1/50. Immune thrombocytopenia after umbilical cord progenitor cell transplant: response to vincristine.An 8-month-old male with X-linked lymphoproliferative disease underwent an unrelated, partially matched (with major mismatch at DR locus), cord blood stem cell transplant. Four months following the transplant, he developed immune thrombocytopenia with hemolytic anemia (Evans syndrome). He received multiple courses of intravenous immunoglobulin, anti-Rh D immunoglobulin, a pulse of high-dose corticosteroids and cyclosporine with some improvement of hemolytic anemia, but no improvement of the thrombocytopenia. Addition of vincristine, resulted in long-term resolution of thrombocytopenia and anemia. No major toxicity was observed during treatment. vincristine should be considered as a treatment for refractory immune thrombocytopenia after hematopoietic stem cell transplantation.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
2/50. Autoimmune thrombocytopenia: a complication of fludarabine therapy in lymphoproliferative disorders.We describe two cases of autoimmune thrombocytopenia precipitated by fludarabine therapy in patients with chronic lymphatic leukaemia. Both were treated with high dose steroids and initially responded with recovery of normal platelet counts. One patient developed recurrent autoimmune thrombocytopenia on two occasions following re-exposure to the drug when his disease had become refractory to all other treatments. A retrospective review of the case notes of 45 patients with lymphoproliferative disorders treated with fludarabine over the past 6 years indicated the development of autoimmune thrombocytopenia in 4.5% (two out of 45) and autoimmune haemolytic anaemia in 6.7% (three of the 45).- - - - - - - - - - ranking = 0.098359764166686keywords = anaemia (Clic here for more details about this article) |
3/50. Salmonella septic arthritis in a patient with acute idiopathic thrombocytopenic purpura treated with steroid.Salmonella has three clinical presentations: self-limiting gastroenteritis, a systemic syndrome (enteric or typhoid fever), and bacteremia with focal infection. Hematogenous infections can cause focal lesions, but unusual manifestations occur more often when predisposing factors such as T cell defect, hemolytic disorders (sickle cell disease, malaria) or trauma are present. Salmonella tend to invade bones and joints. There is no mention of acute idiopathic (immune) thrombocytopenic purpura as a predisposing factor for salmonella septic arthritis; however there are reports about the importance of platelets for the immune response. Here we present a case of salmonella enteritidis septic arthritis following acute idiopathic (immune) thrombocytopenic purpura in a 15-year-old female patient who has been on steroid therapy for the last two weeks.- - - - - - - - - - ranking = 0.5keywords = hemolytic (Clic here for more details about this article) |
4/50. Can drugs cause autoimmune thrombocytopenic purpura?A wide range of medications can cause life-threatening immune thrombocytopenia (ITP), hemolytic anemia, or neutropenia in sensitive individuals. The antibodies associated with these conditions usually require soluble drug to be present in order to react with the cell membrane glycoproteins for which they are specific. However, some patients make drug-independent antibodies (autoantibodies) as well. Occasionally, only autoantibodies are produced following exposure to a drug. Although drugs and other small molecules can become conjugated to proteins in vivo, which may induce an immune response, only fragmentary information is available to explain how exogenous substances sometimes perturb the immune system in such a way that antibodies capable of causing immune cytopenia are produced. Platelets are affected by drug-induced antibodies more often than any other blood element. For many drug-induced thrombocytopenias, the targeted membrane glycoproteins are readily accessible for laboratory investigation and methods for detecting the responsible antibodies are well developed. Techniques for studying cellular aspects of the immune response induced by drugs through in vitro manipulation of T and B lymphocytes are also advancing rapidly. Studies of drug-induced ITP may provide clues to the general mechanisms whereby drugs and other xenobiotics induce immune diseases. Clinicians should consider the possibility of an exogenous trigger in patients who present with apparent autoimmune thrombocytopenia.- - - - - - - - - - ranking = 0.5keywords = hemolytic (Clic here for more details about this article) |
5/50. Fatal lymphoproliferative disease as a complication of Evans syndrome.A 9-month-old boy had bruising and petechiae. Investigation revealed a Coombs-positive hemolytic anemia and immune-mediated thrombocytopenia. The infant was treated with intravenous immunoglobulin and steroids. The infant eventually had recurrent fevers, hepatosplenomegaly, pulmonary nodules, and parenchymal central nervous system (CNS) lesions develop. Results of a lung biopsy revealed a polyclonal lymphoproliferative disease. polymerase chain reaction analysis showed the presence of the Epstein-Barr (EB) viral genome in the lung nodules. The infant died from progressive lung disease 6 months after the initial symptoms of Evans syndrome. Lymphoproliferative disease is known to occur in a variety of settings after immunosuppression, especially in solid organ transplant recipients. We report a case of polyclonal lymphocyte proliferation in a patient with Evans syndrome.- - - - - - - - - - ranking = 0.5keywords = hemolytic (Clic here for more details about this article) |
