Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The bone marrow biopsy specimens of 35 patients with benign and malignant erythroid hyperplasias were examined for the presence of hemoglobin A, hemoglobin F, muramidase (lysozyme), and transferrin, using an indirect immunoperoxidase method (PAP) on Zenker's-fixed paraffin-embedded bone marrow biopsy specimens and particles. Five cases of each of the following entities were studied: erythroleukemia and erythremic myelosis, acute granulocytic leukemia with maturation (FAB M2), polycythemia rubra vera, myeloproliferative syndrome in childhood, megaloblastic anemia (B12 and folate deficiency), erythroid hyperplasia (regenerating bone marrow and hemolytic anemia), and Ph' chromosome positive chronic granulocytic leukemia. Hemoglobin A was present in both the early and late erythroid precursors in all conditions. Hemoglobin F was the predominant hemoglobin in early erythroblasts of pernicious anemia and in both early and late erythroid elements in erythroleukemia and erythremic myelosis. Small quantities of hemoglobin F were present in a few isolated clusters in other conditions. Staining for hemoglobin F may be useful in identifying immature erythroid precursors and in distinguishing some cases of dysplastic erythroid hyperplasia from neoplasia. Additionally, these findings suggest that the maturational switch in hemoglobin synthesis operates with distinct pathways under different conditions.
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PMID:An immunohistochemical study of hemoglobin A, hemoglobin F, muramidase, and transferrin in erythroid hyperplasia and neoplasia. 619 99

Chromosomes of bone marrow from 28 patients with acute nonlymphocytic leukemia (ANLL) (26 with AML, 2 with AMMoL), 19 of whom had chromosome abnormalities, were studied; 11 cases exhibited previously unreported karyotypic abnormalities. The marrows of two cases had 8-21 translocations associated with an iso-X chromosome in the female patient and with 9q13- and a missing Y in the male patient. Usually, AML patients with a 8-21 translocation have been considered to have a good prognosis; however, our cases had rather short survival times. Therefore, the prognosis of AML with an 8-21 translocation but associated with other abnormalities is still not clear. Centromere spreading (CS), which was originally reported in marrow cells of megaloblastic anemia (B12 and folic acid deficiency), was detected in leukemic cells, disappeared during remission, and reappeared on relapse. These findings suggest that CS may be a new type of abnormality in AML. In two patients with atypical hypoplastic anemia and hemolytic anemia, chromosome abnormalities were detected at the anemic stage. One case with CS was associated with atypical hypoplastic anemia and developed AML after 1 year; the other with 48,XY,+i(1q),+3,/12 and -14 had hemolytic anemia and developed AMMoL 3 weeks later. Interestingly, identical clones were detected both before and after the clinical diagnosis of leukemia. These cases strongly support the concept that some chromosome abnormalities precede the clinical manifestations of leukemia. The present study also revealed that lymphocytes in ANLL respond poorly to PHA in the presence of high numbers of blasts but do respond well to mitogens during remission. Therefore, the response of lymphocytes to PHA may serve as one criterion for determining remission.
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PMID:Diagnostic and prognostic significance of chromosome abnormalities in marrow and mitogen response of lymphocytes of acute nonlymphocytic leukemia. 695 Aug 4

The activities of 5-methyltetrahydrofolate (5-CH3THF) related enzymes and DNA polymerase alpha were determined in bone marrow cells obtained from patients with vitamin B12 deficient megaloblastic anemia and compared with those from healthy volunteers and patients with hemolytic anemia. 5-CH3THF homocysteine methyltransferase activity was significantly lower than that in the control subjects. 5,10-methylenetetrahydrofolate reductase activity was only slightly elevated to that in the control subjects. DNA polymerase alpha activity was significantly higher than that in the control. High deoxyuridine suppression test values in vitamin B12 deficient bone marrow cells were improved by tetrahydrofolate, but not by 5-CH3THF. These data indicate that, even though the reverse reaction catalyzed by 5,10-methylenetetrahydrofolate reductase may be operative in vitamin B12 deficiency, it is not sufficient to correct the disturbance in folate metabolism in vitamin B12 deficiency. Increased DNA polymerase alpha activity may be due to compensation for disarranged DNA synthesis.
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PMID:5-Methyltetrahydrofolate related enzymes and DNA polymerase alpha activities in bone marrow cells from patients with vitamin B12 deficient megaloblastic anemia. 703 72

Ten patients with porcine xenograft aortic and mitral valve prostheses were studied three to 36 months (mean 15 months) postoperatively for evidence of hemolysis. Studies included complete blood count, reticulocyte count, red cell indices, percentage of schistocytes on blood smears, bilirubin concentration, lactic dehydrogenase, serum iron, total iron binding capacity, haptoglobin, serum folate and vitamin B12 levels, Coombs' test, methemoglobin reduction test, autologous 51Cr erythrocyte survival, and urinary examination for iron and hemosiderin. All patients were hemodynamically stable. Nine patients had normal valve function and no evidence of hemolysis. One patient with paravalvular aortic regurgitation had mechanical hemolytic anemia with a negative Coombs' test. Porcine valve xenografts do not seem to be associated with hemolysis unless complicated by a paravalvular leak.
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PMID:Erythrocyte survival in patients with porcine xenograft aortic and mitral valves. 706 4

