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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Autoimmune hemolytic anemia (AIHA) rarely occurs in myelodysplastic syndrome (MDS). A 36-year-old Asian female was diagnosed with MDS (refractory cytopenia with multilineage dysplasia, RCMD) and complicated by AIHA 7 months later. Secondary myelofibrosis developed at the same time. Steroid therapy was ineffective and cyclosporin A (CsA) was discontinued due to its neurotoxicity with the development of leukoencephalopathy. However, the patient achieved a good hematological response after the use of mycophenolate mofetil (MMF, CellCept) with a dose of 1 g/day and prednisolone (15 mg/day). Prednisolone was tapered off over the next 3 weeks. The patient did not require any blood support 4 weeks after the use of MMF and has been hematologically stable for 4 months. To our knowledge, this is the first report of using MMF in treating MDS complicated by AIHA. MMF might be considered as a salvage therapy for patients with refractory anemia complicated by AIHA.
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PMID:Myelodysplastic syndrome complicated by autoimmune hemolytic anemia: remission of refractory anemia following mycophenolate mofetil. 1248 69

In order to investigate the clinical characteristics of hematological abnormality in patients with systemic lupus erythematosus (SLE) and inquire into the basis for differential diagnosis, the hematological data of 92 cases with lupus erythematosus-related hematological disorder (SLERHD) were retrospectively analyzed by use of SPSS/PC software. The results showed that these patients were short of specificity in clinical manifestation and hemogram, however, all cases possessed multiple SLE-related autoantibodies, increase of serum globulin level and varying extent dermal and arthral signs. The incidence of primary or initial symptom in the 92 cases was as follow: 65 anemia (72.8%), 39 purpura (42.4%), 17 hemolytic anemia (18.5%), 56 leukopenia (60.9%), 54 thrombocytopenia (58.7%), and 41 pancytopenia (44.6%). The bone marrow examinations showed that the cellularity of nucleated cells was mostly normal, and active proliferation in 57 cases (61.9%) and hypercellularity in 35 cases (38.1%); the G/E ratio was normal in majority, and G/E ratio > 3 in 59 cases (64.1%) and < 3 in 33 cases (35.9%) and G/E < 1 in 17 cases with hemolytic anemia Coombs' test positive; megakaryocyte counts were normal in 11 cases (11.9%), increase in 80 cases (86.9%) and lower than 7/marrow smear in 1 case (1.1%). Neutrophil alkaline phosphatase staining was negative in all of the cases. From above data it is concluded that patients with SLERHD are varied in clinical and blood pictures, but all patients are provided with multiple SLE-related autoantibodies, globulinemia and dermal and arthral signs. It is easy to identify SLERHD from aplastic anemia, myelodysplastic syndrome, idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia and Evans' syndrome by comprehensive and detailed clinical and laboratory examinations.
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PMID:[Clinical features of hematological abnormality in systemic lupus erythematosus-related hematological disorders]. 1251 74

The myelodysplastic syndromes (MDS) comprise a heterogeneous group of clonal hematopoietic stem cell disorders, while, immunological abnormalities are frequently observed in patients with MDS. Several reports revealed that about 10% of MDS patients have clinical autoimmune disorders like skin vasculitis, rheumatic disease, or autoimmune hemolytic anemia. Furthermore, serological immunological abnormalities like hyper- or hypogammaglobulinemia, positivities of antinuclear antibody, positivities of direct Coombs test, or inverted CD4/8 ratios were found in 18-65% of patients with MDS. Recently immunosuppressive therapies including prednisolone, antithymocyte globulin, and cyclosporin A (CsA) are used to treat cytopenia in some patients with MDS. We examined the efficacy of CsA in 50 patients with MDS. Hematologic improvement was observed in 30 (60%) patients especially for erythroid lineage. There were significantly more responders with good karyotype or DRB1*1501 than with intermediate/poor karyotypes or with other HLA types. MDS with erythroid hypoplasia is a rare form of MDS, and has not yet been clearly defined. We reported four patients with MDS with erythroid hypoplasia who had morphological evidence of myelodysplasia and low percentage of erythroid precursors. Rearrangements of the TCR-beta and -gamma genes were seen in these patients using Southern blot and PCR analysis. Also they had skewed TCR usages using TCR repertoire analysis. Their anemia drastically improved with CsA therapy. We have to establish the clinical usefulness of immunosuppressive therapy in MDS patients and simple tools for revealing T-cell mediated myelosuppression in the individual patients for decision-making.
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PMID:Immunosuppressive treatments for myelodysplastic syndromes. 1276 35

