general anaesthetic management, the problems of pre-existing renal disease are the same as in the non-pregnant population. These include the underlying cause of renal impairment, systemic manifestations of renal failure (in particular, hypertension and ischaemic heart disease, thrombocytopenia and anaemia), the patient's medication, altered handling of drugs and fluid management, including the nature and timing of dialysis.

Clinical Trials Testing ASODNs in Hematology and Oncology

The number of ongoing clinical antisense trials represents the growing interest in this technology (Table 2) (2). Generally, systemic AS-ODN treatment is well tolerated and side effects are dose dependent. Dose-limiting tox-icities are thrombocytopenia, hypotension, fever, and asthenia (9,11). Furthermore, elevation of the liver enzymes aspartate aminotransferase and alanine aminotransferase, as well as complement activation and a prolonged activated partial thromboplastin time (aPTT) have been reported (12).

Radiologic Intervention

Because of the shortage of donors and the risks of tumor progression while patients who are not candidates for traditional surgical resection are waiting for OLT, several strategies have been developed in an attempt to achieve local control. These methods include percutaneous ethanol injection, transcatheter arterial chemoembolization (TACE), and radiofrequency ablation (RFA). Although the majority of studies have not shown an improved survival with the use of TACE as primary therapy for HCC, most studies do report significant response rates. At M. D. Anderson Cancer Center, TACE is used selectively. Exclusion criteria include advanced liver disease (Child-Pugh class C), active gastrointestinal bleeding, encephalopathy, refractory ascites, main portal vein thrombosis, extrahepatic spread, hepato-fugal flow, renal failure, and severe thrombocytopenia. In well-selected patients, chemoembolization has been shown to improve survival in two recent randomized controlled studies (Llovet et...

Management of Adverse Events

Hematologic abnormalities are frequently observed and led to dose reduction of peginterferon and ribavirin in a number of subjects in the pivotal randomized controlled trials. Ribavirin is associated with a dose-dependent, extravascular hemolytic anemia in more than 90 of treated patients. Anemia is associated with fatigue, exercise intolerance, and depression. My practice has been to assess symptoms carefully and consider ribavirin dose modifications and or recombinant human erythropoetin (40,000 subcutaneously weekly). On the other hand, interferons, especially peginterferons, cause a significant decrease in peripheral white blood cell count and platelet count due to bone marrow suppression. Most clinicians often tolerate absolute neutrophil counts (ANC) as low as 500 to 750 mm3. However, once the ANC drops below 500 mm3, I will often consider interferon dose reduction and or initiation of filgrastim (300 mcg subcutaneously once or twice weekly). Finally, the role of oprelvekin...

Preoperative abnormalities

Haematological abnormalities include haemolytic anaemia, nocturnal haemoglobinuria, variable but mild jaundice, haemosiderin in the urine leading to iron deficiency, leucopenia, thrombocytopenia, and reticulocytosis. Occasionally, acute renal failure occurs secondary to massive haemolysis. Aplastic anaemia sometimes develops. 9. A poor survival is associated with thrombosis, pancytopenia, myelodysplastic or leukaemic syndromes, thrombocytopenia, or age above 50 years at diagnosis (Socie et al 1996).

Idiopathic Portal Hypertension

As mentioned above, IPH is a diagnosis of exclusion. In the presence of clear signs of portal hypertension and when clinical history, physical examination, imaging and laboratory studies, and biopsy rule out other causes, only then one can diagnose IPH. Biopsy invariably shows concentric fibrosis around the small portal vein branches without cirrhosis. Some authors report preferential involvement of the left lobe with associated right lobe atrophy. Liver-associated laboratory tests are normal or near normal and serve only to exclude other causes of portal hypertension. Anemia and thrombocytopenia are also common findings in IPH, the latter likely due to the significant splenomegaly that characterizes the disease. It is crucial to properly diagnose IPH from a prognostic and management point of view. With proper management of variceal bleeding and splenomegaly and its associated thrombocytopenia, life expectancy is no different from healthy individuals. Its course is stable over many...

Combined B and TCell or Primary TCell Deficiencies

Ataxia telangiectasia (AT) is an autosomal recessive disorder usually presenting between ages 2 and 5 years with ataxia and telangiectasias of the nose, conjunctiva, ears, or shoulders. These patients have T-cell defects secondary to thymic hypoplasia, and IgA deficiency occurs in 50 of patients. A defect in the ATM gene, a protein kinase involved in cell cycle control and DNA repair, is the culprit in this disorder and also leads to the increased risk of malignancy (IUIS Committee, 1999). DiGeorge Syndrome is the result of a congenital defect in migration of the third and fourth branchial arches, leading to thymic hypoplasia and other developmental abnormalities. The severity of the T-cell defect corresponds to degree of thymic aplasia, and those patients with severe T-cell defects are susceptible to opportunistic infections. Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder resulting from a defect in the WAS protein, which is involved in intracellular signaling and...

Prevention of a First Variceal Hemorrhage

Patients with cirrhosis should be endoscopically screened for varices so that appropriate therapy can be initiated if varices are present. Because the prevalence of varices increases with the severity of liver disease, screening is based on Child's classification at the time of the diagnosis of cirrhosis. Patients with Child's Class A cirrhosis should be screened when there is evidence of portal hypertension, such as thrombocytopenia (platelets less than 140,000 per mm3), an enlarged portal vein (diameter > 13 mm), or evidence of collateral circulation on ultrasound. Screening should occur in anyone with Child's Class B or C cirrhosis at the time of the diagnosis of cirrhosis. If the patient is free from varices at initial screening, follow up endoscopy should be carried out. The timing of follow up will vary with the etiology of the patient's liver disease. Patients with ALD require more frequent screening (every 1 to 2 years), whereas those with cirrhosis due to hepatitis C only...

Management options

The obstetric anaesthetist is often called upon to make a decision regarding the advisability of regional analgesia and anaesthesia in these cases. Several textbooks and articles offer guidance on this subject, and the general trend in recent years has been to lower the 'cut-off point' from a platelet count of 100 x 109 1 to 75 x 109 l. In fact, there is no evidence to support this sort of 'all-or-nothing' approach, and every case must therefore be considered on its merits, taking into account the underlying pathology, with the risk of the procedure (epidural spinal haematoma) balanced against the benefits (pain relief, better blood pressure control, avoidance of general anaesthesia). When monitoring platelet levels, the trend as well as the absolute value is important, and the mother with a rapidly falling count should be regarded with more suspicion than the one with a low, but stable, platelet level. In general, patients with a platelet count of greater than 75 x 109 l in the...

