Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 65-year-old female was admitted to our hospital for evaluation of transfusion-dependent progressive anemia. She had a history of rheumatoid arthritis for twenty-four years. Two years earlier, MDS (refractory anemia) was diagnosed based on laboratory findings and bone marrow examination. After diagnosis, the patient received anabolic steroid, Vitamin D3 and Vitamin K2. Although her hemoglobin level was maintained at 8.0 g/dl approximately 9.0 g/dl until January 2009, anemia gradually progressed thereafter. In April, we recognized marked anemia (Hb 6.0 g/dl) and reticulocytopenia (2.0 per thousand), but this was not accompanied by any other significant changes in laboratory findings. Bone marrow examination demonstrated a low percentage of erythroid precursors without an increase of blast cells. Rheumatoid arthritis remained stable by low dose steroid and NSAID administration. We did not recognize evidence suggesting any other cause of acquired PRCA, such as thymoma, human parvovirus B19 infection or drugs. A diagnosis of MDS with erythroid hypoplasia was made. The patient was successfully treated by an immunosuppressive regimen using cyclosporine. MDS with erythroid hypoplasia, coexisting with rheumatoid arthritis, is rare. To our knowledge, this is the third reported case.
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PMID:[Successful treatment with cyclosporine for myelodysplastic syndrome with erythroid hypoplasia following rheumatoid arthritis]. 2062 90

Of 1059 children, 35 children with various hemato-oncologic diseases were diagnosed with parvovirus B19 infection. The clinical spectrum included 11 immunocompromised patients presenting with prolonged pancytopenia, 7 patients with delayed hematologic recovery after stem cell transplantation, 5 patients with parvovirus B19 as possible cause of severe aplastic anemia or myelodysplastic syndrome, and 12 children with hemolytic anemia and transient aplastic crisis.
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PMID:The spectrum of parvovirus b19 infection in a pediatric hemato-oncologic ward. 2150 29

Parvovirus B19 (PVB19) induced severe aplastic anaemia (SAA) or myelodysplastic syndrome (MDS) is rare, and haematopoietic stem cell transplantation (HSCT) in this condition has not been reported so far. 6 children with SAA (n=4) or MDS (n=2) caused by acute PVB19 infection underwent HSCT under the protection of intravenous immunoglobulines. The 4 children with SAA received matched HLA bone marrow from a sibling (n=3) or peripheral unrelated blood stem cells (n=1). 1 patient had delayed erythrocyte engraftment, whereas 3 patients had an uneventful transplantation course. HSCT in one of the 2 children with MDS was complicated by poor graft function, the other patient engrafted without complications. In conclusion, HSCT in children with PVB19 induced SAA or MDS is feasible, even though some patients may develop delayed engraftment or prolonged poor graft function.
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PMID:Stem cell transplantation in 6 children with parvovirus B19- induced severe aplastic anaemia or myelodysplastic syndrome. 2205 31

Cases 1 and 2 were 55- and 68-year-old males, respectively. Both were administered deferasirox (DFX) because they received red blood cell transfusions regularly as treatment for myelodysplastic syndrome refractory anemia. DFX administrations were stopped on the 22nd day in case 1 and on the 78th day in case 2 because significantly reduced hemoglobin values and reticulocyte counts were observed. Bone marrow examinations showed pure red cell aplasia in both cases. In case 1, the reticulocyte ratio recovered to the value before drug administration 21 days after drug withdrawal. In case 2, it started increasing on the 14th day, and had recovered to the value before drug administration by the 42nd day after drug withdrawal. Human parvovirus B19 infections were negative in both cases. Both cases were thought to have drug-induced pure red cell aplasia, probably due to DFX. This drug should be used carefully with regular follow-ups of the reticulocyte count.
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PMID:Development of acute pure red cell aplasia after deferasirox administration in two cases of myelodysplastic syndrome. 2485 Apr 56

Parvovirus B19 is a single-stranded DNA virus which preferentially targets the erythroblast resulting in red cell aplasia, which is temporary in immunocompetent persons. Since the discovery of B19 virus in 1975, a wide variety of blood diseases and cytopenias affecting several blood cell lineages have been documented during or following B19 infection. These include cytopenias affecting the erythroid, megakaryoblastoid, myeloid and lymphoid lineages, as well as a variety of bicytopenias, pancytopenia, bone marrow necrosis / fat embolism syndrome, myelodysplastic syndrome, leucoerythroblastopenia, and hemophagocytic lymphohistiocytosis. B19 infection may also complicate and precede the course of acute leukemia, the significance of which remains to be determined. This review describes the current state of knowledge of the abnormalities of individual blood cell lineages encountered during parvovirus B19 infection, over the almost 40 years since its discovery, and reveals some very interesting themes, which improve our understanding of the pathogenesis of B19 infection with particular reference to the bone marrow.
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PMID:A review of blood diseases and cytopenias associated with human parvovirus B19 infection. 2596 96

Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents. The therapeutic approach to PRCA typically involves immunosuppression, but specific pathogenic subtypes are associated with specific therapeutic approaches. Cyclosporine A, with or without concurrent corticosteroids, appears to be the single most effective immunosuppressive agent.
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PMID:Pure red cell aplasia. 2788 71

Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents. The therapeutic approach to PRCA typically involves immunosuppression, but specific pathogenic subtypes are associated with specific therapeutic approaches. Cyclosporine A, with or without concurrent corticosteroids, appears to be the single most effective immunosuppressive agent.
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PMID:Pure red cell aplasia. 2791 62


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