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)

Four cases of malignant histiocytosis with leukemic manifestations and chronic course were reported. Light microscopic, ultrastructural and ultrastructural cytochemical details of these atypical cells were demonstrated. Ultrastructurally these cells resembled hairy cells most closely among the known varieties of leukemic cells. However, ribosome-lamella complexes were not found and some atypical cells had a few short cytoplasmic projections. In addition, tartrate-resistant acid phosphatase was absent from these cells. We speculate that this leukemic reticuloendotheliosis with a chronic course seen in Japan seems to be analogous to malignant histiocytosis with massive splenomegaly reported by Vardiman et al.
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PMID:Malignant histiocytosis with chronic course. Ultrastructural and ultrastructural cytochemical studies. 14 14

We have studied ten patients with a lymphoproliferative disorder characterized by massive splenomegaly; minimal lymphadenopathy; varying degrees of blood cytopenias; circulating atypical lymphoid cells frequently with "hairy" cytoplasm; monoclonal serum paraprotein; and tartrate-resistant acid phosphatase reactivity in the tumor cells of five patients tested. Although the clinical and laboratory features in most cases prompted a clinical diagnosis of "hairy cell leukemia" (HCL), histologic, ultrastructural and immunohistologic studies of multiple organs revealed distinctive features recognizably different from leukemic reticuloendotheliosis (LRE). Because this B lymphocyte proliferation may be mistaken for LRE in cases where careful histologic study is not performed, it may be responsible in part for the conflicting data in attempts to characterize the cell of origin of the latter disease. Clinical and experimental data in HCL must be questioned if they do not include histopathologic confirmation of the diagnosis.
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PMID:Malignant lymphoma simulating leukemic reticuloendotheliosis: a clinicopathologic study of ten cases. 36 71

A 65-year-old woman had pancytopenia, splenomegaly, and an inaspirable bone marrow. Diagnostic evaluation demonstrated that she had both leukemic reticuloendotheliosis (LRE), or hairy cell leukemia, and an additional lympho-reticular neoplasm, most likely a "histiocytic" lymphoma. The diagnosis of LRE was based on the histopathology of spleen tissue and of a bone marrow biopsy specimen. The diagnosis of diffuse "histiocytic" lymphoma was based on the histopathology of a splenic hilar and a mesenteric lymph node, tumor nodules in the kidney and spleen, and tissue from a mass obstructing a ureter. This is the first well-documented association of a second lympho-reticular neoplasm with LRE. Even relatively gently treatment of the "histiocytic" lymphoma resulted in fatal pancytopenia, illustrating the restricitons on therapy imposed by the marrow impairment due to the LRE.
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PMID:Coexistence of leukemic reticuloendotheliosis and histiocytic lymphoma: a case report. 37

Enzymaticaly homogeneous fractions of lymphocytes, monocytes, and neutrophils were isolated by zonal centrifugation from peripheral blood of a patient with hairy cell leukemia, or leukemic reticuloendotheliosis, LRE,(with leukopenia, neutropenia, lymphocytosis, and massive splenomegaly). To detect enzymatic deficiencies, the cells were analyzed quantitatively for six leukocytic enzymes on three occasions: 1) before splenectomy, 2) 5 days after splenectomy, and 3) 6 weeks after splenectomy. Before splenectomy, the patient's cells showed moderate deficiency of beta-glucuronidase in lymphocytes and monocytes; server to modorate deficiency of lysozyme and myeloperoxidase in monocytes and granulocytes; and complete absence of neutral protease and alkaline phosphates in neutrophils. Full restoration of neutral protease and a three-fold rise in alkaline phosphatase activities occurred in the patient's neutrophils 5 days after splenectomy. Lysozyme and myeloperoxidase returned to normal in both monocytes and neutrophils of the patient. Six weeks following splenectomy, the alkaline phosphatase activity again disappeared from patient's neutrophils, although neutral protease remained normal. The patient's lymphocytes were unresponsive to PHA and PW mitogen before splenectomy but became responsive 6 weeks postoperatively. Monocytic transfomation into macrophges was supressed before and after splenectomy. The findings indicate that developmenally, in lymphocytic leukemia, a biochemical defect involves the patient's monocytes and neutrophils much more severely than it affects the leukemic lymphocytes. Functionally, the results partly explain the susceptibility of LRE patients to microbial infections.
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PMID:Absence of neutral protease and alkaline phosphatase in neutrophils of a case of hairy cell leukemia. 43 13

The experience of the University of Colorado Medical Center affiliated hospitals with leukemic reticuloendotheliosis, hairy cell leukemia, during the past two years has been reviewed. Eight instances were found. The majority of patients in this study presented with fatigue, pancytopenia and splenomegaly. Diagnosis was based upon finding characteristic hairy cells in the blood, bone marrow or spleen. Treatment, unlike other hematopoietic malignant conditions, was primarily surgical, with splenectomy being the treatment of choice, which may lead to prolonged remission in the majority of instances.
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PMID:Surgical leukemia. 68 93