Treatment of thromboembolism in pregnancy

Treatment of thromboembolism is with intravenous heparin initially (although subcutaneous low-molecular weight heparins (LMWHs) are increasingly used) and should not be delayed whilst awaiting investigation. Warfarin is associated with fetal abnormalities and in particular should be avoided in the first trimester and after 36 weeks' gestation. Acute treatment is followed by subcutaneous prophylactic heparin. It had been thought that prophylactic heparin caused stillbirth, prematurity and haemorrhage but more recent reviews controlling for maternal comorbidity have cast doubt on this assertion. Prophylaxis with LMWHs is now recommended because their use is associated with a lower incidence of osteoporosis and thrombocytopenia than unfractionated heparin, they require less monitoring, and they may be given as a once daily dosage. However, LMWHs have a prolonged action and are only partially reversible with protamine, meaning that LMWH prophylaxis may delay administration of regional...

Antiphospholipid Antibodies

A recent single-center study of 600 SLE patients showed that the prevalence of antiphospholipid antibodies was 24 15 had IgG aCL, 9 IgM aCL, and 15 lupus anticoagulant 67 . A cluster of clinical events, characterized by neurologic involvement, thrombocytopenia, and IgG aCL, was observed in this study. The association of neurologic involvement with clinical or laboratory features found in APS (e.g., livedo reticularis or thrombocytopenia) was also described in previous SLE studies 152, 153 . High titers of IgG aCL were strongly associated with CNS involvement 152 . A multivariate analysis showed that aPLs are independently associated with cerebrovascular disease, headache, and seizures in SLE. The presence of lupus anticoagulant (LAC) was independently associated with white matter hyperintensity lesions on MRI 154 .

Myelodysplastic Syndromes and Aplastic Anemia Pathologic and Immunologic Implications

The Myelodysplastic Syndromes (MDS) represent a heterogeneous group of bone marrow diseases of uncertain etiology characterized by a variable degree of cytopenias, predominantly but not exclusively anemia that is often macrocytic (1). The cytopenias reflect both ineffective hematopoiesis (marrow dysplasia or accelerated apoptosis) and increase in marrow leukemic blasts (2). In 85 of cases the marrow is normo to hypercellular but in 15 the marrow cellularity can be below 30 and, on occasion, below 15 , which raises the differential diagnosis with acquired aplastic anemia (3). In such instances it is necessary to depend on the morphologic identification of significant dysplasia of one or more of the myeloid cell lines or the identification of small clusters of blasts on a bone marrow biopsy. Over 50 of all cases occur in patients over the age of 70 years. Each year some 15,000 individuals will be diagnosed with MDS, although this may well be an underestimate. In a recent national survey...

The Antiphospholipid Syndrome

The Antiphospholipid Syndrome (APS), also called Hughes syndrome, is a multi-system thrombophilic disorder associated with circulating AABs directed against negatively charged phospholipids (PL) and PL-binding proteins 54, 55 . APS may occur as an isolated disease entity (primary APS) or in combination with another autoimmune disease, especially systemic lupus erythematosus (secondary APS). The clinical features of APS are caused by venous and or arterial thrombosis and or pregnancy morbidity. According to the localization and severity of thrombosis, the clinical picture is extremely variable, and the complications arising from the disease may be minimal to life-threatening. International clinical and laboratory criteria have provided consensus on the typical features of the syndrome 54 . Several manifestations are relatively common in APS patients (deep vein occlusions affecting the lower limbs, stroke, large vessel occlusions, fetal loss, thrombocytopenia, livedo reticularis)....

Von Willebrands disease

Type IIb (1 ) in which vWF multimers are reduced in the plasma, but increased on the platelet surface.There is decreased platelet survival and a mild to moderate thrombocytopenia. Bleeding time is prolonged. Pregnancy may be associated with worsening thrombocytopenia. Infusions of desmopressin may further reduce the platelet count and should be avoided.

Clinical Safety of Adenoviral Vector Products

Most of the completed and ongoing adenoviral vector clinical trials are early, uncontrolled trials. The absence of an internal control group limits the ability to draw definitive conclusions about the contribution of the adenovirus vector product to an adverse event. Despite this caveat regarding causality assessments, administration of replication defective adenovirus is associated with an acute cellular and cytokine mediated inflammatory response. Individuals have experienced systemic reactions such as fever, chills, hypotension, and laboratory findings consistent with disseminated intravascular coagulation, including thrombocytopenia. An overwhelming systemic inflammatory response, to which has been attributed, at least in part, the death of a volunteer in a trial of ornithine transcarbamylase (OTC) deficiency who received intrahepatic artery injection of a high dose of adenovirus-containing product, has not been observed in other clinical trials, including those that employ...

Selection of Candidates for Surgical Resection

The extent of underlying liver disease is a variable known to have a significant effect on operative morbidity and mortality. The presence of steatosis, fibrosis, and cirrhosis is associated with an increased perioperative risk. Studies have shown that only patients with severe fibrosis are at a higher risk for complications and death from liver failure after resection. In one study, 32 of patients with severe fibrosis (cirrhosis) who underwent a right hepatectomy died from liver failure compared with none of the patients with grade 0 to 3 fibrosis (Farges et al, 1999). As a rule, we do not operate on patients who are Child-Pugh class B or C. Similarly, we do not recommend resection for those patients who manifest clinical or radiologic signs of portal hypertension, including splenomegaly, abdominal collaterals, or thrombocytopenia. In fact, the preoperative portal pressure has been found to be an important factor in determining the risk of postoperative liver decompensation in...

Management General Supportive Care Table 1094

Coagulopathy in the setting of FHF is primarily the result of decreased hepatic production of clotting factors, although intravascular consumption and vitamin K deficiency may also contribute in particular patients. Thrombocytopenia is also quite common and probably of multifactorial etiology. Vitamin K administration (10 mg subcutaneously every day for 3 days) is safe but rarely effective in correcting coagulopathy from FHF. Because the prothrombin time is a very useful marker of prognosis in FHF, fresh frozen plasma (FFP) administration is generally reserved for clinically overt bleeding and invasive procedures. However, occasionally we administer FFP for profound coagulopathy (ie, international normalized ratio > 6) alone, particularly when a decision to proceed with liver transplantation has been made. Typically patients are given

Clinical Presentations Diarrhea

Therefore, endoscopic evaluation should be directed by the patient's symptoms and the need to exclude infectious etiologies. in the upper Gi tract, the duodenum is often the segment that gives the most dramatic endoscopic appearance of GVHD (Figure 48-2). Excessive GI bleeding from duodenal biopsies rarely occurs if the platelet count is above 50,000 mm3. The histologic diagnosis is based on the presence of apoptotic colonocytes, loss of crypts, and, at times, a neutrophilic or eosinophilic infiltrate. Although no definite pathognomonic endoscopic appearance of GVHD has been conclusively established, the appearance of widespread desquamation in the esophagus, duodenum or colon, is highly suspicious for the severest form of GVHD (Cruz-Correa et al,2002).


