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1/37. Autoimmune thrombocytopenia in Waldenstrom's macroglobulinemia.

    Autoimmune phenomena are well-recognised complications of Waldenstrom's macroglobulinemia (WM) and IgM monoclonal gammopathy. Peripheral neuropathy and cold agglutinin hemolytic anemia are the most common reported and occur in 5-10% of patients. Autoimmune thrombocytopenia has been rarely reported in WM and its incidence is not known. In this study we report the case of a 67-year-old man who presented with autoimmune thrombocytopenia who was subsequently found to have WM. Laboratory investigation demonstrated that platelet-associated IgM (PAIgM) but not PAIgG was clearly elevated compared to normal controls. In addition the patient's serum reacted strongly with a panel of donor platelets analysed with an indirect platelet immunofluorescence assay utilising an anti-IgM secondary antibody. Glycoprotein specificity could not however be demonstrated by ELISA techniques for platelet glycoproteins IIbIIIa, IaIIa, IbIXa, and IV. We also reviewed the case records of 104 additional cases of WM diagnosed at our institution between 5/93 and 5/99. Three further cases with clinically significant autoimmune thrombocytopenia were identified. The overall incidence of autoimmune thrombocytopenia (4/105, 3.8%) in this cohort of patients was similar to the incidence of peripheral neuropathy (7/105, 6.7%) and cold agglutinins (3/105, 2.9%).
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2/37. "liver function tests" are not always tests of liver function.

    A child with Wilm's tumor and a child with immune thrombocytopenic purpura (ITP) were each noted to have persistent elevations of aspartate aminotransferase (AST), alanine aminotransferase (ALT), and lactate dehydrogenase (LDH). Both children underwent thorough evaluation for liver disease and, as a result, experienced delays in treatment of the Wilm's tumor and ITP. Eventually both children were found to have extremely elevated serum creatine kinase (CK). Muscle biopsy confirmed diagnoses of Duchenne's muscular dystrophy in one child, and Becker's muscular dystrophy in the second. Hematologists/oncologists should consider obtaining a serum CK to rule out muscle disease in patients with unexplained elevations of AST, ALT, and LDH.
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3/37. A case with myasthenia gravis (MG) emerging after splenectomy for idiopathic thrombocytopenic purpura (ITP): possible effects of thymectomy on autoantibodies.

    The combination of idiopathic thrombocytopenic purpura (ITP) and myasthenia gravis (MG) has been reported infrequently. We report here the development of MG in a patient who underwent splenectomy for ITP ten years earlier, and describe the serum levels of anti-acetylcholine receptor antibodies (anti-AchR) and platelet-associated IgG (PAIgG) followed over 11 years. The concentrations of both autoantibodies have been decreased after thymectomy, suggesting that this procedure is potentially beneficial for treating both MG and ITP.
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4/37. Systemic lupus erythematosus associated with massive ascites and pleural effusion in a patient who presented with disseminated intravascular coagulation.

    A case of systemic lupus erythematosus (SLE) associated with serositis presenting with disseminated intravascular coagulation (DIC) is reported. A 53-year-old woman was admitted because of a fever. Laboratory tests revealed increased plasma levels of fibrinogen degradation products (FDP) and FDP-D-dimer, high titers of anti-nuclear antibody, high serum levels of anti-dna antibody, immune complexes, decreased serum complements, and persistent proteinuria. A CT scan showed massive ascites and pleural effusion, marked edema and swelling of the mesenterium. The patient's condition and immunological abnormalities improved after steroid therapy. The association of DIC and lupus serositis has never been described in the literature.
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5/37. Serum cytokine level during continuous venovenous hemofiltration in toxic shock-like syndrome due to group G beta streptococcus bacteremia in a patient with idiopathic thrombocytopenic purpura.

    We report a case of toxic shock-like syndrome due to a rare infection of group G streptococcus bacteremia in a patient with idiopathic thrombocytopenic purpura and its successful treatment with continuous venovenous hemofiltration (CVVH). As the result of sepsis treatment with CVVH, in addition to administration of vasopressors and antibiotics, serum levels of interleukin-1beta, interleukin-10 and tumor necrosis factor-a fell and shock was controlled.
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6/37. Serologically documented loracarbef (Lorabid)-induced immune thrombocytopenia.

    We report here the first case of severe immune thrombocytopenia induced by a second-generation cephalosporin antibiotic, Loracarbef. A 56-year old white female developed acute severe thrombocytopenia associated with acute respiratory symptoms following administration of Loracarbef. She responded to Loracarbef withdrawal and systemic corticosteroid administration. Loracarbef-dependent platelet-reactive antibodies were demonstrable in her serum by flow cytometry.
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7/37. Anti-c-Mpl (thrombopoietin receptor) autoantibody-induced amegakaryocytic thrombocytopenia in a patient with systemic sclerosis.

