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41/123. Refractory Evans' syndrome treated with allogeneic SCT followed by DLI. Demonstration of a graft-versus-autoimmunity effect.

    Evans' syndrome, a combination of autoimmune haemolytic anaemia and autoimmune (idiopathic) thrombocytopenic purpura, is generally harder to treat and more refractory than the single entities. In a male patient with refractory disease, predominantly thrombocytopenic, an allogeneic reduced intensity BMT from his human leukocyte antigen (HLA)-identical sister was followed by a dramatic platelet peak while he was still experiencing initial engraftment (presumably of autologous origin), but subsequently by progressive relapse associated with mixed chimerism. Five gradually incremental DLI achieved complete donor chimerism, which was associated not only with grade II graft-versus-host disease (GVHD), but also with complete clinical and biological remission for 2 years post-transplant. Long-term FU is necessary before claiming that allogeneic stem cell transplantation (SCT) is capable of curing an autoimmune blood disease. However, there is evidence for a graft-versus-autoimmunity effect in this case.
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42/123. Childhood thrombocytopenia associated with graves disease is distinct from idiopathic thrombocytopenic purpura.

    The authors report a case of thrombocytopenia in a 10-year-old Chinese girl who was also found to have graves disease 6 months later. The clinical, hematologic, and bone marrow features were indistinguishable from chronic idiopathic thrombocytopenic purpura, but the patient's platelet counts were normalized concurrently with the control of thyrotoxicosis with carbimazole treatment. Childhood thrombocytopenia associated with autoimmune thyroid disease is a rarely reported condition and its management differs from that of idiopathic thrombocytopenic purpura.
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43/123. portal vein thrombosis.

    portal vein thrombosis (PVT) is a complication of hepatic disease and a potentially lethal complication of splenectomy. The reported incidence of this complication is low (approximately 1%). However, its true incidence may have been underestimated due to difficulty in making the diagnosis. Herein we report the case of a 19 year-old woman who presented with a 2-year history of idiopathic thrombocytopenic purpura (ITP). Because she had become refractory to medical therapy, she underwent laparoscopic splenectomy. She was discharged on postoperative day 2 after an uncomplicated procedure. She did well, complaining only of mild backache, until postoperative day 21, when she presented with nausea, vomiting, and leukocytosis. CT showed PVT and superior mesenteric vein thrombosis. Despite heparin and fluid administration, her condition worsened. At laparotomy, she had diffuse small bowel edema and congestion. At a second-look procedure 24 h later, nearly all her jejunum and ileum were necrotic. After three procedures, she was left with 45 cm of proximal and 10 cm of distal small bowel. Bowel continuity was restored 8 weeks later. She continued on warfarin anticoagulation therapy for 1 year. Postsplenectomy PVT is most often seen following splenectomy for myeloproliferative disorders and almost never after trauma. The large splenic vein stump and the hypercoagulable state in patients with splenomegaly are thought to be contributory. The presentation of PVT is vague, without defining signs or symptoms. color-flow Doppler and contrast-enhanced CT scans are the best methods for the nonoperative diagnosis of PVT. Aggressive thrombolysis offers the best hope for clot lysis and maintenance of bowel viability. Even vague symptoms must be considered seriously following splenectomy.
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44/123. 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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45/123. Two patients with heparin-induced thrombocytopenia followed by idiopathic (immune) thrombocytopenic purpura: case report.

    heparin-induced thrombocytopenia (HIT) and idiopathic thrombocytopenic purpura (ITP) are separate and well-recognized clinical syndromes, 10both having potential for disastrous outcomes. Sequential occurrence of these two diseases has not been reported in the literature. We report herein two patients who were initially diagnosed as having HIT but whose later clinical course was similar to ITP. Although dysfunction of immune system seems to play a role, the precise mechanisms for the development of these diseases are unclear. As both conditions have different natural histories and require different treatment modalities, it is important to recognize that these two diseases may be seen sequentially.
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46/123. Development of common variable immunodeficiency in a patient with Evans syndrome treated by autologous stem cell transplantation.