Transhepatic Intraportal Shunt System

Right heart failure is an absolute contraindication to TIPS, as these patients respond badly to arrival of high-volume portal venous flow directly into their compromised right hearts. Fatal cases of congestive heart failure have occurred. Severe hepatic encephalopa-thy or liver failure is a relative contraindication to TIPS, as the shunt decreases intrahe-patic portal venous flow in most patients, potentially diminishing already compromised function. That being said, TIPS is often performed in this patient population on a nothing to lose basis, as it is sometimes the only available therapy for a severely encephalopathic patient who is also bleeding. Whereas untreatable encephalopathy, liver failure, and death sometimes do result, many patients do surprisingly well, and seemingly unsalvageable patients can sometimes be salvaged. Severe coagulopathy caused by liver dysfunction is unfortunately common in this patient population, but is only a relative contraindication to TIPS. The...

Preoperative Testing And Preparation

Mesenteric Vein

Cirrhotics, atrophy of the right lobe, hypertrophy of the left lobe, and hypertrophy of the caudate lobe are all common, and can require alteration of the plan of attack. Endoscopy is very desirable preoperatively in cases done for upper gastrointestinal bleeding. Although common, varices are not the only possible reason for upper gastrointestinal bleeding in alcoholic patients. TIPS has no beneficial effect on common nonvariceal causes of bleeding such as Mallory-Weiss tears or gastritis. Blood tests include LFTs, BUN, and Creatinine determination, PT PTT, hemoglobin, hematocrit, and platelet count. Hydration of the patient is desirable, particularly in those with any degree of renal insufficiency. Radiographic contrast presents a special risk to those patients. Prophylactic antibiotics are generally given to patients preoperatively. Many regimens exist. Correction of coagulapathy is sometimes desirable, but fairly controversial. So many of these patients have baseline coagulopathies...

Congenital Anomalies of Granulocytopoiesis Fig 10 af

Detect Eosinophils Tissue

This disorder has an autosomal dominant mode of inheritance and is associated with mild leukopenia and thrombocytopenia. The neutrophilic granulocytes contain predominantly rod-shaped inclusions of a pale- to dirty-blue color, approximately 2-5 im in diameter, which are found on electron microscopy to consist of dense RNA fibrils and are distinguishable from the Dohle

Acquired thrombophilia

Of women with recurrent miscarriage (three or more), 15 have persistently positive results for phospholipid antibodies. If untreated, 90 will have spontaneous abortions or stillbirths in subsequent pregnancies. It is possible that lupus anticoagulant (30 of cases) and anticardiolipin antibodies (70 of cases) are the same autoantibody identified in different assays. Clinical features of the antiphospholipid syndrome are recurrent fetal loss, thrombosis (arterial and venous), thrombocytopenia, haemolytic anaemia, hypertension, pulmonary hypertension and livedo reticularis. Antiphospholipid syndrome is associated with a 5 incidence of thromboembolism or cerebrovascular accident in pregnancy.

Ispinesib and related compounds

For the combination of ispinesib and carboplatin, DLTs included prolonged (X5 days) grade 4 neutropenia, grade 4 thrombocytopenia, and grade 3 febrile neutropenia. The OTR was ispinesib at 18 mg m2 (the Phase II dose) and a car-boplatin target AUC of 6 (a commonly used monotherapy target exposure), both administered for q21 days. At the OTR, gastro intestinal (GI) toxicities were limited to grade 1 2 and minimal reports of grade 1 neuropathy were noted. The incidence of grade 3 4 thrombocytopenia and grade 3 4 neutropenia at the OTR were lower relative to full doses of carboplatin and ispinesib, respectively. At the OTR, is-pinesib concentrations did not appear to be affected by carboplatin and systemic exposures of carboplatin were within 11 of predicted values, suggesting no interaction with ispinesib. One patient with breast cancer had the best response of partial response at cycle 2. A total of 13 28 (46 ) patients had a best response of stable disease (duration 3-9 months) 15 .

Hereditary spherocytosis

A familial haemolytic anaemia of autosomal dominant inheritance in which premature destruction of intrinsically abnormal erythrocytes occurs in the spleen. Specific molecular defects have been found.The clinical severity of the disease varies considerably (Eber et al 1990).Anaesthesia may be required for splenectomy and or cholecystectomy. However, a conservative approach to splenectomy is being adopted avoidance of surgery in those with mild to moderate disease is now recommended. After splenectomy there are increases in haemoglobin, average platelet count, and cholesterol concentration.These are associated with a long-term increased risk of atherosclerotic events, such as stroke and myocardial infarction (Schilling 1997).The possibility of partial splenectomy is being explored (Tse & Lux 1999). If splenectomy is thought necessary, there is controversy as to whether prophylactic cholecystectomy should be undertaken at the same time, and whether or not both should be performed...

Cryoablation and Radiofrequency Ablation

Tumors adjacent to the major vascular channels (portal or hepatic vein or IVC) may not be reliably ablated because the blood flow acts as a thermal sink, protecting the malignant cells on the vessel wall from the applied temperature extremes. The great advantage of these techniques is that they can destroy tumor in disparate parts of the liver and preserve intervening parenchyma. They are, therefore, ideally suited to smaller tumors. Indeed, the larger the amount of dead (frozen or coagulated) left in the liver, the more likely are adverse systemic consequences. These adverse consequences are a variant of a systemic inflammatory response and include thrombocytopenia, myoglobinuria, ATN, and noncardiogenic pulmonary edema. They are increasingly prevalent as the volume of ablated tissue passes 30 of the liver volume, and appear to be more prevalent following cryoabalation than following RFA (6).