    Amegakaryocytic thrombocytopenia (AMT) associated with systemic sclerosis (SSc) has been described in several case reports, but the underlying mechanisms have not been identified. Here we describe a rare case of SSc accompanied by thrombocytopenia and megakaryocytic hypoplasia, in which autoantibody against thrombopoietin receptor (c-Mpl) was detected. A 61-year-old woman with limited SSc was admitted to our hospital because of severe thrombocytopenia (platelet count 0.2 x 10(4)/mm(3)) with gingival bleeding. Her bone marrow was hypocellular with absent megakaryocytes, consistent with AMT. Treatment with corticosteroids and intravenous immunoglobulin infusions resulted in an increased platelet count, and she sustained a remission over a 1-year period, with a platelet count averaging 10.0 x 10(4)/mm(3). Her serum was strongly positive for anti-c-Mpl antibody, and IgG fraction purified from her serum inhibited thrombopoietin-dependent cell proliferation in vitro. Our case report suggests that AMT in patients with SSc could be mediated by the anti-c-Mpl antibody, which functionally blocks an interaction between thrombopoietin and c-Mpl.
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8/37. minocycline-induced immune thrombocytopenia presenting as Schamberg's disease.

    minocycline hydrochloride, a synthetic tetracycline, is a systemic antibiotic that has received much attention over the past several years. Currently, minocycline is considered the most widely prescribed oral antibiotic in the management of acne. minocycline has been associated with autoimmune events, hepatitis, lupus-like syndromes, serum sickness, vasculitis, Sweet's syndrome, and hyperpigmentation. We report a case of a patient who developed drug-induced immune thrombocytopenic purpura (DITP) after taking minocycline. The initial clinical presentation of nonpalpable, discrete nonblanching petechiae and cayenne pepper-like macules on his lower legs was diagnosed as pigmented purpuric dermatosis (Schamberg's disease). We report the first case of DITP with the clinical picture of Schamberg's disease associated with minocycline therapy.
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9/37. Epstein-Barr virus-related lymph node lesion resembling autoimmune disease-like clinicopathological findings in elderly patients. Report of three cases.

    Three cases of Epstein-Barr virus (EBV)-related lymphoproliferative disorders in elderly patients showing autoimmune disease-associated lymphadenopathy-like clinicopathological findings have been reported. Clinically, they were characterized by systemic lymphadenopathy, "B" symptoms, polyclonal hypergammaglobulinemia, elevated serum LDH and transient presence of various autoantibodies, and absence of atypical lymphocytosis in peripheral blood. One case was associated with idiopathic thrombocytopenic purpura. The clinical course was self-limiting. Histologically, they exhibited numerous lymphoid follicles with hyperplastic germinal centers and atypical interfollicular widening with prominent vascular proliferation. In the paracortical area, there was a mixed infiltrate comprising small to medium-sized lymphocytes and plasma cells, and variable numbers of eosinophils and T- and B-immunoblasts. in situ hybridization demonstrated a varying number of EBV-infected lymphocytes in the germinal center as well as in the interfollicular area. polymerase chain reaction demonstrated that neither clonal rearrangement of T-cell receptor gamma-gene nor immunoglobulin heavy-chain rearrangement was detected in two of the cases examined. Although acute EBV infection rarely occurs in older adults, EBV related to reactive lymphoproliferative disorder should be added to the differential diagnosis of autoimmune disease-associated lymphadenopathy and node-based peripheral T-cell lymphoma in elderly patients.
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10/37. amlodipine-induced immune thrombocytopenia.

    BACKGROUND AND OBJECTIVES: Drug-induced thrombocytopenia is a serious, but rare, side-effect of treatment with a number of drugs. In this report, we investigate the suspicion that amlodipine, a calcium-channel blocker, was responsible for immune thrombocytopenia in a 79-year-old patient. PATIENT AND methods: Our patient experienced two attacks of thrombocytopenic purpura after 10 years of treatment with amlodipine. Antibodies to platelets were tested by standard methods. RESULTS: Initially, the platelet count increased owing to treatment with prednisolone and intravenous immunoglobulin g, but decreased shortly after discontinuation of this treatment. The patient's serum was found to contain amlodipine-dependent antibodies to platelets, and he recovered after stopping the drug. CONCLUSIONS: amlodipine can induce immune thrombocytopenia, which may strongly resemble autoimmune thrombocytopenia.
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