Autoimmune diseases occurring concurrently with myelodysplastic syndrome (MDS) with deletion del(5q) including band q31 are very rare and have only been reported twice in the medical literature. We present two additional cases, one patient with del(5q) and trisomy 21 who suffered from rheumatoid arthritis and one patient with isolated del(5q) and autoimmune hemolytic anemia. Both patients had mild leukopenia and severe transfusion-dependent anemia. The rheumatoid arthritis was treated with antirheumatics without additional immunosuppressive medication. Autoimmune hemolytic anemia was controlled with long-term steroid administration. This patient developed additional trisomy 21, 2 years after the initial diagnosis of del(5q). Contrary to previous reports on autoimmune disorders in MDS mentioning improvements of hematological function in response to steroid administration, neither of our patients had a hematological improvement under corticosteroids.
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PMID:Autoimmune disorders in two patients with myelodysplastic syndrome and 5q deletion. 1580 95

A 65-year-old Caucasian man with myelodysplasia was admitted with autoimmune hemolytic anemia and a Hb of 5.6 g/dL. The patient's serum contained anti-K; the DAT on the patient's RBCs reacted 3+ with anti-IgG and 3+ with anti-C3d. K- RBC units were transfused, but there was no sustained increase in Hb level. The samples were referred to the reference laboratory of the National Blood Service. The DAT results remained the same, with anti-K detected only in the serum. An eluate prepared from the patient's DAT-positive RBCs revealed anti-Kp(b) specificity. This study reports an unusual case of autoanti-Kp(b), which is different from previously published cases in that no free anti-Kp(b) was detectable in the serum.
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PMID:Autoimmune hemolytic anemia and a further example of autoanti-Kpb. 1617 69

Eosinophils function primarily as secretory cells and phagocytosis by eosinophils is rarely seen. We describe a case of chronic eosinophilic leukemia (CEL) in a 72-year-old male with a history of previously treated non-Hodgkin's lymphoma (NHL) presenting with erythrophagocytosis by eosinophils and an associated autoimmune hemolytic anemia (AIHA). This patient did not show evidence of relapsed NHL. The patient's blood showed a markedly elevated eosinophil count of 16 x 10(9)/L [normal 0-0.45 x 10(9)/L] on a background of myelodysplasia and features of AIHA. Prominent erythrophagocytosis by eosinophils was visualized in the blood and in the bone marrow. Numerous Charcot-Leyden crystals were also seen in the bone marrow amid increased numbers of eosinophils and the presence of dysplastic granulopoiesis. AIHA is rarely described in the setting of CEL. More significantly, this represents the first case report to describe erythrophagocytosis by eosinophils.
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PMID:Chronic eosinophilic leukemia presenting with autoimmune hemolytic anemia and erythrophagocytosis by eosinophils. 1668 Jul 37

Leukemic patients of different classifications are associated with anemia. Such clinical conditions are often referred to as refractory anemia, paraoxymal nocturnal hemoglobinuria, hemolytic uremia and autoimmune hemolytic anemia, all of which could be categorized as the cancer cachexia. In the present work, we have studied the overall morphology of intact red cells in different leukemic patients along with patients of hypoplastic anemia (HPA) by scanning electron microscopy. We have also studied the ultrastructure of the red cell surface membranes by transmission electron microscopy. For all experiments, erythrocytes from normal individuals served as controls. We have shown direct evidence of the altered red cell (RBC) membrane morphology irrespective of the hemoglobin status of the patients which includes (1) presence of large central holes in RBCs of acute myeloid leukemia (AML), (2) presence of thorn- and horn-like structure in RBCs of acute lymphoblastic leukemia (ALL) and chronic myeloid leukemia (CML) and (3) flaccid appearance of RBCs in chronic lymphocytic leukemia (CLL) patients. A mixture of the above mentioned structures were found in the red cells of patients suffering from myelodysplastic syndrome (MDS) and in case of patients of HPA the RBCs lost the normal biconcave structures. TEM studies revealed presence of pores with diameters ranging from 100 to 200nm on the RBC membrane surface of myeloid leukemia with AML being the most prominent among others. Such pathophysiological alterations of the RBC morphology in leukemic patients could be identified as characteristic signature of the onset of anemia associated with the disease.
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PMID:Red cell morphology in leukemia, hypoplastic anemia and myelodysplastic syndrome. 1687 91