GI Manifestations in Other Primary Immunodeficiency Diseases

Patients with AT seemingly have an increased frequency of malignancy, as noted above, although an increased risk of GI malignancy has not been reported in the literature. GI manifestations, including giardiasis, occur in the subset of AT patients with IgA deficiency. Besides oral can-didiasis, GI manifestations are not frequently seen in DiGeorge's syndrome. Chronic intestinal viral infections have been reported to cause diarrhea and necrotizing enterocolitis. The most prominent GI manifestation in WAS is intestinal hemorrhage due to thrombocytopenia. Of note, the mouse model of WAS has colitis, and nonspecific colitis has been reported in WAS.

Supplemental Reading

Audibert F, Friedman SA, Frangieh AY, Sibai BM. Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J Obstet Gynecol 1996 175 460-4. Bacq Y. Acute fatty liver of pregnancy. Semin Perinatol 1998 22 134-40.

Intrahepatic Cholestasis of Pregnancy

Liver disease in pregnancy. Clin Perspectives Gastroenterol 2001 4 351(17). AFLP acute fatty liver of pregnancy ALT alanine aminotransferase AST aspartate aminotransferase HELLP hemolysis, elevated liver enzymes, and low platelets syndrome ICPH intrahepatic cholestasis of pregnancy PT prothrombin time. Adapted from Larson AM. Liver disease in pregnancy. Clin Perspectives Gastroenterol 2001 4 351(17). AFLP acute fatty liver of pregnancy ALT alanine aminotransferase AST aspartate aminotransferase HELLP hemolysis, elevated liver enzymes, and low platelets syndrome ICPH intrahepatic cholestasis of pregnancy PT prothrombin time.

Enterocytozoan bienusi and Encephalitozoon intestinales Microsporidiosis

This organism's clinical presentation is similar to that of cryptosporidium and isospora. In immunocompetent patients, the diarrheal illness is usually self-limited. In immunocompromised persons, particularly AIDS patients with CD4 < 100 mm3, it is a common cause of chronic diarrhea and can be remitting over months. Treatment depends on the species identified. For Encephalitozoon intestinales, the preferred regimen is albendazole 400 mg orally 2 times daily for at least 3 weeks alternatively, metronidazole 500 mg orally three times daily has variable efficacy. Only recently, a small study among immunocom-prised patients identified an effective treatment for Enterocytozoan bieneusi fumagillin 60 mg d for 14 days (Molina et al, 2002). Fumagillin, however, is associated with high rates of neutropenia and thrombocytopenia. For AIDS patients, again immune reconstitution with antiretroviral therapy is often the best therapy.

Portal andor Splenic Vein Thrombosis

In isolated SVT, symptoms are limited to splenomegaly and associated abdominal pain and possible gastro-esophageal varices. Children may also show anemia and thrombocytopenia. If refractory to conservative treatment, such cases may require spleenectomy or preferably transar-terial partial splenic embolization. This alleviates throm-bocytopenia and anemia and decreases splenic blood flow, thus diminishing splenic vein pressure and possibly diminishing varices as well. Partial splenic embolization has the added advantage of preserving splenic immune function.

PKCa LY900003 ISIS 3521 Affinitac

In a phase I trial, ISIS 3521 was delivered over a period of 21 d by continuous iv infusion followed by a 7-d rest period (39). Doses were increased from 0.5 to 3.0 mg kg d. Twenty-one patients with incurable malignancies were treated in five patient cohorts. The maximum tolerated dose was 2.0 mg kg d, equivalent to pharmacologically active doses against human xenografts in mice. The dose-limiting toxicities were thrombocytopenia and fatigue at a dose of 3.0 mg kg d. Evidence of tumor response lasting up to 11 mo was observed in three of four patients with ovarian cancer. Another phase I study analyzed the combination of ISIS 3521 with 5-fluorouracil and leukovorin (5-FU LV) in patients with advanced cancer. ISIS 3521 was tolerable when given with 5-FU LV. Partial remission was seen in 2 of 14 patientsp however, it is uncertain whether clinical activity is a result of enhanced drug interaction (40). A phase I II trial of ISIS 3521 with carboplatin and paclitaxel in NSCLC has been...

Shwachman Diamond Syndrome

There are several mutations in the SBDS gene discovered to date and with the phenotypic variation among individuals, gene testing may become a tool similar to its use in CF as this form of testing becomes more available. By and large the diagnosis is made in infancy and is based on the constellation of clinical features, along with blood tests and radiologic investigations. Acinar and ductal exocrine pancreatic dysfunction may be quantified by pancreatic stimulation tests but they are demanding and no standard methodology has been established. Serum testing for pancreatic enzymes may be useful as a diagnostic tool in suspected patients because serum pancreatic isoamylase remains low in SDS patients in contrast to the serum cationic trypsinogen that may increase with advancing age. Bone marrow dysfunction is characterized in peripheral blood counts by persistent or intermittent anemia, leukopenia, and or thrombocytopenia (Dror et al, 2002). Radiologic investigations reveal abnormal...

Toxicity of Intravenously Administered ARCAs in the Absence or Presence of Docetaxel

A synergistic antitumor efficacy was observed when CV787 was combined with taxane in the prostate tumor xenograft 88 . To examine the toxicity of CV787 in combination with the chemotherapeutic agent docetaxel, we conducted a fourth toxicology study in C57BL 6. The 4-day and 2 8-day effects were evaluated in mice given a single daily dose of dexamethasone (Decadron) each day for 3 consecutive days, a single injection of CV787 at low (1 x 108 particles per animal), medium (3 x 109 particle per animal), or high (1 x 1011 particles per animal) dose, and a single dose of the chemotherapeutic agent, docetaxel (100 mg m2). Six different treatment groups were established using identical treatment regimens for animals in subgroups sacrificed at 4 days and at 28 days. Each treatment subgroup consisted of eight randomly assigned mice. The ARCA virus vehicle and all three doses of virus were given intravenously via tail vein injection. All virus injections were given on day 1 at 1-2 h prior to...

