1/53. Successful treatment of refractory acquired pure red cell aplasia (PRCA) by allogeneic bone marrow transplantation.This case describes a 16-year-old woman treated successfully by a bone marrow transplant from her HLA-identical brother for refractory acquired pure red cell aplasia. Conditioning was as for severe aplastic anaemia with cyclophosphamide 4 x 50 mg/kg and antithymocyte globulin. Complete donor type engraftment at 3 months reversed to full autologous reconstitution at 2 years with normal haemopoiesis. The potential implications on pathogenesis of the disease as well as on treatment of autoimmune disorders by stem cell transplantation are discussed.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/53. Splenic lymphoma presenting as warm autoimmune hemolytic anemia associated with pure red cell aplasia.BACKGROUND AND OBJECTIVE: Warm autoimmune hemolytic anemia (AIHA) is a condition in which peripheral red blood cell (RBC) destruction is induced by the presence of an autoantibody. Pure red cell aplasia (PRCA) represents an isolated process of decreased erythropoiesis. The combination of both is quite rare, with a very poor prognosis. We describe a patient with isolated splenic lymphoma whose presentation was a combination of AIHA and PRCA. The patient was resistant to all treatment. MATERIALS AND methods: Erythroid colony assays were performed, in order to compare the effect of the patient's serum on colonies with that of a normal control. RESULTS: The patient's serum significantly suppressed normal erythroid colony growth. A red cell eluate revealed the presence of a warm autoantibody. CONCLUSIONS: The patient's serum contained warm autoantibody responsible for peripheral RBC destruction and a humoral factor, perhaps the warm autoantibody, which suppressed bone marrow erythropoiesis. Establishing an early diagnosis, and treatment of the underlying disease might result in a better prognosis.- - - - - - - - - - ranking = 2.2789332145687keywords = hemolytic (Clic here for more details about this article) |
3/53. Acquired pure red cell aplasia--a case report.A married female patient of 36 years with chronic anaemia, because of pure erythroid aplasia with a haemolytic component and hypothyroidism due to antithyroid auto-antibodies, was subsequently discovered as a case of systemic lupus erythematosus (SLE). She was treated with corticosteroid and immunosuppressive therapy and her anaemia was corrected. The response of erythroid aplasia to corticosteroid and other immunosuppressive agents suggests that immunological factors play a role in erythroid aplasia in SLE. The occurrence of red cell aplasia in association with a variety of immune phenomenon supports the concept that in SLE, erythroid aplasia may be of immune aetiology.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
4/53. Pure red cell aplasia caused by parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature.Human parvovirus B19 (PV B19) is one of the several recently described 'emerging viruses' and has been identified as the etiological agent of 'fifth disease' in childhood. Human PV B19, which is the etiological agent of transient erythroblastopenia in hemolytic anemia, is also a recognized rare cause of red cell aplasia in immunocompromised patients, including transplant recipients. To date, 26 cases of PV B19-induced red cell aplasia have been reported in solid organ transplant recipients. Twelve patients had cyclosporine-based immunosuppression and 14 had tacrolimus-based immunosuppression. Sixteen of these patients required treatment with commercial intravenous immunoglobulin alone, 1 required treatment with intravenous immunoglobulin and plasmapheresis, 4 required intravenous immunoglobulin and erythropoietin, 1 required treatment with intravenous immunoglobulin and conversion of tacrolimus to cyclosporine, 1 had improvement in hematocrit with erythropoietin alone and in 3 patients the disease was self-limiting. Herein, we report a case of pure red cell aplasia caused by acute PV B19 infection in a renal transplant recipient in whom the immunosuppressive regimen included prednisone, mycophenolate mofetil and tacrolimus and the red cell aplasia resolved with discontinuation of mycophenolate mofetil.- - - - - - - - - - ranking = 0.45578664291374keywords = hemolytic (Clic here for more details about this article) |
5/53. Anti-CD20 monoclonal antibody for the treatment of severe, immune-mediated, pure red cell aplasia and hemolytic anemia.Immune-mediated, acquired pure red cell aplasia (PRCA) is a rare disorder frequently associated with other autoimmune phenomena. Conventional immunosuppressive treatment is often unsatisfactory. Rituximab is a monoclonal antibody against the CD20 antigen, highly effective for in vivo B-cell depletion. An 18-month-old girl with both severe PRCA and autoimmune hemolytic anemia, refractory to immunosuppressive treatment, received 2 doses of rituximab, 375 mg/m(2) per week. The drug was well tolerated. After anti-CD20 therapy, substitutive treatment with intravenous immunoglobulin was started. The treatment resulted in marked depletion of B cells; a striking rise in reticulocyte count ensued, with increasing hemoglobin levels, finally leading to transfusion independence. The child is now 5 months off-therapy, with normal hemoglobin and reticulocyte levels. This case suggests a role of anti-CD20 monoclonal antibody for treatment of patients with antibody-mediated hematologic disorders. (blood. 2001;97:3995-3997)- - - - - - - - - - ranking = 2.2789332145687keywords = hemolytic (Clic here for more details about this article) |