    We describe a case report of a patient who developed common variable immunodeficiency (CVID) after autologous haematopoietic stem cell transplantation (SCT) for recurrent Evans syndrome. The disease manifested as attacks of haemolytic anaemia, thrombocytopenia and neutropenia from the age of 12 years. Presence of autoantibodies to blood elements was confirmed together with C4 deficiency. The patient also suffered from dermatitis herpetiformis Duhring without signs of coeliac disease. Autologous T cell-depleted peripheral blood stem cell (PBSC) transplant following conditioning regimen was performed at the age of 20 years. Immunological reconstitution was incomplete and 2 years after SCT he fulfilled laboratory criteria for common variable immunodeficiency (CVID). The patient was found to be a carrier of a risk haplotype for development of CVID DRB1*03/DQB1*0201. We conclude that T cell-depleted SCT here performed for autoimmune manifestations can hasten development of CVID in genetically predisposed patients.
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47/123. Refractory immune thrombocytopenia in systemic lupus erythematosus: response to mycophenolate mofetil.

    Immune thrombocytopenia (IT) is a common manifestation of systemic lupus erythematosus (SLE). Although severe IT (<20 x 10(9)/L) occurs in about 5-10% of patients, usually in the context of active disease, the absence of randomized controlled trials has not allowed the development of evidence-based guidelines for managing this condition. Conventionally, high-dose glucocorticoids are considered first-line therapy. Adjunctive medical and surgical treatments for patients with an absent or partial response to glucocorticoids have met with varying degrees of success. We describe an SLE patient with IT refractory to high-dose corticosteroids, pulse methylprednisolone and intravenous immunoglobulin therapy, whose platelet counts normalized during therapy with mycophenolate mofetil (MMF). Pending further controlled studies to confirm this observation, we suggest that MMF may be considered as a therapeutic option in the treatment of glucocorticoid-refractory immune thrombocytopenia in SLE.
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48/123. cyclosporine A in the treatment of a patient with immune thrombocytopenia accompanied by myelodysplastic syndrome and nephrotic syndrome.

    We describe a complete remission with cyclosporine A in a myelodysplastic syndrome (MDS) patient who had a 9-year history of nephrotic syndrome (NS) due to autoimmune nephritis. A 72-year-old woman with MDS and NS rapidly developed thrombocytopenia with multiple spontaneous bleeding episodes and profound proteinuria. She showed persistent platelet refractoriness to platelet transfusions. A flow cytometry examination strongly detected antiplatelet autoantibodies on the surface of her platelets. The treatment with high-dose corticosteroids and intravenous immunoglobulin did not lead to complete improvement in the platelet count, bleedings and proteinuria. However, a low dose of cyclosporine A resulted in a sustained normal range of blood platelet count and negative proteinuria. This finding suggests that, in selected cases, cyclosporine A can be an attractive alternative for MDS patients who also have immune-mediated diseases.
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49/123. Familial association of autoimmune thrombocytopenia and hyperthyroidism.

    An association between thrombocytopenia and thyrotoxicosis in a single individual is well documented, and the theories for this event include a common immunologic cause or a thyrotoxic-induced decrease in platelet survival. We report the first description of the coexistence of autoimmune thrombocytopenic purpura (AITP) and Graves' disease in several members of the same family, in which four females were thrombocytopenic and two of these were also hyperthyroid. All four patients had high titers of antiplatelet antibodies, and the two hyperthyroid cases were positive for thyroid-stimulating immunoglobulins (TSI). The familial occurrence of two autoimmune disorders is very uncommon, and suggests a genetic etiology. The HLA phenotype was determined and the antigens B8 and DR3, which are reported with high frequency in both diseases, were present in three patients. Although the etiologic cause is still unknown, our findings further support the theory that a genetic predisposition underlies autoimmune disease.
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50/123. Acute and chronic immune thrombocytopenic purpura. Disorders that differ in more than duration.

    Immune thrombocytopenic purpura is a relatively common disease that occurs in both pediatric and adult populations. The acute form predominates in children and the chronic form is seen much more often in adults. The diagnosis is one of exclusion but often can be made on the basis of thorough history taking and physical examination and a limited number of laboratory studies. More intensive diagnostic studies, including bone marrow examination, may be needed to rule out other causes of thrombocytopenia. For patients in whom spontaneous remission does not occur, effective treatments are available, and morbidity and mortality rates are quite low.
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Last update: September 2014