Chemotherapy in the HAART

Good risk group was 51 for ACVBP versus 47 for CHOP (p 0.85), in the intermediate risk group, 28 for CHOP versus 24 for low-dose CHOP (p 0.19), and in the poor risk group 11 for low-dose CHOP versus 3 for vincristine and steroid (p 0.14). Only significant factors in this study for overall survival proved to be HAART (relative risk (RR) 1.6, p 0.0002),HIV score (RR 1.7, p 0.0001), and the IPI score (RR 1.5,p 0.0012), but not the intensity of chemotherapy.96 An infusional regimen of cyclophosphamide, doxorubicin, and etoposide (CDE) with and without HAART (only didanosine) resulted in complete response rate of 45 and median overall survival was 12.8 months. At the time of the analysis, 30 in the pre-HAART group were alive compared with 47 in the HAART group. Further, patients in the HAART group experienced less nonhematologic toxicity (22 vs 42 ), thrombocytopenia (31 vs 52 ), and anemia (9 vs 27 ).97

Treatment of Anemia in Elderly Patients with CKD

Given the limited data available, elderly patients with CKD, whether on dialysis or not, should probably be evaluated and treated for their anemia as generally recommended for patients with CKD regardless of age (5). The most recent clinical practice guidelines and recommendations for the National Kidney Foundation KDOQI for Anemia in CKD recommend that Hgb testing be carried out at least annually in all patients with CKD, regardless of stage or cause, and that a diagnosis of anemia should be made and evaluation should be undertaken when the Hgb level is less than 13.5 g dL in adult males and 12.0 g dL in adult females. This evaluation should include a complete blood count (CBC), red blood cell indices (mean corpuscular hemoglobin MCH , mean corpuscular volume MCV , mean corpuscular hemoglobin concentration MCHC ), white blood cell count, and differential and platelet count, absolute reticulocyte count, serum ferritin to assess iron stores, and serum TSAT or content of Hb in...

Peptic Ulcer Disease

Prior to extensive oral surgical or periodontal procedures, physicians should consult with the dentist of record who should be advised of the patient's serology, especially if the patient has had a history of ulcer perforation and subsequent hemorrhage resulting in anemia. Delayed healing and risk of bacterial infection, particularly anaerobic bacterial infection due to tissue hypoxia, and the potentially grave side effects of respiratory depression induced by narcotic analgesics, are examples of such associated oral surgical risks in the chronically anemic GI patient. Cimetidine and rantidine, drugs commonly prescribed for duodenal ulcer patients, have occasionally been associated with thrombocytopenia and may compete with antibiotics or antifungal medications as noted below.

Liver Diseases

The physician should advise dental professionals that a dental patient presenting with a history of liver cirrhosis deserves special attention. Patients with cirrhosis may have significant hemostatic defects, because of nutritional deficiency, an inability to synthesize clotting factors, or because of secondary thrombocytopenia. Medical management such as replacement with fresh frozen plasma and platelets can be employed prior to anticipated oral surgical or periodontal procedures. Specifically, a complete blood count, prothrombin time or international normalized ratio, partial thromboplastin time , platelet count, and bleeding time should be obtained and transmitted to the dentist (Glick, 1997). The dentist can assist the physician with regard to dietary counseling deemed necessary to manage nutritional deficiency.

Laboratory Findings

Blood and peritoneal fluid cultures will yield organisms of enteric origin in about one-fourth of patients. Yeast may be isolated from peritoneal fluid, especially in infants who had been treated with antimicrobials. In the event of an outbreak in a nursery, it is important to evaluate both cases and matched concurrent controls. Viruses can be detected antigenically or through genetic methods. In some infants, the white blood count may be very low or very high and the platelet count usually will be diminished and falling rapidly. At least 50 of infants with NEC have platelet counts of 50,000 per millimeter or less (45). Prothrombin and partial thromboplastin times are elevated. Hyponatremia is common at the outset of NEC.


Pre-eclampsia encompasses HELLP (haemolysis, elevated liver enzymes and low platelets) syndrome, eclampsia and possibly acute fatty liver of pregnancy. Although the disease is progressive, a mother may be asymptomatic until she presents with an eclamptic fit, and although pre-eclampsia is a disease of pregnancy, terminated only by delivery, pre-eclampsia, HELLP syndrome and eclampsia may all present only after delivery.


There have been few published cases of polycythaemia complicating pregnancy. There may be associated thrombocytopenia or thrombocythaemia. Thrombocytopenia may be dilutional and may not reflect function. Thrombotic events (arterial and venous) do occur and prophylactic aspirin and heparin have been given to prevent them.

GI Bleeding

GI bleeding may complicate GVHD, and is associated with a worse prognosis overall from SCT (Nevo et al, 1999). GI bleeding may be due to severe GVHD or may result from gastric antral vascular ectasia (GAVE). GAVE appears as diffuse antral telangectasias which may not be apparent due to the bright red blood adherent to the antral mucosa. Endoscopic control of bleeding in this condition is often unsuccessful, because bleeding is slow and diffuse and not readily amenable to bicap cautery or YAG laser therapy. Antrectomies have been attempted often with disastrous results. Cryotherapy, which may be effective for the control of chronic GI blood loss due to the telangectasias associated with watermelon stomach, appears less effective in SCT patients due to prolonged thrombocytopenia. We therefore recommend supportive therapy and correction of thrombocytopenia if possible. The chapter on upper GI bleeding has a section on GAVE (see Chapter 28, Upper Gastrointestinal Bleeding).

Figure 1736

Blood film abnormalities, microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure occur in accelerated hypertension and acute vascular rejection. A renal biopsy usually distinguishes acute vascular rejection, and malignant hypertension should be obvious clinically. The microangiopathy of cyclosporine can be difficult to differentiate from hemolytic uremic syndrome however, glomerular pathology usually is less marked and vascular changes more obvious with cyclosporine toxicity. De novo hemolytic uremic syndrome also has been reported in patients treated with tacrolimus (FK-506) 27 .


In a patient with decompensated cirrhosis, treatment can be extremely challenging with the risk of major adverse events (eg, severe thrombocytopenia, variceal hemorrhage, etc) and further decompensation (eg, worsening hepatic encephalopathy), and should therefore, be handled in liver transplant centers with the requisite experience and infrastructure, and preferably referred for clinical trials until the safety and efficacy data of treatment are established. However, patients with advanced bridging fibrosis or early cirrhosis (Child's class A) can often be treated in the community without major complications. The efficacy of treatment in patients with advanced fibrosis or compensated cirrhosis has been derived mostly from subgroup analysis of larger clinical trials, and suggests that the response is lower than in patients without cirrhosis. The main treatment option for such patients is liver transplant.


Cocaine, or its water-insoluble derivative crack, causes central and peripheral dopa-minergic and adrenergic stimulation resulting in euphoria, increased alertness, vasoconstriction and hypertension. Myocardial ischaemia and arrhythmias may occur, and convulsions, intracranial haemorrhages and renal, hepatic and haematological impairment (including thrombocytopenia) have been reported. Cocaine abuse has been associated with increased incidence of spontaneous abortion, placental abruption, premature labour and fetal morbidity and mortality. Prolonged action of suxamethonium has also been reported. Diagnosis may be difficult since its use is often denied and the presentation may resemble that of pre-eclampsia and phaeochromocytoma. Urine remains positive for cocaine metabolites up to 3 days after use, and testing has been suggested in all at-risk groups (e.g. known users of other drugs, unbooked pregnancies, etc.).

