1/164. factor v Leiden and antibodies against phospholipids and protein S in a young woman with recurrent thromboses and abortion.We describe the case of a 39-year-old woman who suffered two iliofemoral venous thromboses, a cerebral ischemic infarct and recurrent fetal loss. Initial studies showed high levels of antiphospholipid antibodies (APAs) and a moderate thrombocytopenia. After her second miscarriage, laboratory diagnosis revealed that the woman was heterozygous for the factor v Leiden mutation and had a functional protein s deficiency as well as anti-protein S and anti-beta 2-glycoprotein i antibodies. The impairment of the protein c pathway at various points could well explain the recurrent thromboses in the patient and supports the role of a disturbed protein c system in the pathophysiology of thrombosis in patients with APAs.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
2/164. An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome.The autoimmune lymphoproliferative syndrome (ALPS) affords novel insights into the mechanisms that regulate lymphocyte homeostasis and underlie the development of autoimmunity. This syndrome arises early in childhood in persons who inherit mutations in genes that mediate apoptosis, or programmed cell death. The timely deletion of lymphocytes is a way to prevent their accumulation and the persistence of cells that can react against the body's own antigens. In ALPS, defective lymphocyte apoptosis permits chronic, nonmalignant adenopathy and splenomegaly; the survival of normally uncommon "double-negative" CD3 CD4- CD8- T cells; and the development of autoimmune disease. Most cases of ALPS involve heterozygous mutations in the lymphocyte surface protein Fas that impair a major apoptotic pathway. Detailed immunologic investigations of the cellular and cytokine profiles in ALPS show a prominent skewing toward a T-helper 2 phenotype; this provides a rational explanation for the humoral autoimmunity typical of patients with ALPS. Prospective evaluations of 26 patients and their families show an ever-expanding spectrum of ALPS and its major complications: hypersplenism, autoimmune hemolytic anemia, thrombocytopenia, and neutropenia. Defective apoptosis may also contribute to a heightened risk for lymphoma.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
3/164. A fatal case of autoimmune thrombocytopenia with an IgM anti-GPIb/IX following one antigen mismatched unrelated donor bone marrow transplantation.We report the case of a 32-year-old patient with ALL who developed autoimmune thrombocytopenia 2 months following allogeneic bone marrow transplantation. An IgM autoantibody against the platelet glycoprotein Ib/IX complex was observed. Treatment with high-dose steroids and intravenous immunoglobulin g failed to produce any benefit and the thrombocytopenia led to fatal gastrointestinal haemorrhage. The possible factors contributing to post-allograft thrombocytopenia and potential management strategies are discussed.- - - - - - - - - - ranking = 7keywords = thrombocytopenia (Clic here for more details about this article) |
4/164. Unusual clinical presentation in a patient with myelodysplastic syndrome, with subsequent hematological remission and suppression of the malignant clone following treatment with cyclosporine A, erythropoietin and granulocyte colony-stimulating factor.A 35-year-old female presented with isolated thrombocytopenia of autoimmune origin. One and a half years later, hypoplastic myelodysplastic (MDS) was diagnosed. Following treatment with cyclosporin A, erythropoietin and granulocyte colony-stimulating factor, the patient has achieved a sustained hematological remission which is still ongoing after 3 years. Furthermore, to the best of our knowledge, this is the third case described in the literature where treatment with cytokines alone or in combination with immunosuppressive agents has resulted in a long standing cytogenetic response in MDS.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
5/164. Multicentric warfarin-induced skin necrosis complicating heparin-induced thrombocytopenia.Two patients developed catastrophic multicentric skin necrosis while receiving warfarin to treat venous thromboembolism complicated by immune-mediated heparin-induced thrombocytopenia (HIT). Patient 1 developed skin necrosis involving the breasts, thighs, and face, as well as venous limb gangrene and bilateral hemorrhagic necrosis of the adrenal glands, resulting in death. The second patient developed bilateral mammary necrosis necessitating mastectomies, as well as skin necrosis involving the thigh. Neither patient had an identifiable hypercoagulable syndrome, other than HIT. HIT may represent a risk factor for the development of multicentric warfarin-induced skin necrosis (WISN).- - - - - - - - - - ranking = 5keywords = thrombocytopenia (Clic here for more details about this article) |
6/164. Pernicious anemia, gastric carcinoid, and autoimmune thrombocytopenia in a young woman.The association between gastric carcinoid tumors and pernicious anemia is well recognized. Such tumors occur in the presence of achlorhydria, chronic atrophic gastritis, hypergastrinemia, and enterochromaffin-like cell hyperplasia. In this case report, a 29-year-old woman with pernicious anemia and autoimmune thrombocytopenia who developed gastric carcinoid tumors of the gastric body is described. This is the second description of pernicious anemia associated with autoimmune thrombocytopenia. This association in a young woman together with the therapeutic options and decisions that were taken in the treatment of the patient are discussed.- - - - - - - - - - ranking = 6keywords = thrombocytopenia (Clic here for more details about this article) |