Anemia is a frequent extraenteric complication of inflammatory bowel disease (IBD, Crohn's disease and ulcerative colitis). A systematic review of the literature shows that the overall prevalence of anemia ranges from 8.8% to 73.7% but differs whether in a setting of Crohn's disease or ulcerative colitis. A disabling complication of IBD, anemia worsens the patient's general condition and quality of life, and increases hospitalization rates. Different factors, including vitamin B12 and folic acid deficiency, bone marrow suppression secondary to drug therapy, autoimmune hemolytic anemia and the coexistence of myelodysplastic syndromes are involved in the pathogenesis of anemia in IBD. The main types of anemia in IBD are iron deficiency anemia and anemia accompanying chronic diseases. Correct diagnostic definition of anemia is a fundamental step in guiding the choice of therapeutic options, since the co-presence of different pathogenetic factors may sometimes require a more complex treatment plan. A review of anemia in IBD, its pathogenetic features, epidemiology, diagnosis and therapy based on evidence from recent studies is the focus of this article.
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PMID:Anemia in inflammatory bowel disease. 1697 72

Several associations between hematological malignancies and autoimmunity directed against hematopoietic cells exist. Antibody mediated elimination of mature blood cells such as autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) are frequent complications of non-Hodgkin lymphomas, most prominently chronic lymphocytic leukemia. Autoimmunity directed against hematopoietic precursor cells is the hallmark of aplastic anemia, but many features of this disease are shared by two related disorders, paroxysmal nocturnal hemoglobinuria (PNH) and myelodysplastic syndrome (MDS). While the clinical associations between hematological malignancy and autoimmunity have been described many decades ago, only in the last several years have the common pathogenetic mechanisms been elucidated. We summarize the recent progress made in understanding how hematological malignancy gives rise to autoimmunity directed against blood cells and vice versa, and illustrate parallels in the etiology of malignant and autoimmune hematological disorders. Specifically, recent progress in the recognition of the association of lymphoproliferative disorders and autoimmunity against mature blood cells, and common pathogenetic background of aplastic anemia, paroxysmal nocturnal hemoglobinuria, and myelodysplastic syndrome are discussed.
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PMID:Autoimmunity and malignancy in hematology--more than an association. 1739 77

Hemolytic anemia and pure red cell aplasia (PRCA) after allogeneic hematopoietic stem cell transplantation (HSCT) have been reported to be mainly related to ABO-incompatibility between donor and recipient. Autoimmune hemolytic anemia (AIHA) without ABO-incompatibility has been also reported after allogeneic HSCT, especially with T-cell depletion. However, optimal management of AIHA or PRCA remains unclear. A 54-year-old male with myelodysplastic syndrome (MDS) underwent haploidentical human leukocyte antigen-mismatched HSCT using in vivo alemtuzumab and developed AIHA and PRCA simultaneously 15 months after transplantation, following the administration of cidofovir and probenecid for persistent cytomegalovirus (CMV) antigenemia and retinitis. AIHA was successfully treated with rituximab, and subsequently PRCA with cyclosporine without relapse of MDS or recurrence of CMV infection. The clinical course suggested that AIHA was mainly caused by humoral immune response, while PRCA was mainly caused by cell-mediated immune response in this patient, although these immune responses might be related to each other.
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PMID:Late onset of autoimmune hemolytic anemia and pure red cell aplasia after allogeneic hematopoietic stem cell transplantation using in vivo alemtuzumab. 1791 51


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