Figure 1015

New-onset azotemia, proteinuria, and hypertension occurring in the second half of pregnancy should be distinguished from pre-eclampsia. Most cases of preeclampsia are associated with only mild azotemia significant azotemia is more suggestive of renal disease. Azotemia in the absence of proteinuria or hypertension would be unusual in preeclampsia, and thus, would be more suggestive of intrinsic renal disease. Thrombocytopenia, elevated liver function test results, and significant anemia are not typical features of renal disease (except for thrombotic microangiopathic syndromes) and are features of the variant of preeclampsia known as the hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome.

Figure 514

The pelvic and parenchymal hemorrhages are secondary to severe thrombocytopenia. Microscopically, many myeloblasts are seen in the interstitial infiltrates. Interstitial infiltration by hematologic neoplasms is usually bilateral, diffuse, and more prominent in the cortex 14 . Renal failure is unusual. When it does occur, affected patients generally present with relatively acute renal failure and a benign urinalysis. The kidneys are grossly enlarged, as may be demonstrated by renal ultrasound, by CT scan, or in some cases even by physical examination. The differential diagnosis in this setting includes obstruction and other tubulointerstitial disorders. The presence of large kidneys without hydronephrosis on ultrasonography in a patient with lymphoma or leukemia, however, is highly suggestive of tumor infiltration. The renal prognosis is dependent on the responsiveness of the tumor to radiation or chemotherapy. A rapid reduction in renal size and...

Figure 1735

The microangiopathic hemolysis of recurrent hemolytic uremic syndrome (HUS) is identical to the original disease, with extensive erythrocyte fragmentation and thrombocytopenia. The incidence of HUS recurrence is difficult to assess. At one extreme, five of 11 children suffered graft loss because of recurrent disease. However, most series have reported substantially lower recurrence rates no recurrences in 16 adults and children, one of 34 grafts in 28 children, and two probable recurrences of 24 grafts in 20 children 4,45,46 . Graft loss occurs in 10 to 50 of patients with recurrence. HUS has been diagnosed 1 day to 15 months after transplantation (usually in less than 2 months), and the incidence of recurrence is increased in patients receiving grafts less than 3 months after their initial disease. Treatment of recurrent disease is plasma exchange for plasma or cryosupernatant, or plasma infusions, and dose reduction of cyclo-sporine. Recurrence may be prevented by aspirin and...

Figure 31

The disease is inherited as an X-linked trait in some families, however, autosomal recessive and perhaps autosomal dominant forms exist. Clinically, the disease is more severe in males than in females. End-stage renal disease develops in persons 20 to 40 years of age. In some families, ocular manifestations, thrombocytopenia with giant platelets, esophageal leiomyomata, or all of these also occur. In the X-linked form of Alport's syndrome, mutations occur in genes encoding the a-5 chain of type IV collagen (COL4A5). In the autosomal recessive form of this syndrome, mutations of either a-3 or a-4 chain genes have been described. On light microscopy, in the early stages of the disease the glomeruli appear normal. With progression of the disease, however, an increase in the mesangial matrix and segmental sclerosis develop. Interstitial foam cells are common but are not used to make a diagnosis. Results of immunofluorescence typically are negative, except in glomeruli with...

Craf1 ISIS 5132

Cunningham et al. (31) reported the results of a trial testing continuous iv infusion of ISIS 5132 for 21 d every 4 wk in 34 patients with a variety of solid tumors refractory to standard therapy. Toxicities up to 4.0 mg kg were not dose limiting. Doses of 2.0-4.0 mg kg are comparable with doses in mice at which activity was observed in human xenograft models. Grade 3 fever occurred in 2 of the 34 patients treated. One patient treated with 5.0 mg kg had fever as a dose-limiting toxicity. Three grade 3 or 4 thrombocytopenia and one grade 3 leukopenia were observed. Two patients developed sepsis one of them, while septic, manifested grade 4 thrombocytopenia, grade 4 hyperbilirubinemia, and a grade 3 elevation in aspartate aminotransferase the other developed grade 4 thrombocytopenia. Leukopenia was mild, and no patient had neutropenia. One patient with therapy refractory ovarian cancer had a dramatic reduction in her CA-125 level (97 ), and two other patients had prolonged disease...

Metastatic Disease

Gemcitabine (1500 mg m2) at a rate of 10 mg m2 min. The drug was given weekly for 3 consecutive weeks every 4 weeks in both arms of the study. Patients treated with the fixed-dose-rate regimen experienced more toxicity, with a 49 and 37 occurrence of neutropenia and thrombocytopenia versus a 28 and 10 occurrence, respectively, in patients treated in the conventional schedule (Tempero et al, 2003). Patients on the fixed-dose-rate had a higher response rate (11.6 vs 4.1 ), median survival (8 vs 5 months, p .013) and 1-year survival (23.8 vs 7.3 ) than patients treated on the conventional schedule. This strategy is now being tested in randomized phase III studies.

Management Issues

All cellular lines of the bone marrow may be abnormal. The most common hematologic abnormality is neutropenia and it is usually intermittent. As well, anemia with low reticulocytes and thrombocytopenia may be identified. As a result of bone marrow dysfunction SDS patients are at risk of bleeding, developing severe infections, and suffering from periodontal disease. in cases of life threatening infection, granulocyte-colony stimulating factor (G-CSF) may be required. All three cell lines can be involved, and patients with this complication are at a greater risk of developing severe aplasia, advanced myelodysplastic syndrome, or acute myeloid leukemia. SDS-related leukemia carries a poor prognosis. ongoing consultation with an hematolo-gist would be advisable to decide how often to perform

Analgesia for labour

Regional analgesia is the method of choice. Good analgesia prevents hypertensive episodes associated with contraction pain. Well-conducted epidural or combined epidural-spinal analgesia may be beneficial to the compromised fetus by improving uteroplacental perfusion. A combination of low-dose local anaesthetic and opioid may be given by continuous epidural infusion or intermittent boluses, and this can be supplemented as necessary should instrumental or operative delivery be required. A pre-epidural platelet count should be performed (if trends suggest that platelet numbers are decreasing significantly, a platelet count should be repeated immediately before epidural injection is commenced). Current opinion suggest that a platelet count of at least 80 x 109 l is advisable before instituting central neural blockade, although any stated lower safe limit is entirely arbitrary, and the relative risks and benefits of regional analgesia and anaesthesia must be considered for each patient....

