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)

Two infants with lethargy, vomiting, convulsions, coma and marked metabolic acidosis were found to have very high concentrations of methylmalonic acid in their serum and urine. In vitro studies of fibroblasts demonstrated that the infants had different variants of methylmalonic acidemia.Vitamin B(12) was given in two different forms at 1 month of age and at 12 months of age. Each trial continued for 4 months but neither infant showed a clinical or biochemical response.In both infants hyperglycinemia, neutropenia and thrombocytopenia developed during acute metabolic crises only. Hypoglycemia was found in patient 2. Hyperammonemia was severe in patient 2 during acute crises but never appeared in patient 1. When clinically well, both infants continued to excrete abnormal amounts of methylmalonic acid in the urine and both had persistent compensated metabolic acidosis.Marked hyperuricemia developed in patient 1 at 18 months of age and led to progressive renal failure. Allopurinol therapy was necessary to keep the uric acid concentration within the normal range. Renal function returned to normal, as indicated by a marked increase in the renal clearance of creatinine and uric acid.Patient 1 is physically and mentally retarded, and has moderate hypotonia, hepatomegaly and persistent vomiting. Patient 2 has developed normally.The urine concentrations of methylmalonic acid in the four parents were normal.
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PMID:Methylmalonic acidemia: 6 years' clinical experience with two variants unresponsive to vitamin B12 therapy. 3 17

Lipopolysaccharide (LPS) isolated from Salmonella minnesota R595 or from Escherichia coli 0111:B4 induces hypotension in rhesus monkeys with normal complement levels. This hypotension is accompanied by decreased total peripheral resistance. The depletion of C3 and terminal complement components by prior intraperitoneal administration of the anticomplementary protein cobra factor did not alter the hemodynamic changes which occur following the rapid injection of 5 mg/kg of R595 LPS, the infusion of 500 microgram/kg of R595 LPS, or the injection of 500 microgram/kg of 0111:B4 LPS. We conclude that the LPS-induced hemodynamic changes in the subhuman primate are medicated by pathways which do not require the participation of C3. The kinetics and extent of the neutropenia and thrombocytopenia resulting from the injection of 0111:B4 or R595 LPS were not latered by prior depletion of greater than 95% of the plasma C3.
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PMID:The effect of complement depletion on bacterial lipopolysaccharide (LPS)-induced hemodynamic and hematologic changes in the Rhesus monkey. 9 42

Many diuretic, antiarrhythmic, and antihypertensive drugs have potential hematologic toxicity. Any patient with cardiovascular disease receiving long-term drug therapy or massive doses of a drug should be watched carefully for early detection of hemolysis, thrombocytopenia, neutropenia, or other toxic effects.
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PMID:Hematologic toxicity of drugs used in cardiovascular disease. 10 88

Among the various infectious and parasitic disease liable to produce granulopenia, the authors selected certain diseases which represented their personal experience. Classically, leukopenia may occur during typhoid fever, but was not present in all of the series of 114 adults they observed with this disease. It only occurs after antibiotic treatment (Thiamphenicol in the majority of cases). During acute brucellosis (188 cases studied) granulopenia was constant. It occurs early and is lasting. It reappears during septicemic relapse. Leukopenia is corrected during treatment by tetracycline antibiotics. Neutropenia during disease is frequent but usually labile. This characteristic explains why there was no granulopenia in 90 cases of mumps and 64 cases of chickenpox. Finally, during Kala Azar, 8 cases confirmed certain already well known data: the considerable reduction in granulocytes but also anemia and thrombocytopenia.
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PMID:[Granulopenia of infectious origin]. 17 55

Erythroblastic transformation of chronic granulocytic leukemia was found in seven of 67 unselected patients with blast crisis. This morphologic picture of erythroblastic transformation was indistinguishable from that in erythroleukemia or Di Guglielmo's syndrome. The median survival of the patients with erythroblastic transformation was two months, considerably less than the four-month median survival in the entire series of 67 patients. Only two brief partial remissions were obtained with combination chemotherapy. The causes of death were primarily hemorrhage and infection, related to thrombocytopenia and neutropenia. In this regard, the patients with erythroblastic transformation resembled all the patients with blast crisis and patients with acute leukemia in general. The erythroblastic transformation seems to represent a morphologic variant of chronic granulocytic leukemia blast crisis, without apparent prognostic or therapeutic implications.
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PMID:Erythroblastic transformation of chronic granulocytic leukemia. 26 30