6/53. Epstein-Barr virus associated diffuse large B-cell lymphoma complicated by autoimmune hemolytic anemia and pure red cell aplasia.A 53-year old man with systemic lymphadenopathy and hepatosplenomegaly was diagnosed with diffuse large B cell-lymphoma after inguinal lymph node biopsy. Anemia was noted, direct and indirect Coombs tests were positive, and the haptoglobin level was low. However, the bone marrow aspirate revealed erythroid aplasia. Co-existing autoimmune haemolytic anemia (AIHA) and pure red cell aplasia (PRCA) were diagnosed. in situ hybridization with Epstein-Barr virus (EBV) encoded small rna (EBER) showed positive findings in lymphoma cells. Southern blot hybridization revealed immunoglobulin heavy chain gene rearrangement and a clonal EBV terminal repeat, indicating monoclonal proliferation of EBV in infected B cells. The patient was treated with CHOP, resulting in a complete remission (CR). AIHA and PRCA subsided after 3 courses of chemotherapy. In conclusion, this case demonstrates not only the association of B-cell lymphoma with autoimmune disorders but also the involvement of EBV in these conditions.- - - - - - - - - - ranking = 1.823146571655keywords = hemolytic (Clic here for more details about this article) |
7/53. Successful treatment of pure red cell aplasia with rituximab in patients with chronic lymphocytic leukemia.Pure red cell aplasia (PRCA) is a rare complication in patients with chronic lymphocytic leukemia (CLL). It is characterized by reticulocytopenia and by an absence of red cell precursors in the bone marrow. Unlike autoimmune hemolytic anemia, which is characterized by an increased number of reticulocytes, positive coombs test findings, and a high serum level of lactate dehydrogenase. Two patients with B-cell CLL are reported to have developed PRCA, one while on chemotherapy with fludarabine and one seeking treatment for de novo PRCA. Both responded dramatically to therapy with monoclonal antibody rituximab (Rituxan) in a short period of time and continued to be transfusion-independent. These are the first 2 reported patients for whom rituximab treatment for PRCA in CLL was successful, and this treatment deserves further investigation.- - - - - - - - - - ranking = 0.45578664291374keywords = hemolytic (Clic here for more details about this article) |
8/53. Pure red cell aplasia and acute hepatitis during pregnancy.Pure red cell aplasia during pregnancy is rare. We present a case in a 26-year-old pregnant woman, referred to our hospital at 31 weeks' gestation because of severe anemia caused by acute hepatitis. She was treated with repeated blood transfusions and the pure red cell aplasia gradually remitted during the pregnancy. A live infant was delivered by cesarean section at 34 weeks' gestation. Postpartum, the pure red cell aplasia and hemolytic anemia remitted completely. Our case illustrates that pure red cell aplasia may occur late in pregnancy associated with acute viral hepatitis and is reversible during pregnancy without any necessity for steroid therapy.- - - - - - - - - - ranking = 0.45578664291374keywords = hemolytic (Clic here for more details about this article) |
9/53. Pure red cell aplasia associated with carbamazepine.Therapeutic indications for carbamazepine have grown in recent years and more and more patients are taking this drug for various neurological and psychiatric disorders. Though haematological complications like aplastic anaemia etc., due to carbamazepine therapy are well known pure red cell aplasia is exceedingly rare. A 33 year old man was diagnosed to have pure red cell aplasia with positive Coomb's test following 5 months of carbamazepine therapy. He recovered completely. carbamazepine induced pure red cell aplasia is a reversible condition if recognised early.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
10/53. life-threatening human parvovirus B19 infection transmitted by intravenous immune globulin.infection of human parvovirus B19 (B19) is usually a self-limiting febrile illness, but can sometimes be life-threatening under certain circumstances, such as aplastic crisis in patients with haemolytic anaemia, hydrops fetalis in pregnant women and fulminant hepatitis. B19 can be transmitted through respiratory secretions, transplacentally and by transfusion of blood or blood products. In the present case, administration of intravenous immune globulin (i.v.Ig) transmitted B19 infection and consequently caused pure red cell aplasia and aggravation of hepatitis to fulminant hepatitis. Our case may raise important questions as to the safety of i.v.Ig and possible contamination by B19.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
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