The Experts below are selected from a list of 237 Experts worldwide ranked by ideXlab platform
Makoto Hirokawa - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and management of acquired pure red cell aplasia
Hematology-oncology Clinics of North America, 2009Co-Authors: Kenichi Sawada, Makoto Hirokawa, Naohito FujishimaAbstract:Pure red cell aplasia is a syndrome characterized by a severe normocytic anemia, Reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Although the causes and natural course of this syndrome are variable and although the anemia in some patients can be managed by treatment of an underlying inflammatory or neoplastic disease, the pathogenesis of a large number of cases is autoimmune, including those associated with thymoma, and are best managed with immunosuppressive therapy.
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acquired pure red cell aplasia updated review of treatment
British Journal of Haematology, 2008Co-Authors: Kenichi Sawada, Naohito Fujishima, Makoto HirokawaAbstract:Pure red cell aplasia (PRCA) is a syndrome characterized by a severe normocytic anaemia, Reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Primary PRCA, or secondary PRCA which has not responded to treatment of the underlying disease, is treated as an immunologically-mediated disease. Although vigorous immunosuppressive treatments induce and maintain remissions in a majority of patients, they carry an increased risk of serious complications. Corticosteroids were used in the treatment of PRCA and this has been considered the treatment of first choice although relapse is not uncommon. Cyclosporine A (CsA) has become established as one of the leading drugs for treatment of PRCA. However, common concerns have been the number of patients treated with CsA who achieve sustained remissions and the number that relapse. This article reviews the current status of CsA therapy and compares it to other treatments for diverse PRCAs.
Kenichi Sawada - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and management of acquired pure red cell aplasia
Hematology-oncology Clinics of North America, 2009Co-Authors: Kenichi Sawada, Makoto Hirokawa, Naohito FujishimaAbstract:Pure red cell aplasia is a syndrome characterized by a severe normocytic anemia, Reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Although the causes and natural course of this syndrome are variable and although the anemia in some patients can be managed by treatment of an underlying inflammatory or neoplastic disease, the pathogenesis of a large number of cases is autoimmune, including those associated with thymoma, and are best managed with immunosuppressive therapy.
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acquired pure red cell aplasia updated review of treatment
British Journal of Haematology, 2008Co-Authors: Kenichi Sawada, Naohito Fujishima, Makoto HirokawaAbstract:Pure red cell aplasia (PRCA) is a syndrome characterized by a severe normocytic anaemia, Reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Primary PRCA, or secondary PRCA which has not responded to treatment of the underlying disease, is treated as an immunologically-mediated disease. Although vigorous immunosuppressive treatments induce and maintain remissions in a majority of patients, they carry an increased risk of serious complications. Corticosteroids were used in the treatment of PRCA and this has been considered the treatment of first choice although relapse is not uncommon. Cyclosporine A (CsA) has become established as one of the leading drugs for treatment of PRCA. However, common concerns have been the number of patients treated with CsA who achieve sustained remissions and the number that relapse. This article reviews the current status of CsA therapy and compares it to other treatments for diverse PRCAs.
Naohito Fujishima - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and management of acquired pure red cell aplasia
Hematology-oncology Clinics of North America, 2009Co-Authors: Kenichi Sawada, Makoto Hirokawa, Naohito FujishimaAbstract:Pure red cell aplasia is a syndrome characterized by a severe normocytic anemia, Reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Although the causes and natural course of this syndrome are variable and although the anemia in some patients can be managed by treatment of an underlying inflammatory or neoplastic disease, the pathogenesis of a large number of cases is autoimmune, including those associated with thymoma, and are best managed with immunosuppressive therapy.
