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1/421. Pigmented purpura-like eruption as cutaneous sign of mycosis fungoides with autoimmune purpura.

    We describe the clinical and laboratory findings of a young man with mycosis fungoides. The disease was associated, since the early stages, with autoimmune purpura. Interferon alfa (IFN-alpha) administration improved this patient's condition, both the purpuric eruption and patchy cutaneous lesions, thus suggesting T-cell abnormalities may be responsible for the development of the disease. ( info)

2/421. Simultaneous onset of alopecia areata and idiopathic thrombocytopenic purpura: A potential association?

    alopecia areata (AA) has been associated with a variety of autoimmune diseases. Immune thrombocytopenic purpura (ITP) is an autoimmune disease marked by isolated thrombocytopenia. We present a young girl with the simultaneous onset of AA and ITP. To the best of our knowledge, this association has not previously been reported. We hypothesize that ITP may be one of many autoimmune diseases associated with AA via antibodies that cross-react between platelet membrane glycoproteins and follicular glycoproteins. ( info)

3/421. Idiopathic thrombocytopenic purpura after a living-related liver transplantation.

    BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is a rare complication after liver transplantation. We describe three cases of ITP in pediatric patients after a living-related liver transplantation (LRLT). methods: Of 266 patients who underwent an LRLT between June 1990 and June 1996, severe thrombocytopenia developed in three pediatric patients after transplantation, and ITP was also diagnosed. The original disease was biliary atresia in all cases, and the patients were given a partial liver graft from a living-related mother and subsequently treated with tacrolimus and low-dose steroids as an immunosuppressive regimen. RESULTS: The duration until the onset of ITP after transplantation in the three cases was 1 day, 3 months, and 13 months, respectively. The platelet-associated IgG levels increased in all cases. A preceding viral infection was suspected in two of the three cases. All patients were treated with intravenous gamma globulin with a transient recovery of thrombocytopenia in two cases and a sustained recovery in another. CONCLUSIONS: Transplant clinicians need to be aware of the possibility of ITP complication because a sudden onset of severe thrombocytopenia can occur even in patients who are apparently doing well after undergoing an LRLT. ( info)

4/421. Remission of idiopathic thrombocytopenic purpura after femoral lengthening. Clinical case followed for 5 years.

    We report on a patient with chronic idiopathic thrombocytopenic purpura (ITP) who went into remission after femoral lengthening. Although it is possible that spontaneous remission (frequency 5%-10%) of chronic ITP coincided with the femoral lengthening, limb lengthening could also have caused the thrombocytosis. This case suggests a close relationship between osteogenesis and hematopoiesis during regenerate bone formation. Limb lengthening can therefore be defined as the formation not only of bone and soft tissue but also of hematopoietic tissue. ( info)

5/421. Primary percutaneous transluminal coronary angioplasty performed for acute myocardial infarction in a patient with idiopathic thrombocytopenic purpura.

    A 72-year-old female with idiopathic thrombocytopenic purpura (ITP) complained of severe chest pain. electrocardiography showed ST-segment depression and negative T wave in I, aVL and V4-6. Following a diagnosis of acute myocardial infarction (AMI), urgent coronary angiography revealed 99% organic stenosis with delayed flow in the proximal segment and 50% in the middle segment of the left anterior descending artery (LAD). Subsequently, percutaneous transluminal coronary angioplasty (PTCA) for the stenosis in the proximal LAD was performed. In the coronary care unit, her blood pressure dropped. Hematomas around the puncture sites were observed and the platelet count was 28,000/mm3. After transfusion, electrocardiography revealed ST-segment elevation in I, aVL and V1-6. Urgent recatheterization disclosed total occlusion in the middle segment of the LAD. Subsequently, PTCA was performed successfully. Then, intravenous immunoglobulin increased the platelet count and the bleeding tendency disappeared. A case of AMI with ITP is rare. The present case suggests that primary PTCA can be a useful therapeutic strategy, but careful attention must be paid to hemostasis and to managing the platelet count. ( info)

6/421. Further evidence that exacerbation of ulcerative colitis causes the onset of immune thrombocytopenia: a clinical case.

    Ulcerative colitis associated with immune-mediated thrombocytopenia is rare. It has been suggested that antigenic mimicry between platelet surface antigen and bacterial glycoprotein plays a role in this association. We present a case in which exacerbation of UC sequentially induced development of ITP associated with elevation of PAIgG. In the case, two episodes of ITP occurred, with each preceded by exacerbation of UC. After remission of UC, ITP remitted and PAIgG simultaneously decreased. In the first episode, the onset of ITP was about 1 month after the exacerbation of UC. However, in the second episode, the onset of ITP was much faster, 11 days after that of UC, and the magnitude of elevation of PAIgG was much higher in the second episode. This may provide further evidence that ITP is causally associated with UC, and is the result of immunostimulation from luminal antigens and altered immunoregulation. ( info)

7/421. Perioperative management of a patient with Werlhof disease undergoing myocardial revascularization.

    We herein report the case of a patient with idiopathic thrombocytopenic purpura (Werlhof disease) and coronary artery disease undergoing myocardial revascularization. The use of monomeric immunoglobulins, corticosteroids, platelets transfusion, use of a cell saver, normothermic cardiopulmonary bypass, aprotinine and homologous blood transfusion were combined in order to minimize the risk of bleeding complications in the postoperative period. ( info)

8/421. Idiopathic thrombocytopenia after cytomegalovirus infection in a renal transplant recipient.

    infection with cytomegalovirus (CMV) is a frequent complication of organ transplantation and presents a spectrum of disease ranging from asymptomatic viremia to life-threatening tissue-invasive disease. CMV is also lymphotrophic, with the potential to induce autoimmune disease, although immunosuppressive therapy may prevent or attenuate the clinical course in transplant patients. We report a case of idiopathic thrombocytopenic purpura occurring in a renal transplant recipient after primary CMV infection and discuss the possible mechanisms involved. ( info)

9/421. aortic valve replacement in a patient with idiopathic thrombocytopenic purpura.

    A patient with aortic regurgitation and idiopathic thrombocytopenic purpura underwent a successful valve replacement. Cardiac surgery requiring a cardiopulmonary bypass in idiopathic thrombocytopenic purpura can be safely carried out with the preoperative intravenous administration of high-dose gammaglobulin, which may thereby reduce the need for either perioperative transfusion or prophylactic splenectomy. ( info)

10/421. Giant platelet disorders in African-American children misdiagnosed as idiopathic thrombocytopenic purpura.

    A retrospective chart review of six African-American children with a diagnosis of macrothrombocytopenias (MTCP) was performed to evaluate the accuracy of their diagnosis. The following was diagnosed in the six children with MTCP: Fechtner syndrome (two children), Sebastian syndrome (one child), and unnamed MTCP (three children). In five families, chronic idiopathic thrombocytopenic purpura (ITP) was diagnosed in the propositus, which resulted in therapy using steroids, intravenous immunoglobulin (IVIG), and in one case splenectomy. Bleeding symptoms were generally mild. All six patients had thrombocytopenia ranging from 10 to 125 x 10(9)/L with mean platelet volume of 8 to 20 fL. Bleeding times were abnormal in two of three patients, and platelet aggregation was abnormal in three of four patients tested. bone marrow aspirates were reported as increased megakaryocytes in the three patients on whom the procedure was performed. Ultrastructural morphology of platelets and leukocytes was performed in all six patients demonstrating giant platelets in all six patients and leukocyte inclusions in three patients. Differentiating MTCP from the more common ITP can be difficult but important in avoiding unnecessary diagnostic studies and potentially harmful therapy associated with ITP. ( info)
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