7/164. Anti-CD20 chimeric monoclonal antibody treatment of refractory immune-mediated thrombocytopenia in a patient with chronic graft-versus-host disease.BACKGROUND: Autoimmune thrombocytopenia in chronic graft-versus-host disease may represent an instance of B-cell dysregulation leading to clinical disease. OBJECTIVE: To attempt to treat refractory immune-mediated thrombocytopenia in a patient with chronic graft-versus-host disease by using anti-CD20 chimeric monoclonal antibody. DESIGN: Case report. SETTING: Academic medical center. PATIENT: A patient with chronic graft-versus-host disease after allogeneic peripheral blood stem-cell transplantation who had severe refractory immune-mediated thrombocytopenia. INTERVENTION: Weekly infusion of rituximab, 375 mg/m2, for 4 weeks. MEASUREMENTS: platelet count, CD3 cell count, and CD19 cell count. RESULTS: Rituximab therapy resulted in marked depletion of B cells in the peripheral blood and decreased levels of platelet-associated antibody. The increase in platelet count persisted despite tapering and discontinuation of immunosuppressive therapy for chronic graft-versus-host disease. CONCLUSION: The efficacy of rituximab for the treatment of immune-mediated thrombocytopenia suggests that this drug may have activity in other autoimmune diseases or chronic graft-versus-host disease.- - - - - - - - - - ranking = 8keywords = thrombocytopenia (Clic here for more details about this article) |
8/164. Difficulties in the management of an incomplete form of refractory thrombotic thrombocytopenic purpura, the usefulness of vincristine.BACKGROUND: Although several etiologies can be identified in thrombotic thrombocytopenic purpura (TTP), idiopathic cases are still frequent. Incomplete forms are more rare. Currently, the diagnosis may be made in cases of thrombocytopenia and microangiopathic hemolytic anemia. According to the literature, mortality and morbidity are significantly improved with plasma exchange. However, treatment in refractory forms remains problematic. CASE REPORT: A 33-year old woman presented with an incomplete form of TTP, refractory to a combination of therapeutics. The patient underwent plasma infusion, plasma exchange, and then was started on corticosteroids. She also received intravenous immunoglobulins and antiplatelet agents in close proximity to vincristine (Oncovin) infusion. The main biological indicators used were the platelet count, hematocrit, LDH, and the presence of schistocytes. Following vincristine treatment, the patient's condition rapidly improved. CONCLUSION: vincristine administered after the failure of standard therapeutics was effective in this refractory form of TTP.- - - - - - - - - - ranking = 1keywords = thrombocytopenia (Clic here for more details about this article) |
9/164. Chronic autoimmune thrombopenia/neutropenia in a boy with persistent parvovirus B19 infection.OBJECTIVE: We report an 11-year-old boy presenting with splenomegaly, chronic thrombocytopenia and concordant neutropenia. RESULTS: In contrast to autoantibodies against platelets, there were no detectable neutrophil-specific autoantibodies present in this patient. Extensive serologic investigations revealed increased IgM- and IgG-antibody titers against parvovirus B19. A nested polymerase chain reaction (PCR) showed parvovirus B19-specific sequences in the patient's bone-marrow cells but not in the serum. Specific antibodies against the structural proteins VP1 and VP2 in addition to those against non-structural protein NS1 of parvovirus B19 were detected by Western blot analysis. thrombocytopenia and neutropenia responded to immunosuppressive therapy and subsequent splenectomy, the latter being necessary due to severe side-effects of steroid medication. CONCLUSION: Autoimmune thrombocytopenia/neutropenia may have been triggered and/or sustained by a chronic parvovirus B19 infection. patients with this very rare disorder should be screened for this virus.- - - - - - - - - - ranking = 2keywords = thrombocytopenia (Clic here for more details about this article) |
10/164. Successful treatment of thrombocytopenia and hemolytic anemia with IvIG in a patient with lupus-like syndrome after mismatched related PBSCT.hematopoietic stem cell transplantation (HSCT) is a treatment option for autoimmune diseases but can also cause clinical features similar to those of autoimmune diseases. In some of these cases the autoimmune-like condition is associated with autoimmune cytopenia, a complication that can be unresponsive to established treatment strategies and which may be fatal. The majority of cases reported on immune hemolytic anemia have been of alloimmune origin due to ABO red blood cell antigen incompatibilities between donor and recipient. We now report a patient with a lupus-like syndrome, presenting with severe thrombocytopenia and hemolytic anemia 9 months after HLA-mismatch, ABO compatible-related PBSCT who experienced no response to high-dose steroids, but who had a sustained response to repeated IvIG therapy.- - - - - - - - - - ranking = 5.9172170076364keywords = thrombocytopenia, alloimmune (Clic here for more details about this article) |
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