Circulating T-lymphocytes from a 13-year-old boy with autoimmune anaemia, severe neutropenia and thrombocytopenia inhibited autologous and normal homologous bone marrow myeloid colony formation in vitro. This inhibition was abolished when the patient's antithymocyte globulin and complement-treated T-lymphocytes were used. T-lymphocytes from normal individuals did not cause such an inhibition. The patient's lymphocytes showed no inhibitory effect on erythroid colony formation. Investigation of the patient's serum failed to disclose any leucoagglutinin, lymphocytotoxin or humoral factor against myeloid colony formation. These findings indicate that T-lymphocytes may play a role in the pathogenesis of neutropenia in immune pancytopenia.
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PMID:Immune pancytopenia. 31 88

The clinical and laboratory features of 72 patients with Felty's syndrome described within the last ten years have been compared with Felty's five original patients. Felty's syndrome appears to be a variant of rheumatoid arthritis with extra-articular manifestations in which leukopenia (usually due to neutropenia) and splenomegaly occur, although not always at the same time. Both are manifestations of the underlying disease process and are not necessarily otherwise related. The mechanism of the leukopenia is complex and abnormalities in leukocyte function appear to be as important as the leukopenia in predisposing patients with Felty's syndrome to infection. Functional abnormalities of the leukocytes in this syndrome are due in part to immune complex formation. Hypocomplementemia associated with this process may be another cause for the increased susceptibility to infection. It is proposed, therefore, that therapy in Felty's syndrome be directed at the underlying disease process, and gold salts and penicillamine should be considered for this purpose. Splenectomy should be reserved for specific situations, such as hemolytic anemia, severe thrombocytopenia, leg ulcers, and infections associated with profound leukopenia that are not responsive to medical therapy.
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PMID:Felty's syndrome: an analytical review. 33 Sep 14

Consecutive newborn autopsy cases were divided into infected and noninfected groups on the basis of pathologic findings and cultures, and were compared to a concomitant consecutive group of neonatal survivors with proven bacterial sepsis. Newborns dying with bacterial infection often demonstrated leukopenia, neutropenia, and thrombocytopenia, usually associated with normal bone marrow cell production. Those with nonfatal sepsis frequently had neutrophilia with an increase in absolute band counts. Of infected newborns 80% showed one or more hematologic abnormalities as did 43% of newborns dying without bacterial infection. Of newborns dying with bacterial infection 13% had no hematologic abnormality. Blood cultures were negative in 18% (seven) of the infants dying with bacterial infection. Abnormalities of the white blood cell, differential and platelet counts are not invariably specific for bacterial infection nor do normal values adequately exclude it. Blood cultures may be negative in newborns dying with significant foci of bacterial infection.
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PMID:Diagnosis of neonatal bacterial infection: hematologic and pathologic findings in fatal and nonfatal cases. 45 May 62

The pattern of amphotericin B toxicity was assessed retrospectively in a group of 20 children with cancer who had received one or more courses of the drug for treatment of systemic fungal infection. Azotemia was the most frequent complication, developing during 23 of 24 treatment courses. Other major toxic effects, in decreasing order of frequency, were anemia, hypokalemia, thrombocytopenia, and neutropenia. Infusion side effects, including drug-related fever, chills, and nausea, were also frequently seen. Seventeen of 20 patients were treated for disseminated histoplasmosis. Nineteen of 20 patients had acute leukemia. Although interaction with other agents could not be excluded, amphotericin B appeared to be the major causative agent for the toxic reactions noted. In no patient, however, was administration of amphotericin B stopped because of drug toxicity.
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PMID:Toxicity of amphotericin b in children with cancer. 46 22

In the past decade, we have studied four unrelated children with what we believe is a previously unreported disorder affecting the bone marrow and exocrine pancreas. During infancy these patients had the onset of severe, transfusion-dependent, macrocytic anemia plus a variable degree of neutropenia and thrombocytopenia. Their bone marrows had normal cellularity but were characterized by remarkable vacuolization of erythroid and myeloid precursors, hemosiderosis, and ringed sideroblasts. The vacuoles probably represented manifestations of cellular degeneration and death. In two patients, in vitro bone marrow cultures showed abnormal erythroid and myeloid progenitor cell growth and, in one child, abnormal vacuolated erythroid colonies. Family histories were unrevealing, parents were hematologically normal, and both sexes were involved. There was no evidence of specific nutritional deficiencies or exposure to agents associated with marrow vacuolization. A number of therapeutic interventions produced no effect. One child had clinical malabsorption. This child and one other had extensive pancreatic fibrosis at autopsy. The other two patients had findings indicating exocrine pancreatic dysfunction. Two children had splenic atrophy. This new syndrome, with associated bone marrow and exocrine pancreatic dysfunctions, differs in several respects from the syndrome of pancreatic liposis and neutropenia described by Shwachman et all and Bodian et al, and from other conditions with vacuolization of the marrow or sideroblastosis.
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PMID:A new syndrome of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreatic dysfunction. 50 2


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