The splenic red cell volume has been measured directly by an isotope method with quantitative scanning in 10 patients with leukaemic reticuloendotheliosis (hairy cell leukaemia). The volume ranged between 211 and 726 ml (mean 410 ml, SD 158) and this constituted 15--48% (mean 28.1%, SD 9.5) of the total circulating red cell volume. This is an exceptionally large pool when compared with that found in myeloproliferative and lymphoproliferative disorders with the same degree of splenomegaly. It is consistent with the histological features which show marked red cell accumulation in the splenic cord areas. The red cell pooling in the spleen thus appears to be a significant factor in the anaemia and there was fairly good correlation between the percentage of improvement in the anaemia and the percentage of red cell volume contained in the spleen. By direct measurement of the splenic red cell pool, it is possible to predict the extent to which splenectomy will benefit the anaemia and this may also provide an indirect measure of the extent of bone marrow dysfunction in the causation of the anaemia.
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PMID:Splenic red cell pooling in hairy cell leukaemia. 87 2

A case of reticuloendotheliosis in a 7-year-old Gordon Setter is reported. Progressive weakness, debility, and diarrhea with melena were the presenting complaints. Clinical evaluation revealed splenomegaly, severe nonregenerative anemia, thrombocytopenia and leukopenia with many large blast cells in the peripheral blood. Necropsy revealed extensive involvement of bone marrow, spleen, liver, lymph nodes, kidneys, and adrenals. Gastric and duodenal ulcers were also present. The course, clinical findings and cellular characteristics of the case presented, closely resemble those reported in the syndrome of reticuloendotheliosis in cats.
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PMID:Reticuloendotheliosis in a dog. 126 Dec 71

A 63-year-old man presented with fever, easy bruisability, splenomegaly and pancytopenia. Bone marrow aspiration was unsuccessful, and marrow biopsy revealed crowding by sheets of mononuclear cells; a diagnosis of leukemic reticuloendotheliosis (LRE) was made and the patient underwent splenectomy. There was no hematologic improvement, and the patient continued to have a significant requirement for erythrocytes and platelet transfusions. Within two months of beginning oxymetholone therapy (50 mg orally three times a day) the patient's platelet count had normalized, followed by improved erythrocyte and leukocyte counts. When the drug was discontinued, the peripheral blood counts deteriorated drastically; he again demonstrated hematologic improvement when oxymetholone therapy was reinstated. We feel that by demonstrating a hematologic response to oxymetholone, relapse when it was withdrawn, and another remission upon readministration, that we have provided stronger evidence than previously reported for the efficacy of this drug in LRE.
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PMID:A case of leukemic reticuloendotheliosis responding to oxymetholone. 709 84

A patient with leukemic reticuloendotheliosis had splenomegaly, neutropenia, and a severe underproduction anemia. During a three-year period, the hematocrit was never in the normal range, and periodic transfusions were required. However, after an episode of hepatitis that was positive for B surface antigen, the spleen became smaller, the number of neutrophils increased, the transfusion requirement disappeared, and the hematocrit rose to normal. Several mechanisms for this observation are proposed.
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PMID:Beneficial effect of hepatitis in leukemic reticuloendotheliosis. 724 95

Hairy cell leukaemia, previously known as leukaemic reticuloendotheliosis, is an indolent lymphoproliferative disorder of unknown etiology. It typically affects males, causes marked splenomegaly and moderate enlargement of the liver, whilst lymphadenopathy is inconspicuous. Pancytopenia is characteristic with unusually profound monocytopenia, variable reduction in platelets, and the presence in the peripheral blood and marrow of abnormal small lymphocytes having irregular cytoplasmic margins. Ultrastructure, combined with cytochemistry and flow cytometry, have refined diagnosis. A variant exists between this classical entity and B prolymphocytic leukaemia, where blastic transformation or massive lymph node enlargement are found, and this is of ominous significance. In all these patients with this entity conventional chemotherapy is ineffective and shortens survival. Our previous experience with splenectomy results in excellent clinical control for long periods of time, but without disease eradication. There followed a vogue for the use of interferon but this is limited by high cost and dose-dependent side-effects. Contemporary management centres on the purine analogues, where durable responses are possible with fludarabine and deoxycoformycin, but best with 2'chlorodeoxyadenosine (2-CDA). To document the efficacy of the latter agent, we analysed the outcome in seventeen consecutive patients treated over the last five years. Four were ineligible for analysis, although two had 2-CDA. The other thirteen, managed on a standard seven-day course of 0.1 mg/kg 2-CDA given as a continuous intravenous infusion, all responded promptly. Apart from transient leucopenia complications have been minimal, and oral co-trimoxazole prophylaxis for pneumocystis carinii was maintained during the first one year. In all thirteen there was a rapid return to normal of peripheral blood count and marrow on aspiration and trephine biopsy. Even in the longest follow-up clinical and haematologic remission has been maintained and no patients have required retreatment. One individual has relapsed in the marrow at two years. Despite the relative expense of the agent the excellent treatment outcome and patient acceptability, coupled with its safety, leads to the recommendation that in South Africa - as elsewhere in the world - this be regarded as the first line of treatment.
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PMID:Malignancy: 2'-Chlorodeoxyadenosine Effectively Induces Complete Remission in Hairy Cell Leukaemia. 1139 82


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