Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To determine whether warm-antibody autoimmune hemolytic anemia (AIHA) responds to treatment with intravenous gammaglobulin (IVGG), we conducted separate pilot studies at three institutions enrolling a total of 37 patients. We combined these results with a review of 36 cases of AIHA treated with IVGG reported in the literature. Sixteen clinical variables were examined to determine associations with response to IVGG. Overall, 29 of 73 patients (39.7%) responded to IVGG therapy. Two variables were strongly related to a good response to IVIG: the presence of hepatomegaly (with and without splenomegaly) and a low pre-treatment hemoglobin. A trend towards a better response was observed in the 11 children. Overall, IVGG provided acute benefit in only 1/3 of patients and therefore cannot be recommended as standard therapy for AIHA. It may, however, be useful as adjunctive treatment in selected cases, such as in those with a pre-treatment hemoglobin < 6-7 gm/dl or those with hepatomegaly, and in clinical settings where the toxicity of other treatments may be an important consideration.
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PMID:Efficacy of intravenous immunoglobulin in the treatment of autoimmune hemolytic anemia: results in 73 patients. 823 93

A 70-year-old woman was admitted to our hospital in November 1992 for evaluation of anemia. Physical examination revealed anemia, jaundice, swelling of axial and inguinal lymph nodes, and splenomegaly. Abnormal hematological findings were as follows: Hb of 3.9 g/dl, reticulocyte count of 58.2% (61.7 x 10(4)/microliters), hyperplasia of normal erythroblasts in bone marrow, and eosinophilia (21.0%, 2352/microliters) in peripheral blood. Routine laboratory examinations revealed polycolonal hypergammaglobulinemia 3.0 g/dl, a high level of serum LDH (797 IU/I) and a total bilirubin of 2.4 mg/dl (indirect, 1.6 mg/dl). The serum haptoglobin level was very low (< 5 mg/dl). Results of serological examinations were as follows: IgG of 3366 mg/dl, CH50 of 16.0 U/ml, positive Coombs test 2+, and positive tests for antinuclear antibody, rheumatoid factor, and cold agglutinin. CRP was negative. PHA-stimulated lymphocyte blast formation, NK activity, and ADCC activity were found to be suppressed, and the percentage of CD4-positive lymphocytes in peripheral blood was also low. An axillary lymph node biopsy revealed reactive lymphadenitis. No signs or history suggested allergy, collagen disease, or parasitic infection. Autoimmune hemolytic anemia (AIHA) complicated by immunologic abnormalities and eosinophilia was diagnosed. Oral prednisolone markedly reduced the hemolytic anemia, eosinophilia, lymph node swelling, and splenomegaly, but NK activity remained low.
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PMID:[Autoimmune hemolytic anemia with eosinophilia in elderly patient]. 892 99

A 24 year old female with a 4 year history of anemia and absolute lymphocytosis was evaluated and found to have T cell large granular lymphocyte (T-LGL) leukemia associated with autoimmune hemolytic anemia, neutropenia, mild thrombocytopenia and splenomegaly. In an effort to ameliorate her symptomatic cytopenias, she was treated with prednisone and subsequently methotrexate without success. In February 1993, she underwent splenectomy for symptomatic anemia. Splenectomy resulted in an increased hemoglobin concentration to normal levels, resolution of all laboratory evidence of hemolysis, and disappearance of thrombocytopenia. This response has been durable despite persistence of the abnormal LGL clone. We suggest that splenectomy may be an effective treatment for autoimmune hemolytic anemia and/or thrombocytopenia often associated with T-LGL leukemia. As this disease often exhibits a chronic clinical course with morbidity resulting from consequences of resultant cytopenias rather than visceral involvement with leukemic LGL, effective treatment of cytopenias despite persistence of the abnormal LGL clone is beneficial.
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PMID:Resolution of autoimmune hemolytic anemia following splenectomy in CD3+ large granular lymphocyte leukemia. 903 Nov 24