6/50. Immune cytopenias as the presenting finding in primary sjogren's syndrome.A diagnostic delay of several years in primary sjogren's syndrome is common, even in patients who present with sicca symptoms. It is much more likely in cases with prominent symptomatic extraglandular involvement. We report on three such patients who presented as Coomb's positive haemolytic anaemia, systemic symptoms with agranulocytosis and gingival bleeding due to immune thrombocytopenia, to alert clinicians to the fact that primary sjogren's syndrome may present as clinically significant immune-mediated cytopenia in the absence of sicca symptoms. sjogren's syndrome, a common autoimmune disorder, should be considered in the differential diagnosis of apparently 'idiopathic' cytopenias and actively sought by directed history, Schirmer test and autoantibody screening.- - - - - - - - - - ranking = 0.098359764166686keywords = anaemia (Clic here for more details about this article) |
7/50. An immunological syndrome featuring transverse myelitis, Evans syndrome and pulmonary infiltrates after unrelated bone marrow transplant in a patient with severe aplastic anemia.A patient with severe aplastic anemia underwent a matched unrelated bone marrow transplant, following which he developed a complex autoimmune syndrome. This featured transverse myelitis, immune mediated Coombs positive hemolytic anemia and immune thrombocytopenia (Evans syndrome), pulmonary infiltrates, eosinophilia, muscle pains and cramps and lichenoid dermatitis all of which may represent manifestations of graft-versus-host disease as they showed response to immunosuppression. Thus, although immune-mediated cytopenias after an allogeneic bone marrow transplant are rare, they should be considered as a possible cause of cytopenia in post-transplant patients.- - - - - - - - - - ranking = 0.5keywords = hemolytic (Clic here for more details about this article) |
8/50. thrombocytopenia in systemic-onset juvenile chronic arthritis: report of two cases with unusual bone marrow features.Two children with systemic-onset juvenile chronic arthritis (S-JCA) suffering from severe, symptomatic thrombocytopenia are reported. Case 1 is a 10-year-old girl who had fever, chronic polyarthritis and hepatosplenomegaly for 6 months and severe gum bleeding and generalised petechiae for 1 week. Immune thrombocytopenic purpura (ITP) was indicated by bone marrow findings of megakaryocytic hyperplasia, and her thrombocytopenia responded well to steroid therapy without recurrence, but progressive, deforming polyarthritis with anaemia and thrombocytosis persisted. Case 2 is a 7-year-old girl who presented with spiking fever, arthritis and skin rashes and was suspected of having S-JCA. She developed a severe nosebleed with impending shock while receiving full-dose aspirin. pancytopenia and elevated liver enzymes were also noted. Bone marrow aspiration revealed erythroid hyperplasia, maturation arrest of myeloid series with increased blasts, and adequate megakaryocytes. Her thrombocytopenia resolved in 1 week without specific treatment. Her subsequent clinical course confirmed the diagnosis of S-JCA, while thrombocytopenia did not recur during follow-up.- - - - - - - - - - ranking = 0.098359764166686keywords = anaemia (Clic here for more details about this article) |
9/50. Induction of remission with intravenous immunoglobulin and cyclophosphamide in steroid-resistant Evans' syndrome associated with dermatomyositis.Evans' syndrome is characterised by the simultaneous or sequential occurrence of Coombs'-positive haemolytic anaemia (AIHA) and immune thrombocytopenia without underlying aetiology. It has been found to be associated with collagen vascular diseases, especially systemic lupus erythematosus (SLE) and scleroderma. However, Evans' syndrome with dermatomyositis is very rare. A 59-year-old woman, who had been taking high-dose prednisolone for a month and cyclosporin for 10 days for dermatomyositis, developed purpura on the left popliteal fossa. The platelet and haemoglobin levels decreased to 77,000/mm3 and 9.8 g/dl, respectively. Antiplatelet antibody was positive. thrombocytopenia responded to intravenous immunoglobulin (IVIG) for a short time, but further decreased in a week. Her blood film showed features of haemolytic anaemia. Laboratory findings showed reticulocytosis and a positive direct Coombs' test. bone marrow examination showed a mild hyperplasia of erythroid precursors and megakaryocytes. The patient was successfully treated with cyclophosphamide in addition to oral prednisolone. AIHA in connective tissue disease may develop gradually and show a benign clinical course in most patients. Therefore, we suggest that patients with dermatomyositis and anaemia should always be checked for haemolysis if there is no other explanation.- - - - - - - - - - ranking = 0.29507929250006keywords = anaemia (Clic here for more details about this article) |
10/50. Thrombotic thrombocytopenic purpura presenting as bilateral flank pain and hematuria: a case report.Thrombotic thrombocytopenic purpura (TTP) is a rare disease whose incidence is now increasing. We present a case of a 37-year-old man who presented with bilateral flank pain and hematuria, subsequently diagnosed with TTP. Thrombotic thrombocytopenic purpura has classically been characterized by the pentad of fever, microangiopathic hemolytic anemia, neurologic symptoms, renal dysfunction, and thrombocytopenia. The pathogenesis of the disease has been a mystery until recently. We review the current literature regarding the pathophysiology and management of this disorder. Our discussion focuses on the importance of understanding this disease while considering the differential diagnosis of a patient presenting with anemia and thrombocytopenia because the common pitfall of rapidly administering platelets to a patient with TTP may lead to a disastrous outcome.- - - - - - - - - - ranking = 0.5keywords = hemolytic (Clic here for more details about this article) |
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