Cases reported "Purpura, Thrombocytopenic"

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1/57. Long-term treatment of refractory thrombocytopenia in a patient with wiskott-aldrich syndrome with vincristine, immunoglobulin, and methylprednisolone.

    We report a child with wiskott-aldrich syndrome with severe, refractory, symptomatic thrombocytopenia who achieved an excellent response to combination therapy with vincristine 1.5 mg/m(2) x 1 day, intravenous immunoglobulin 1 g/kg x 3 days, and methylprednisolone 25 mg/kg x 3 days (VIM) for 7 years after failing multiple treatments. He did not have a histocompatible donor for bone marrow transplantation. When the patient ceased to respond to this regimen, he was rescued with pulse dexamethasone. vincristine, immunoglobulin, and methylprednisolone might serve as a novel treatment option for the patient with refractory thrombocytopenia. Our patient had a sustained remission of symptomatic thrombocytopenia without toxicity. Furthermore, pulse dexamethasone might be an alternative treatment option to which patients with wiskott-aldrich syndrome may respond.
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2/57. Unwanted corticosteroid effects in childhood bone marrow failure, renal failure and brain damage: case report.

    The case report of the corticosteroid complication in an eight-year-old girl with immune thrombocytopenic purpura is presented. She was treated with high dosage corticosteroids and incurred severe side effects, including bone marrow depression, renal magnesium stones, osteoporosis, depression of affect, convulsions with cerebral damage and adrenal suppression.
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3/57. Thrombocytopenic purpura associated with brucellosis: report of 2 cases and literature review.

    Mild hematologic abnormalities are common in the course of human brucellosis; however, they generally resolve promptly with treatment of the disease. Occasionally, thrombocytopenia is severe and can be associated with bleeding into the skin (purpura) and from mucosal sites. We describe 2 patients infected with brucella melitensis who presented with thrombocytopenic purpura, and we review 41 additional cases from the literature. patients ranged in age from 2 to 77 years, and both sexes were affected equally. In the majority of cases, examination of the bone marrow revealed abundant megakaryocytes. Possible mechanisms involved in thrombocytopenia include hypersplenism, reactive hemophagocytosis, and immune destruction of platelets. Recognition of this complication is essential, since hemorrhage into the central nervous system is associated with a high mortality rate.
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4/57. Chronic myelomonocytic leukemia developed 2 years after the onset of immune thrombocytopenic purpura like syndrome.

    An 80-year old man was diagnosed as having immune thrombocytopenic purpura based on epistaxis, purpura and by the platelet count 8 x 10(9)/l. Prednisolone and gamma globulin were administered and the platelet count had been kept around 50 x 10(9)/l during his follow up. Two years from the onset of immune thrombocytopenic purpura he was admitted because of leukocytosis (79 x 10(9)/l with 79% monocytes), anemia and thrombocytopenia. Hypercellular bone marrow with dysplasia of three lineages was observed. In the bone marrow cytogenic analysis, a -6, clonal cytogenic abnormality was observed. 45XY, der(6), t(6;6)(q16;q23). He was diagnosed as having chronic myelomonocytic leukemia. This is a difficult case in which it was diagnosed as refractory thrombocytopenia as a subgroup of myelodysplastic syndrome, rather than immune thrombocytopenic purpura. which might have preceded the development of chronic myelomonocytic leukemia.
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5/57. Scleroderma associated with anemia and thrombocytopenia that responded well to cyclosporin.

    A 55-year-old woman came to our hospital because of cutaneous sclerosis of the limbs in September 1996, and was diagnosed with scleroderma based on a skin biopsy. In August 1997, the cutaneous sclerosis became progressive (hemoglobin level, 4.3 g/dl; platelet count, 7 x 10(9)/l). The laboratory results were positive for the direct coombs test, bone marrow aspiration showed a dry tap, and the bone marrow biopsy showed marked fatty marrow. indium-111 bone marrow scintigraphy showed a markedly decreasing uptake. These findings indicated bone marrow hypoplasia associated with hemolytic anemia. After prednisolone therapy (60 mg) was initiated, the direct coombs test became negative but the blood cell count did not increase. Then, 300 mg of cyclosporin was initiated and anemia and thrombocytopenia improved. The cyclosporin dosage was gradually decreased and the patient's hematological condition was good, although the cutaneous sclerosis changed only a little. This is a rare and interesting case of a patient with scleroderma associated with bone marrow insufficiency and hemolysis who responded well to cyclosporin.
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6/57. bone marrow examination before steroids in thrombocytopenic purpura or arthritis.

