1/37. Immune thrombocytopenia and hemolytic anemia as a presenting manifestation of hodgkin disease.A very unusual clinical presentation of hodgkin disease with immune thrombocytopenia and autoimmune hemolytic anemia is reported. A 6.5-year-old boy presented with thrombocytopenia, Coombs' positive hemolytic anemia, and multiple small posterior cervical lymph nodes. After a course of high-dose methylprednisolone therapy with a diagnosis of Evans syndrome, complete response for thrombocytopenia and partial response for anemia was achieved. Six weeks later there was a sudden increase in the size of left posterior cervical lymph nodes and a biopsy was compatible with hodgkin disease, mixed cellularity type. The child was successfully treated with chemotherapy and radiation therapy. He has been off therapy for 28 months and has no clinical or laboratory evidence of autoimmune cytopenia. A combination of immune thrombocytopenia and autoimmune hemolytic anemia may be associated with hodgkin disease. The recognition of this clinical picture as a complication of hodgkin disease has important implications. This complication appeares to be managed best by the definitive treatment of hodgkin disease.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
2/37. Crescentic glomerulonephritis with positive antineutrophil cytoplasmic autoantibody specific for myeloperoxidase associated with autoimmune hemolytic anemia and thrombocytopenic purpura.A 41-year-old woman was admitted to the hospital with severe uremia, hemolytic anemia, and thrombocytopenic purpura. Emergency hemodialysis with plasmapheresis was started in view of consideration of hemolytic uremic syndrome (HUS), which resulted in improvement of renal function and platelet count. Positive antineutrophil cytoplasmic autoantibody specific for myeloperoxidase (MPO-ANCA) suggested crescentic glomerulonephritis, which was pathologically evidenced by renal biopsy. The diagnosis of MPO-ANCA associated crescentic glomerulonephritis with autoimmune hemolytic anemia (AIHA) and thrombocytopenic purpura were confirmed. Three courses of steroid pulse therapy with heparin were successfully performed, followed by oral prednisolone and warfarin. Such a case has not been previously reported to our knowledge.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
3/37. 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.- - - - - - - - - - ranking = 0.14285714285714keywords = hemolytic (Clic here for more details about this article) |
4/37. Membranous nephropathy associated with chronic immune thrombocytopenic purpura in childhood.Membranous nephropathy has been associated with many autoimmune diseases. We describe a child with membranous nephropathy associated with chronic immune thrombocytopenic purpura (ITP) and Coombs'-positive hemolytic anemia. After 3 years of ITP, the patient developed nephrotic syndrome during a flare of ITP. A biopsy specimen showed membranous nephropathy. Treatment with corticosteroids led to improvement of the thrombocytopenia and resolution of the proteinuria. Two years later, the patient again developed thrombocytopenia and proteinuria. Both conditions resolved after treatment with corticosteroids. This case suggests that ITP can cause membranous nephropathy.- - - - - - - - - - ranking = 0.14285714285714keywords = hemolytic (Clic here for more details about this article) |
5/37. Thrombocytopenic purpura and cardiomyopathy in pregnancy reversed by combined plasma exchange and infusion.thrombocytopenia and hemolytic anemia have been seen with thrombotic thrombocytopenic purpura (TTP), hellp syndrome (hemolysis, elevated liver enzymes, and low platelets), and hemolytic uremic syndrome (HUS). Differentiating between TTP, HUS, and hellp syndrome is often difficult. Coexistence of TTP and HELLP is possible. Cardiomyopathy occurring in pregnancy can be idiopathic or associated with TTP. We describe a previously healthy woman who developed thrombocytopenia and hemolysis at 34 wk gestation. The patient underwent delivery after transfusion with platelets and RBCs. The suspicion of TTP was raised, and plasma exchange was begun by the third hospital day. On the seventh day of treatment, the patient developed shortness of breath, and an echocardiogram showed global hypokinesis with an ejection fraction of 25%. Plasma infusion, one unit q 4 h, was initiated in addition to the daily plasma exchange. The patient improved and her ejection fraction normalized. plasma exchange and infusion and corticosteroids were gradually tapered off. von willebrand factor (vWF) protease activity in the plasma upon transfer was completely deficient with the presence of inhibitor. This case illustrates that vWF protease assay and detection of inhibitor can be used for the diagnosis of TTP during pregnancy; and a severe cardiomyopathy in TTP can be reversed rapidly with combined plasma exchange and infusion.- - - - - - - - - - ranking = 0.28571428571429keywords = hemolytic (Clic here for more details about this article) |
