Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Cytotoxic chemotherapy often induces sustained, severe granulocytopenia in patients with leukemic reticuloendotheliosis. Many of the patients so treated subsequently develop serious infections. Poor marrow reserve has been implicated but lacks supporting evidence as the cause of the granulocytopenia. In six patients we studied with leukemic reticuloendotheliosis, bone marrow showed severe granulocytopenia, blood neutrophil response after intravenous hydrocortisone injection was poor, and leukocyte migration to the site of inflammation showed suboptimal neutrophilia and poor or no mononuclear response. Splenic hypersequestration and pooling were probably not important factors in causing neutropenia, since similar results were seen in patients without spleens. These findings suggest that in this disease the marrow granulocyte reserve and leukocyte mobilization are impaired and the neutropenia is due to poor granulocyte production and not to increased migration of leukocytes to tissues. Cytotoxic chemotherapy should be used with caution in patients with this disease.
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PMID:Impaired marrow granulocyte reserve and leukocyte mobilization in leukemic reticuloendotheliosis. 90 43

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