Figure 313

Hemoglobin and platelet values within 30 days of diagnosis of primary systemic amyloidosis. Anemia was not a prominent feature. When present, it usually is due to multiple myeloma, renal insufficiency, or gastrointestinal bleeding. Thrombocytosis was relatively common in 9 of patients the platelet count was over 500 X 109 L. Functional hyposplenism from amyloid replacement of the spleen may occur 8 . Hyposplenism is manifested by the presence of Howell-Jolly bodies and occurs in about one fourth of patients. (Adapted from Kyle and Gertz 5 .)

Figure 1016

Most pregnant women with acute renal failure have acute tubular necrosis secondary to either hemodynamic factors, toxins, or serious infection. Occasionally, glomerulonephritis or obstructive nephropathy may be seen. Acute cortical necrosis may complicate severe obstetric hemorrhage. Acute renal failure may be a complication of the rare syndrome of acute fatty liver of pregnancy, a disorder that occurs late in gestation characterized by jaundice and severe hepatic dysfunction. This syndrome has features that overlap with the hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome variant of preeclampsia as well as microangiopathic syndromes (eg, hemolytic uremic syndrome and thrombotic thrombocytopenic purpura). HELLP hemolysis, elevated liver enzymes, and low platelet count.

Risk Factors

Most patients in an ICU are at risk for SRES, and those most susceptible to gastroduodenal mucosal injury are patients with severe systemic disease. Mechanical ventilation for at least 48 hours and coagulopathy (platelet count of < 50,000, a partial thromboplastin time of more than twice that of control subjects, or an International Normalized Ratio of prothrombin time of > 1.5) have been identified as the two single most important risk factors (Cook et al, 1994). Other risk factors include shock, sepsis, multiple or severe trauma, extensive burns (greater than 35 of body surface area), central nervous system (CNS) lesions (ie, intracranial hypertension), renal failure, liver dysfunction, multiple system organ failure, aspiration pneumonia, postsurgical states, acute coronary syndromes, and length of ICU stay (Cook et al, 1999). The probability of SRES hemorrhage clearly increases proportionally to the number of risk factors present.


In March 1990, a 42-year-old man known to be an episodic heavy drinker and regular smoker suddenly developed a right visual field defect caused by a left occipital brain infarct. A few weeks prior to the index stroke he had an anteroseptal and inferior myocardial infarct, and after discharge from hospital, he had started to drink large amounts of alcohol daily. In April 1995, he developed a right occipital brain infarct. This 'accident' was again preceded by a drinking bout of about two weeks (900 g ethanol per week). On admission, clinical examination showed tubular vision. A computed tomography head scan revealed two separate infarcts located on the left and right occipital lobes. Duplex imaging of the carotid and vertebral arteries showed neither occlusions nor significant stenoses, but transthoracic echocardiography (TTE) showed left ventricular inferior and anteroapical hypokinetic segments without any thrombi. Three days after the onset of the latter stroke, the patient's...


Platelets (see fig. 18.1) are not cells but small fragments of megakaryocyte cytoplasm. They are 2 to 4 m in diameter and possess lysosomes, endoplasmic reticulum, a Golgi complex, and Golgi vesicles, or granules, that contain a variety of factors involved in platelet function. Platelets have pseudopods and are capable of ameboid movement and phagocytosis. In normal blood from a fingerstick, the platelet count ranges from 130,000 to 400,000 platelets L (averaging about 250,000 L). The count can vary greatly, however, under different physiological conditions and in blood from different places in the body. When a blood specimen dries on a slide, platelets clump together therefore in stained blood films, they often appear in clusters.


There is extensive literature on the efficacy and safety of MTX in pediatric rheumatology and there is growing acceptance of its use in children with CD (Mack et al 1998 Woo et al, 2000 Giannini et al, 1992 Ravelli et al, 1998). Although uncommon, the potential risk of hepatic fibro-sis with advancing accumulated dose should be monitored. Potential bone marrow toxicity and interstitial pneu-monitis are also a concern. Dosing in children involves weekly parenteral administration, either via subcutaneous or intramuscular injection. Taking MTX orally often leads to intolerable nausea and absorption is variable, but no studies have been done in children comparing oral MTX with parenteral use for maintenance therapy in CD. Parenteral MTX has the added benefit of improved compliance. Because the mechanism of action of MTX is via inhibition of folate metabolism, supplementation with folate at a dose of 1 mg d is a recommended. We typically dose MTX at weekly subcutaneous injections of 10 mg...

Preventive Therapy

Destruction of the bile ducts can also obliterate small portal vein branches, resulting in presinusoidal noncirrhotic portal hypertension, similar to that seen in hepatic sarcoidosis. As a consequence, patients with PBC may have significant portal hypertension even with fairly early, noncirrhotic-stage PBC therefore, upper endoscopy to screen for varices should be performed at diagnosis. If present, varices should be managed with p-blockade and band ligation as appropriate. No validated interval for variceal screening has been identified. We have shown that it is very unlikely for patients to have varices with a platelet count of a 200,000 L as a surrogate marker for hypersplenism secondary to portal hypertension. Consequently, endoscopy is performed every 1 to 2 years in those with platelets < 200,000 L. Because variceal hemorrhage may not be a consequence of end-stage liver disease in PBC, a single bleed is generally not considered an indication for liver transplantation.

HELLP Syndrome

Periportal Lymph Breast Cancer

Gestational thrombocytopenia is seen in 4 to 8 of uncomplicated pregnancies, whereas thrombocytopenia in preeclampsia ranges from 15 to 50 . Those who develop gestational thrombocytopenia are sevenfold more likely to develop HELLP. Platelet levels in HELLP syndrome are frequently less than 100,000 L, and there is a positive correlation between the extent of platelet decline and the severity of liver abnormalities. Elevations of serum aminotransferases FIGURE 120-2. Eclampsia hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP). Periportal fibrin deposition, hemorrhage, and hepatocellular necrosis seen in severe eclampsia. The classic histologic picture of HELLP is one of periportal or focal parenchymal necrosis, hyaline deposits, and vascular microthrombi. In some cases, fibrin exudate has been reported similar to that seen in eclampsia. Courtesy of Carolyn A Riely, MD. FIGURE 120-2. Eclampsia hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP)....