6/37. plasma exchange in the treatment of immune disease.plasma exchange was investigated as an alternative to the use of toxic drugs to remove unwanted antibody. Studies in rabbits immunized with bovine serum albumin demonstrate that exchange transfusion after a primary immunization results in a rebound of antibody to above preexchange levels. However, exchange transfusion seven, 11, or 18 days after secondary immunization results in permanent lowering of antibody levels. plasma exchange with the continuous flow centrifuge was used in four patients with hematologic diseases. White cell isoantibodies were removed in a septic leukopenic patient, permitting white cell transfusions. Two patients with immune thrombocytopenia were exchanges; one showed prompt and permanent elevation of platelet count, while the other did not improve. A patient with immune hemolytic anemia had stabilization of hemoglobin levels and decreased Coombs reactivity following plasma exchange.- - - - - - - - - - ranking = 0.14285714285714keywords = hemolytic (Clic here for more details about this article) |
7/37. 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.- - - - - - - - - - ranking = 0.05096929517753keywords = anaemia (Clic here for more details about this article) |
8/37. thrombocytopenia during the course of acute poststreptococcal glomerulonephritis.A four-year old boy was admitted to the hospital due to acute thrombocytopenic purpura. Three days later he developed edema, hematuria and hypertension. The diagnosis of acute poststreptococcal glomerulonephritis was based upon the evidence of previous sore throat, hypocomplementemia and increased antistreptolysin O titer. Renal biopsy was contraindicated due to throbocytopenia. An extensive work-up was done to exclude mebranoproliferative glomerulonephritis and systemic diseases such as hemolytic uremic syndrome or systemic lupus erythematosus. The clinical outcome of the nephritis and thrombocytopenia was excellent in respect to both conditions. To the best of our knowledge concurrent occurrence of acute thrombocytopenic purpura and poststreptococcal glomerulonephritis is very rare; there are only four similar cases reported in the literature. A careful work-up and follow-up are mandatory to exclude systemic disease.- - - - - - - - - - ranking = 0.14285714285714keywords = hemolytic (Clic here for more details about this article) |
9/37. A 20-year-old woman with Hashimoto's thyroiditis and Evans' syndrome.Here we report the case of a 20-year-old female patient previously diagnosed with Hashimoto's thyroiditis and overt hypothyroidism, and who had been taking synthetic thyroxine (100 microg/day) for eight months. She experienced intermittent dizziness and generalized weakness, and was diagnosed as having severe autoimmune hemolytic anemia (AIHA). We prescribed prednisolone treatment and continued synthetic thyroxine administration. Two years and five months later, she developed idiopathic thrombocytopenic purpura (ITP) and was diagnosed with Evans' syndrome. Thereafter, laparoscopic splenectomy was performed because her autoimmune hemolytic anemia was refractory and dependent on steroid therapy. The HLA genotypes of the patient were HLA-A*020101/A* 2602, HLA-B*270502/B*5401, HLA-Cw*0102/Cw*020202, HLA-DRB1*0404/DRB1*0405, and HLA-DQB1*0302/DQ B1*0401. Hashimoto's thyroiditis is often associated with other nonendocrine autoimmune diseases, and antithyroid antibodies are frequently observed in Evans' syndrome (coexistence of AIHA and ITP). However, there is no report of Evans' syndrome developing in patients with overt hypothyroidism and Hashimoto's thyroiditis. This case suggests that three autoimmune diseases (AIHA, ITP, and Hashimoto's thyroiditis) might share a common immunogenetic pathway in pathogenesis.- - - - - - - - - - ranking = 0.28571428571429keywords = hemolytic (Clic here for more details about this article) |
10/37. OPD4-positive T-cell lymphoma of the liver in systemic lupus erythematosus.Primary malignant lymphoma of the liver occupying the right lobe, 14 x 9 x 7 cm in size, developed in a 30-year-old man with a 4-year history of autoimmune hemolytic anemia. The diagnosis of systemic lupus erythematosus (SLE) accompanying thrombocytopenia had been made clinically 10 months earlier. The liver biopsy specimen revealed diffuse proliferation of large lymphoma cells expressing the activated helper/inducer T-cell phenotype (LCA , UCHL1 , OPD4 , LN3 , MT1-, L26-, MB1-, Leu M1-, Ki-1-, KP1-). The lymphoma was successfully treated by chemotherapy and irradiation. Intractable thrombocytopenia provoked fatal esophageal hemorrhage. At autopsy, no lymphomatous lesion was identified, and the hepatic right lobe contained an encapsulated necrotic lesion without any viable tumor cells. The bone marrow revealed marked hyperplasia of erythroid and megakaryocytic series. Extramedullary hematopoiesis was demonstrated in the liver, spleen and lymph nodes. This is the second case of primary hepatic T-cell lymphoma associated with SLE.- - - - - - - - - - ranking = 0.14285714285714keywords = hemolytic (Clic here for more details about this article) |
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