The present report describes a case of right atrial thrombus in an active 49-year-old man with a primary antiphospholipid syndrome. In 1984, the patient was admitted for autoimmune hemolytic anemia; during the hospitalization it was diagnosed a chronic hepatitis B. In July 1991, the patient had fever, mild jaundice, splenomegaly and pancytopenia; a diagnosis of hairy cell leukemia was made but it was not subsequently confirmed. Interferon therapy was started and the patient's clinical course mildly improved. However, over the same year, he experienced again a clinical deterioration. Lumbar ischemic ulcers occurred. The patient underwent elective splenectomy. Bone marrow biopsy revealed mielodisplastic syndrome. Necrotizing vasculitis with granulomatosis was diagnosed. The patient's condition improved after splenectomy. Repeated laboratory tests showed positivity for antiphospholipid antibodies. Transthoracic and transesophageal echocardiography demonstrated the presence of a right atrial thrombus, confirmed by nuclear magnetic resonance. The patient was started on long-term anticoagulant therapy, that resulted effective in reducing thrombus size.
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PMID:[Primary antiphospholipid antibody syndrome with left atrial intracardiac thombosis]. 924 43

The autoimmune lymphoproliferative syndrome (ALPS) affords novel insights into the mechanisms that regulate lymphocyte homeostasis and underlie the development of autoimmunity. This syndrome arises early in childhood in persons who inherit mutations in genes that mediate apoptosis, or programmed cell death. The timely deletion of lymphocytes is a way to prevent their accumulation and the persistence of cells that can react against the body's own antigens. In ALPS, defective lymphocyte apoptosis permits chronic, nonmalignant adenopathy and splenomegaly; the survival of normally uncommon "double-negative" CD3+ CD4- CD8- T cells; and the development of autoimmune disease. Most cases of ALPS involve heterozygous mutations in the lymphocyte surface protein Fas that impair a major apoptotic pathway. Detailed immunologic investigations of the cellular and cytokine profiles in ALPS show a prominent skewing toward a T-helper 2 phenotype; this provides a rational explanation for the humoral autoimmunity typical of patients with ALPS. Prospective evaluations of 26 patients and their families show an ever-expanding spectrum of ALPS and its major complications: hypersplenism, autoimmune hemolytic anemia, thrombocytopenia, and neutropenia. Defective apoptosis may also contribute to a heightened risk for lymphoma.
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PMID:An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome. 1018 30

New Zealand Black (NZB) mice spontaneously produce anti-erythrocyte autoantibodies (AEA) in association with splenomegaly, thus serving as a model for autoimmune hemolytic anemia. Although these autoimmune traits are inherited as a dominant fashion, expression in F(1) hybrids of NZB and most non-New Zealand strains is suppressed due to the contribution of wild-type modifying genes present in the latter strains. Using chromosomal microsatellite markers in the (C57BL/6 x NZB)F(1) x NZB backcross progeny, we mapped C57BL/6 modifying loci for AEA production and splenomegaly. Generation of AEA was found to be down-regulated by a combined effect of two major independently segregating dominant alleles-one linked to D7MIT30 on chromosome 7 and the other linked to D10MIT42 on chromosome 10. Splenomegaly was modified mainly by a single C57BL/6 allele linked to D4MIT58 on chromosome 4. Thus, the autoimmune hemolytic anemia in the NZB strain is under multigenic control and a combined action of not only susceptibility but also modifying alleles with suppressive activities affects the outcome of disease features in the progeny. There are potentially important candidate genes which may be linked to the regulation of AEA and splenomegaly.
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PMID:Genetic regulation of anti-erythrocyte autoantibodies and splenomegaly in autoimmune hemolytic anemia-prone new zealand black mice. 1060 44

Autoimmune hemolytic anemia (AIHA) is a disorder associated with the destruction of red blood cells (RBCs) by autoantibodies. We report a rare case of AIHA in an infant rhesus macaque (Macaca mulatta) which received a continuous administration of four drugs, a dopamine agonist. dopamine receptor inhibitor, and two gamma-aminobutyric acid receptor inhibitors into the brain during the course of neurophysiological experiments. The main clinical findings were severe anemia and splenomegaly. Hematological and serological examinations revealed the appearance of peripheral erythroblasts and autoantibodies against RBCs. Medical treatments, including washed RBC transfusion and corticosteroids, transiently improved the animal's anemia, but euthanasia was decided on 331 days after the start of the experiment. The pathological findings revealed severe anemia, splenomegaly, and extramedullary hematopoiesis in the liver and kidneys. These findings and the clinical course suggest that this anemia was a warm-antibody type of AIHA induced by the administration of the drugs for the neurophysiological experiment.
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PMID:Autoimmune hemolytic anemia (AIHA) in an infant rhesus macaque (Macaca mulatta). 1095 Apr 57

