Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cytokines, by definition, exert an effect on haematopoiesis. Diseases characterized by haematopoietic insufficiency, such as aplastic anaemia, should therefore be investigated for abnormal expression of these regulatory proteins. In studies on hairy cell leukaemia, a severe deficiency was found in the production of interleukin-3 (IL-3), granulocyte-macrophage colony stimulating factor (GM-CSF), granulocyte CSF, IL-6 and tumour necrosis factor alpha (TNF alpha). Further studies on IL-6 at the mRNA and protein levels revealed that peripheral blood mononuclear cells and even hairy cells could be stimulated by interferon alpha (IFN alpha) to produce IL-6. It is interesting to speculate on the beneficial effects of IFN alpha therapy on the expansion of normal haematopoiesis and suppression or even elimination of malignant cells. Studies on a patient with angio-immunoblastic lymphadenopathy, another disease showing haematopoietic insufficiency, who developed severe aplastic anaemia, showed massive increases in IFN gamma and TNF alpha levels in serum; IL-6 and GM-CSF levels were below the limit of detection. These results correlated with an abnormal distribution of CD4+ and CD8+ T lymphocytes in the patient's blood and were compatible with the suppressive effects of IFN gamma and TNF alpha on haematopoiesis.
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PMID:The role of cytokines in haematopoiesis. 898 45

Late clonal complications of aplastic anemia (AA) such as acute leukemia, myelodysplastic syndromes or paroxysmal nocturnal hemoglobinuria have been recognized for a long time. To our knowledge, chronic myelogenous leukemia (CML) as a late complication of severe aplastic anemia has as yet not been reported. We report here a case of AA treated successfully with antilymphocytic globulin and cyclosporin in whom Ph1 negative, BCR/ABL negative CML developed 8 years after diagnosis of AA. This case of atypical, secondary CML was refractory to treatment with interferon alpha and hydroxyurea.
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PMID:Atypical chronic myelogenous leukemia following immunosuppressive therapy for severe aplastic anemia. 1051 77

To determine the clinical characteristics and outcome of patients with chronic hepatitis C virus (HCV) infection presenting severe autoimmune cytopenia unrelated to interferon alpha therapy, we analyzed characteristics and outcomes of 35 patients with HCV (16 from our departments and 19 from the literature). We considered active autoimmune hemolytic anemia (AHA) as a decrease of at least 2 g/dL in hemoglobin levels, an increase of at least 0.6 mg/dL in the serum unconjugated bilirubin level, a reticulocyte count >5%, and a positive direct Coombs test. Severe neutropenia was defined as a neutrophil count <0.5 x 10(9)/L, and severe thrombocytopenia as a platelet count <30 x 10(9)/L. We identified the following cytopenias: AHA (17 cases), severe thrombocytopenia (16 cases), aplastic anemia (2 cases), severe neutropenia (1 case), refractory sideroblastic anemia (1 case), and pure red cell aplasia (1 case). Three patients simultaneously presented 2 types of severe cytopenias. Twenty-seven patients (77%) were female and 8 (23%) male, with a mean age at diagnosis of cytopenia of 51.7 years (range, 18-84 yr). Immunologic markers were detected in 19 (68%) of 28 patients, the most frequent being hypocomplementemia in 16 (57%), cryoglobulins in 15 (54%), antinuclear antibodies in 12 (43%), and rheumatoid factor in 5 (18%). Other associated processes were autoimmune diseases in 14 (50%) of 28 and human immunodeficiency virus (HIV) coinfection in 3 (9%) of 32. We found clinical and immunologic differences between HCV patients with AHA and those with severe thrombocytopenia. Patients with HCV-related AHA showed a higher prevalence of associated autoimmune diseases (71%), cryoglobulins (67%), and cirrhosis (59%). All had a good response to corticosteroids, but a poor prognosis (47% mortality). In contrast, patients with HCV-related severe thrombocytopenia had a lower prevalence of associated autoimmune diseases (11%), a poorer response to corticosteroids (55%), and lower mortality (6%), with HIV/HBV coinfections in some patients. The 35 cases presented demonstrate that different types of immune-mediated cytopenias may be severe and clinically significant in patients with HCV infection. Hemolytic anemia and severe thrombocytopenia were the most frequent cytopenias observed. Most patients responded well to corticosteroids, although a higher rate of mortality was observed in those with liver cirrhosis.
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PMID:Severe autoimmune cytopenias in treatment-naive hepatitis C virus infection: clinical description of 35 cases. 1264 Jan 85

We present history of 16-year-old boy, HBsAg carrier, treated with interferon alpha at the age of 6 because of hepatitis B (HBeAg/antyHBe seroconversion). In August 2002--admitted to Department of Pediatric Oncology due to pancytopenia--diagnosis of severe aplastic anaemia was made (bone marrow cellularity--10%). We found no relative donor for hematopoietic cells transplantation and started immunosuppresive therapy (ATG, G-CSF, methyloprednisolon, cyclosporin). Haematologic parameters were improving. At day +60 he was admitted to our Department due to the increase in aminotransferases and cyclosporin activity. He was treated with cefuroxim, acyclovir and drugs improving liver cell function, cyclosporin was stopped. Presence of HBV DNA in serum confirmed HBV reactivation--a boy received lamivudine and cyclosporin again (as a maintenance therapy of aplastic anaemia). Aminotransferase activity and haematological parameters returned to normal. This case indicates the possibility of HBV reactivation in the course of immunosuppressive therapy (e.g. after antithymocytic globulin and cyclosporin) for aplastic anaemia.
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PMID:[Immunotherapy in aplastic anaemia as a cause of reactivation of hepatitis B virus-immunologic aspects]. 1659 88