A study of 196 healthy geriatric females and 26 males confirmed the high prevalence of anemia in this population. Forty-two females (21.4%) had a hematocrit of less than 36% and nine (34%) of the males had a value of less than 40%. A careful evaluation, including a therapeutic trial of oral iron in some subjects, made a diagnosis of iron deficiency anemia in only three and the anemia of chronic disease could be diagnosed in two. No subject had folate or B12 deficiency and hemolytic anemia was not present. Thus, in most of these subjects, the lower hematocrit was not due to commonly recognized causes. Further evaluation revealed a high prevalence of leukopenia in this population; 30% having counts less than 5 X 10(3)/microliter and 10% having values less than 4 X 10(-3)/microliter. Of most importance, leukocyte counts were significantly lower in anemic subjects in whom a high incidence of neutropenia was also demonstrated. This suggests that the mechanisms of the unexplained anemia and neutropenia is an overall reduction in hematopoietic cell numbers. This conclusion is consistent with the observation that many aged subjects have a decreased ability to mount an adequate leukocyte response to infection. These findings suggest that the presence of anemia marks an overall reduction in hematopoietic reserve and provides a clue to those aged subjects of greatest risk of an inadequate response to stress.
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PMID:The anemia of senescence. 727 May 45

In a 81-year-old woman, who for many years had been treated with iron and vitamin B12 injections because of a 'tendency to anaemia', congenital haemolytic anaemia on the basis of glucose-6-phosphate dehydrogenase (G6PD) deficiency was diagnosed. The iron and vitamin medication was discontinued and after a blood transfusion because of signs of heart failure, the patient could leave the hospital in good condition. After instruction with regard to provocative factors, like eating of broad beans, no more haemolytic events occurred. Of her children and grandchildren, 2 sons and 1 granddaughter were G6PD deficient.
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PMID:[Glucose-6-phosphate dehydrogenase deficiency in an 81-year-old]. 809 Feb 51

Many anemias and diseases of the blood-forming elements increase in incidence with advancing age. Iron deficiency remains the cause of most cases of anemia and may be related to inapparent blood loss. Other responsive anemias that can be managed by the primary care physician include vitamin B12 and folate deficiencies and drug- or autoimmune-related hemolytic anemia. Nonresponsive anemias, including hematologic malignancies, are quite common in older patients and require referral to a specialist. Platelet disorders are often produced by certain drugs and, like most refractory anemias, often require a bone marrow aspirate for diagnosis.
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PMID:Common blood disorders: a primary care approach. 846 84

Completely different entities might be with the same possibility in the baseline of interweaving of symptoms and signs of nervous system damage. One of them, the deficiency of vitamin B12 very frequently causes megaloblastic anemia and funicular myelosis. In the case of our patient, after the clinical picture of hemolytic anemia was revealed, by slow-progressive course was developed neurologic deficiency that, according to its features, could have the deficiency of cobalamin and folic acid in its etiologic background. On the basis of disease course, clinical finding, numerous clinical investigations so as the reaction to applied therapy it was assumed that the patient had besides confirmed autoimmune hemolytic anemia the pernicious anemia as the associated cause of anemic syndrome and the basic reason of the development of neurologic deficiency. Described is the frequent associated occurrence of pernicious anemia and antiglobulin positive hemolytic anemia, so as the significant association of pernicious anemia with the deficiency of immunoglobulins that was otherwise observed in our patient as the permanent IgA deficiency.
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PMID:[Neurologic disorders in a patient with autoimmune hemolytic anemia]. 992 Oct 81

Anew case of cobalamin C disease associated with hemolytic-uremic syndrome (HUS) in the neonatal period is described. A 28-day-old boy presented with failure to thrive, hypotonia, pancytopenia, and features of HUS (microangiopathic hemolytic anemia, thrombocytopenia, and renal failure). The possibility of the diagnosis of an underlying vitamin B12 disorder was prompted by evidence of megaloblastic changes on the peripheral smear and by finding in the literature a suggested association of neonatal HUS with this cobalamin-related metabolic disorder. Amino acid analysis showed elevated homocysteine levels in the plasma and increased levels of both homocysteine and methyl malonic acid in the urine. Diagnosis of cobalamin C disease was confirmed by complementation studies using skin fibroblasts. Therapy included parenteral hydroxocobalamin, carnitine, and leucovorin calcium (folinic acid). Cobalamin C disease should be considered in the diagnosis of patients presenting with HUS in infancy who have unexplained megaloblastosis, pancytopenia, neurologic impairment, and failure to thrive. Early diagnosis and institution of therapy may be effective in improving survival and quality of life.
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PMID:Cobalamin C disease presenting as hemolytic-uremic syndrome in the neonatal period. 1197 7

Haematological symptoms can be helpful for the diagnosis of metabolic diseases. A megaloblastic anemia orientates to folate and cobalamine anomalies when associated with homocystinemia and decreased plasma methionine levels, or to congenital oroticuria (hypochromia), Pearson syndrome (sideroblasts and vacuolisation of precursors) and thiamine transporter abnormality (sideroblasts) in the absence of homocystinuria. An hemolytic anemia orientates to anomalies of anaerobic glycolysis, heme synthesis, or iron metabolism, and Wilson disease. A pancytopenia orientates to organic aciduria, lysinuric protein intolerance, mevalonic aciduria and lysosomal storage diseases (Gaucher, Niemann Pick, Wolman) when hepatosplenomegaly is present. Uremic hemolytic syndrome and hemophagocytic lymphohistiocytosis respectively orientate to B12 anomalies, lysinuric protein intolerance, lysosomal storage diseases and organic aciduria.
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PMID:[Hematologic manifestations of inborn errors of metabolism]. 1220 94


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