Scabetic Lesion

Trichophyton Tonsurans Infection

CT scan of the same infant several days later with congenital toxoplasmosis. Note the rapid progress with massive loss of brain parenchyma and multiple scattered areas of calcification. Peripheral white blood count was remarkable for 96 eosinophils. There were numerous eosinophils in the cerebrospinal fluid. In toxoplasmosis, anemia, thrombocytopenia, and at times severe leukopenia may be present. The cerebrospinal fluid is xanthochromic, has an elevated protein level, and may contain erythrocytes and leukocytes.

Gauchers disease

In children, anaemia and thrombocytopenia are present in 80 and 60 respectively. A number of coagulation abnormalities have been reported, including factor IX deficiency and acquired von Willebrand's disease. In 32 patients with type 1 disease who did not have thrombocytopenia, 22 had abnormal platelet aggregation (Gillis et al 1999). It has been suggested that abnormal coagulation tests may be secondary to the presence of increased cerebroside levels interfering with assays (Billett al 1996). 1. Bleeding manifestations are common, even in patients without thrombocytopenia, and may result from platelet dysfunction (Gillis et al 1999).

Rhagades Syphilis

Congenital Syphilis Baby Skin

Marked hepatosplenomegaly in an infant with congenital syphilis. Hepatomegaly occurs in 50 to 60 of affected infants. It is frequently associated with jaundice, anemia, splenomegaly and ascites. In the spectrum of congenital syphilis there may be no clinical signs of disease at birth or there may be many clinical manifestations which include intrauterine growth retardation, skin manifestations, hepa-tosplenomegaly, jaundice, anemia, thrombocytopenia, and osseous changes.

Cure Of Diseases

Interferon-a is given by subcutaneous injection and most patients can be taught to self-administer the drug. The usual dose in chronic HBV infection is 5 MU daily or 10 MU thrice weekly for a duration of 16 weeks. I find that the daily dosing schedule is usually better tolerated by patients. Interferon almost always causes flu-like symptoms, which may vary from mild to debilitating. Usually these symptoms improve after the first few weeks of treatment despite continued therapy. Interferon is myelosup-pressive and patients must be monitored for neutropenia and thrombocytopenia. Autoimmune disorders can be exacerbated or unmasked by interferon and the treatment is generally contraindicated in patients with autoimmune diseases with the exception of stable hypothyroidism. Depression may occur, especially in patients with a prior history. This treatment should not be used in patients with a history of major depression, suicidal attempts or ideation, or major affective disorders. As...

Hydrops Fetalis

Photos Hydrops Fetus Weeks

Nonimmune hydrops fetalis due to parvovirus B19 infection in a premature infant born at 24 weeks gestation. Note the gross hydrops fetalis. Laboratory analysis was remarkable for a hemoglobin level of 1.4 g dL, hematocrit of 4 , platelet count of 10,000 mm3, and WBC count of 6000 mm3 (which, when corrected for nucleated red blood cells, showed a WBC count of 0). Figure 2.117. Nonimmune hydrops fetalis due to parvovirus B19 infection in a premature infant born at 24 weeks gestation. Note the gross hydrops fetalis. Laboratory analysis was remarkable for a hemoglobin level of 1.4 g dL, hematocrit of 4 , platelet count of 10,000 mm3, and WBC count of 6000 mm3 (which, when corrected for nucleated red blood cells, showed a WBC count of 0).

Bone Marrow

Percutaneous aspiration of the posterior iliac spine is the current method of choice for obtaining a bone marrow sample. It is a relatively safe procedure, and with some practice it can be done more easily and with less pain than sternal aspiration. Marrow aspirate and a core sample can be obtained in one sitting with a single biopsy needle (e.g., a Yamshidi needle). When proper technique is used, the procedure is not contraindicated by weakened host defenses or thrombocytopenia. However, there is a significant risk of postproce-dural hemorrhage in patients with severe plas-matic coagulation disorders (e.g., hemophilia), in patients on platelet aggregation inhibitors, and in some pronounced cases of thrombocyto-sis. In all cases the biopsy site should be compressed immediately after the needle is withdrawn, and the patient should be observed. The procedure should be taught by hands-on training in the clinical setting.

Adjunctive Therapy

Argatroban, lepirudin, and bivalirudin are all direct thrombin inhibitors. These agents should replace heparin in cases in which the diagnosis of heparin-induced thrombocytopenia (HIT) type II is confirmed or even suspected. HIT type II is an immune-mediated disorder characterized by the formation of antibodies against the heparin-platelet factor 4 complex, resulting in thrombocytopenia, platelet aggregation, and the potential for arterial and venous thrombosis. The possibility of HIT type II should be raised in patients who demonstrate a platelet count drop to less than 100,000, or by greater than 50 from baseline, in the setting of heparin therapy (usually 5-12 days after initial exposure). Unexplained thrombotic events should also evoke this diagnosis, even in the setting of a normal platelet count. Impaired renal function must be taken into account when selecting the appropriate agent argatroban is the only direct thrombin inhibitor that is hepatically cleared.

Abdominal Pain

An initial examination by ultrasonography should be performed to assess for the presence of gallstones and a thickened gallbladder wall, suggestive of inflammation. If this is equivocal, biliary scintigraphy should be performed to rule out acute cholecystitis. If the gallbladder fills and CCK fails to elicit gallbladder emptying this suggests chronic cholecystitis. The sensitivity of biliary scintigraphy is preserved as long as hepatic bilirubin metabolism is intact. A total bilirubin > 10 mg dL makes this diagnostic test less useful. Once acute or chronic cholecystitis is diagnosed, surgical therapy should be considered even in the SCT patient. Because thrombocytopenia frequently coexists, surgery is delayed until platelet counts can be maintained over 100 K mm3 with or without platelet transfusions. Such patients require triple antibiotic therapy until the cholecys-tectomy can be safely performed. Another option to consider is endoscopic gallbladder stenting, which has been...

Liver Biopsy

There are few true contraindications to liver biopsy. Significant coagulopathy with INR > 1.5, platelet count < 60,000, or prolonged PTT should all be corrected prior to percutaneous biopsy. Relative contraindications include ascites, large hemangiomas, and intra-abdominal infections. Patient compliance is critically important, and those not willing to cooperate cannot undergo biopsy. In these scenarios, alternative methods such as transvenous routes can be used to obtain tissue.

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