    Corticosteroids were used to treat two children with presumed idiopathic thrombocytopenic purpura and one with juvenile rheumatoid arthritis without examination of the bone marrow. Of the two with presumed idiopathic thrombocytopenic purpura, one had Fanconi's anaemia and the other may have had aplastic anaemia. The third child had acute lymphoblastic leukaemia. The diagnosis of Fanconi's anaemia was delayed. A diagnostic and therapeutic dilemma was caused in the second case. In the third, delayed diagnosis and, perhaps, compromised outlook resulted. These three cases re-emphasize the well aired caveats about the diagnosis of idiopathic thrombocytopenic purpura and juvenile rheumatoid arthritis and provide further support for the arguments of those who believe that if corticosteroids are to be used to treat such children, their bone marrow should be examined first.
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7/57. Acquired amegakaryocytic thrombocytopenia purpura and eosinophilic fasciitis: a long relapsing and remitting course.

    Acquired amegakaryocytic thrombocytopenia purpura (AATP) is a rare disorder of unclear etiology characterized by severe thrombocytopenia, preservation of erythroid and myeloid cell lines, and absence of megakaryocytes in the bone marrow. We report herein a patient who developed eosinophilic fasciitis preceding a diagnosis of AATP. Longitudinal follow-up and treatment of this individual show a relapsing and remitting disease course which appears closely related to the dosing of cyclosporine. Later in the treatment course, dosing of anti-thymocyte globulin (ATG) appeared to have important beneficial contributions in the management of this patient's disease.
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8/57. Relapse of idiopathic thrombocytopenic purpura caused by influenza a virus infection: a case report.

    We report a patient with idiopathic thrombocytopenic purpura (ITP) in remission, who relapsed as a result of an influenza a virus infection. A 41-year-old woman presented with fever elevation, coughing, and generalized petechiae. Her platelet count had decreased to 1 x 10(9)/l. She had been diagnosed with ITP at age 23, and continuous complete remission had followed steroid therapy and splenectomy. Influenza A antigen was positive in her pharyngeal aspirate, and oseltamivir was effective for her symptoms. Findings of a bone marrow smear were typical for ITP. Steroid therapy resulted in a second complete remission. Although the development of ITP caused by influenza infection and a relapse caused by an influenza vaccination have been previously described, a relapse caused by a sporadic infection has never been documented to our knowledge. physicians should carefully monitor the hematological data of influenza patients, especially those with ITP, even in remission.
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9/57. Acquired amegakaryocytic thrombocytopenic purpura.

    Acquired amegakaryocytic thrombocytopenia is an unusual hematologic disorder characterized by thrombocytopenia in association with markedly diminished bone marrow megakaryocytes. We report a case that responded to treatment with cyclosporine but not to IL-11. The bone marrow biopsy, repeated after resolution of thrombocytopenia, showed normal number of megakaryocytes.
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10/57. Immune thrombocytopenic purpura associated with pulmonary tuberculosis.

    A 22-year-old woman was admitted into our hospital because of generalized purpura and abnormalities in her chest X-ray. Isolated thrombocytopenia and elevated platelet-associated IgG levels were detected, while the bone marrow examination was normal. mycobacterium tuberculosis was detected in the bronchoalveolar lavage fluid, and consequently she was diagnosed as having active tuberculosis. High-dose immunoglobulin therapy combined with anti-tuberculosis drugs not only rapidly and continuously corrected thrombocytopenia but also cured pulmonary tuberculosis. This case suggests a causal association between immune thrombocytopenia and tuberculosis as well as the safety and efficacy of the anti-tuberculosis drugs combined with high-dose immunoglobulin therapy.
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