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acquired pure red cell aplasia updated review of treatment
British Journal of Haematology, 2008Co-Authors: Kenichi Sawada, Naohito Fujishima, Makoto HirokawaAbstract:Pure red cell aplasia (PRCA) is a syndrome characterized by a severe normocytic anaemia, Reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Primary PRCA, or secondary PRCA which has not responded to treatment of the underlying disease, is treated as an immunologically-mediated disease. Although vigorous immunosuppressive treatments induce and maintain remissions in a majority of patients, they carry an increased risk of serious complications. Corticosteroids were used in the treatment of PRCA and this has been considered the treatment of first choice although relapse is not uncommon. Cyclosporine A (CsA) has become established as one of the leading drugs for treatment of PRCA. However, common concerns have been the number of patients treated with CsA who achieve sustained remissions and the number that relapse. This article reviews the current status of CsA therapy and compares it to other treatments for diverse PRCAs.
Elizabeth S Allen - One of the best experts on this subject based on the ideXlab platform.
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how we evaluate red blood cell compatibility and transfusion support for patients with sickle cell disease undergoing hematopoietic progenitor cell transplantation
Transfusion, 2018Co-Authors: Elizabeth S Allen, Randin Nelson, Willy A FlegelAbstract:Multiple hematopoietic progenitor cell (HPC) transplantation options for patients with sickle cell disease (SCD) are currently under investigation. Patients with SCD have a high rate of alloimmunization to red blood cell antigens, often complicating transfusion support. Transfusion reactions, including acute and delayed hemolytic reactions, have been observed despite immunosuppressive regimens. Allogeneic donor transplants have been shown to carry a risk of prolonged Reticulocytopenia and acute hemolysis with severe anemia in nonmyeloablative regimens. We discuss our experience providing transfusion support to patients with SCD undergoing HPC transplantation, propose an outline for a complete pretransplantation evaluation, and discuss donor/recipient compatibility issues and their implications.
James B Bussel - One of the best experts on this subject based on the ideXlab platform.
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suppression of erythropoiesis by intrauterine transfusions in hemolytic disease of the newborn use of erythropoietein to treat the late anemia
The Journal of Pediatrics, 1993Co-Authors: Andromachi Scaradavou, Steven Inglish, Powers Peterson, Julie Dunne, Frank A Chervenak, James B BusselAbstract:Hemolytic disease of the fetusand newborn has three phases of anemia: in utero, in the first week of life, and the weeks and months after birth. Intrauterine transfusions can amellorate the severity of both fetal and early anemia, but late anemia and the need for transfusion remain significnt problems. Bone marrow hypoplasia-probably a result of suppression of erythropoiesis from the intrauterine transfusions-was documeted in the three patients tested in our study. Because erythropoietin (EPO) levels have been found to be low (i.e., normal) in these previously transfused patients despite the degree of anemia, we treated four affected infants with EPO, 200 μl/kg subcutaneosuly three (times a week, and noted reticulocytosis and increased hemoglobin values 2 to 4 weeks later. One patient again had reticulocytopenic anemia when the EPO therapy was stopped but responded to retreatment. Our study indicate that EPO treatment maybe effective in the management of late anemia and could help to decrease the need for postnatal transfusions.
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suppression of erythropoiesis by intrauterine transfusions in hemolytic disease of the newborn use of erythropoietein to treat the late anemia
The Journal of Pediatrics, 1993Co-Authors: Andromachi Scaradavou, Steven Inglish, Powers Peterson, Julie Dunne, Frank A Chervenak, James B BusselAbstract:Hemolytic disease of the fetus and newborn has three phases of anemia: in utero, in the first week of life, and in the weeks and months after birth. Intrauterine transfusions can ameliorate the severity of both fetal and early anemia, but late anemia and the need for transfusion remain significant problems. Bone marrow hypoplasia--probably a result of suppression of erythropoiesis from the intrauterine transfusions--was documented in the three patients tested in our study. Because erythropoietin (EPO) levels have been found to be low (i.e., normal) in these previously transfused patients despite the degree of anemia, we treated four affected infants with EPO, 200 microliters/kg subcutaneously three times a week, and noted reticulocytosis and increased hemoglobin values 2 to 4 weeks later. One patient again had reticulocytopenic anemia when the EPO therapy was stopped but responded to retreatment. Our study indicates that EPO treatment may be effective in the management of late anemia and could help to decrease the need for postnatal transfusions.