Twenty-one patients of autoimmune hemolytic anemia (AIHA), aged 2 months to 57 years were analyzed. The common presenting feature was pallor (89%), fever (38%), Jaundice (43%) and hepatomegaly and splenomegaly was seen in 76% and 81% respectively. Fifteen cases were of idiopathic etiology and in 6 cases the etiology could be identified as systemic lupus erythematosus, systemic sclerosis, pregnancy, maternal AIHA, typhoid fever and myelodysplastic syndrome (one each). Hemoglobin level ranged between 1.9 to 11.7 gm/dl (mean 6.8 gm/dl) and reticulocyte counts between 6% to 42% (mean (20.2%). Four patients had thrombocytopenia. Direct antiglobulin test (DAT) was positive in 19 and indirect antiglobulin test (IAT) in 7 cases. There was no correlation between DAT positivity and severity of anemia. All patients had warm antibodies of IgG type. Ten of fourteen patients responded to steroid therapy. Patients with secondary AIHA had a significantly poorer prognosis compared to the idiopathic group.
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PMID:Clinico-hematological spectrum of auto-immune hemolytic anemia: an Indian experience. 1099 63

We report a series of 31 cases of splenic marginal zone lymphomas with an enlarged spleen and a multimicronodular macroscopic pattern. Two groups, A and B, were distinguished based on the presence (A) or absence (B) of a lymphoplasmacytic component with monoclonal immunoglobulin expression in the cytoplasm. There were no differences between the groups as far as age, sex, spleen weight, and progression. The only difference was the presence in group A of a monoclonal serum component and autoimmune disorders, particularly autoimmune hemolytic anemia. In most cases in which a liver and/or bone marrow biopsy was performed, lymphomatous infiltration was detected. Seven cases had a seric monoclonal IgM of 5 g/L or more and liver or bone marrow infiltration, corresponding to the definition of Waldenstrom's macroglobulinemia. Lymphoma cells had a monocytoid, centrocytoid and, in group A, lymphoplasmacytic morphology. The lymphomatous cells were positive for CD20, CD45 RA, and bcl-2. They expressed IgD in 9 cases, partially in 6, and were negative for IgD in 9 of the 24 cases studied. Progression seems to be slow, with a long survival. Three patients presented with transformation into a large B-cell lymphoma, which was responsible for death in two patients.
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PMID:Splenic marginal zone lymphoma with or without plasmacytic differentiation. 1111 78

The case of a 22-yr-old male who after a brief febrile episode developed autoimmune hemolytic anemia and right pulmonary infiltrate with pleural effusion is presented. Cytologic examination of the pleural fluid revealed lymphocytosis and hemophagocytosis, primarily of red blood cells (RBCs) by mature histiocytes. There was accompanying splenomegaly, laboratory evidence of hepatic dysfunction, and retroperitoneal lymphadenopathy. Besides profound reduction of red blood cells in the peripheral blood, there was reduction of lymphocytes and platelets. As a neoplastic process was ruled out by bone marrow and pleural biopsies, the disease was considered to be virus-induced and was halted and progressively regressed with early institution of vigorous antiinflammatory therapy with adrenocortical steroids. Upon reviewing the case, examination of the bone marrow biopsy disclosed limited hemophagocytosis of RBCs and lymphocytes by histiocytes and considerable viral cytopathic effect on hematopoietic cells (red and white cell precursors and megakaryocytes), which by appropriate immunolabelling was identified as induced by Epstein-Barr virus. A virus-related acquired hemophagocytic syndrome in its early stages was probably present, yet an undesirable clinical outcome was averted by early institution of vigorous steroid therapy. The need to recognize early hemophagocytic changes in cytologic specimens for early institution of appropriate therapy is emphasized. The possibility of erythrophagocytosis, also manifested during the course of an autoimmune hemolytic process and unrelated to hemophagocytic syndrome, is discussed.
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PMID:Serous fluid cytology as a means of detecting hemophagocytosis in Epstein-Barr virus-induced autoimmune hemolytic anemia. 1159 10


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