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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe the case of a young Algerian, aged 32, suffering from mild icterus, accompanied by a marked splenomegaly. The blood count revealed a moderate degree of anaemia with reticulocytosis, pronounced anisocytosis, micro-spherocytes, bulls eye cells, folded cells, hypochrome cells, a marked polychromasia and a mild erythroblastosis. Present also were hyperbilirubinaemia, raised plasma haemoglobin, zero haptoglobin, a reduced osmotic fragility and half-life of erythrocytes. Haemoglobin electrophoresis showed 17.25% haemoglobin F, 62.8% haemoglobin C+A2 and no haemoglobin A. The genetic study indicated that the patient was a double heterozygote C/beta thalassaemia, his mother and his son both suffering from this disease. This thalassemic gene of type beta (0) totally inhibited the synthesis of haemoglobin A, the defect found in our patient.
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PMID:[Haemoglobinosis C/beta-thalassemia double heterozygosity in an Algerian patient with total suppression of haemoglobin A synthesis (author's transl)]. 67 Sep 35

A 16-year-old male (S.F.) and his 21-year-old sister (D.H.) from a large family of Italian and Swedish descent had virtually identical lipoprotein pattern and complete absence of LCAT activity. Both had typical corneal opacities and mild anemia with target cells. S.F., but not D.H., presented with proteinuria, which has increased over three years of follow-up. His kidney biopsy revealed lipid deposits in the glomerular basement membrane. Ten relatives in 4 generations had normal LCAT activity and/or lipoprotein pattern. The patients and their relatives had haptoglobin type 2. Factors that might influence the different clinical presentation in our patients (previous renal disease, diet, abnormal lipoproteins), prognosis, and treatment (diet, enzyme replacement, cholestyramine) are discussed.
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PMID:Familial LCAT deficiency. Report of two patients from a Canadian family of Italian and Swedish descent. 74 43

Since 1967 a syndrome characterized by renal disease, normochromic anaemia and corneal opacities has been described in 7 members of 3 different Norweigan families. The patients have low levels of esterified cholesterol and lack LCAT (lecithin: cholesterol acyltransferase) activity in plasma. The genetic basis of the disorder seems to be the presence of a LCAT deficiency gene in double dose. This gene is probably the result of a single mutational event. Linkage studies revealed non-random assortment between LCTA deficiency and serum haptoglobin (Hp) types. After Hp subtyping a combined lod score of 3-41 at a recombination fraction of 0-00 was obtained. Association was revealed between the LCAT deficiency gene and the Hp-1S gene. We propose that the LCAT gene is situated close to the alpha-haptoglobin locus on chromosome no. 16.
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PMID:Genetics of LCAT (lecithin: cholesterol acyltransferase) deficiency. 80 50

Heinz body anemia was induced in dogs by consecutive oral administration of 200 mg/kg and 60 mg/kg of aminopyrine, 200 mg/kg of phenacetin and 5 mg/kg of acetylphenylhydrazine, for either 8 or 16 weeks. Biochemical analysis showed a decrease of haptoglobin in plasma and reduced-glutathion in red blood cells in association with anemia. Hematologically, an increase in osmotic fragility and cell volume of erythrocytes were also noted. Pathological examination revealed marked erythrophagia and hemosiderosis in the reticuloendothelial system of treated animals. A new anti-inflammatory analgesic 31252-S (3-(1-hydroxy-2-piperidinoethyl)-5-phenylisoxazole citrate) was also tested for the hemolytic effects in dogs. Blood analyses revealed a slight increase of Heinz bodies and a decrease of reduced-glutathion in red blood cells without anemia. These findings suggest that 31252-S have weaker oxidant properties than those of aminopyrine and phenacetin. The present studies showed that the concentration of reduced-glutathion in red blood cells of non-treated dogs was approximately 50% lower than that of the erythrocytes of normal human beings. This finding appeared to correspond with high susceptibility of red blood cells to hemolytic effects of oxidant-drugs in dogs.
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PMID:[Oxidant-drug induced hemolytic anemia in dogs]. 124 68

In conclusion, many specialized tests for the evaluation of anemia and RBC abnormalities can readily be performed in-house or by commercial laboratories. Tests include RBC indices, examination of blood smears (for morphological changes, infectious agents, basophilic stippling, and polychromasia), reticulocyte counts, iron stains, serum iron determinations, Heinz body counts, and methemoglobin tests. These diagnostic tests should routinely be used for the evaluation of hematologic abnormalities that are detected by a CBC. Accurate interpretation of test results will provide vital information on the pathophysiological and/or etiologic mechanisms of disease and point the way to successful therapeutic intervention. Failure to adequately resolve some hematologic abnormalities, particularly in breeds in which heritable disease is a consideration, may require additional tests, including serum haptoglobin, ferritin and erythropoietin measurement, or assays for RBC enzymes, such as methemoglobin reductase, PK, and PFK. These tests usually are available only through research laboratories or hematology specialists, and it is advisable to consult with a veterinary clinical pathologist for referral to the appropriate individual or laboratory.
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PMID:Specialized hematology tests. 147 Jul 72

In autoimmune haemolytic anaemia, the presence of antibodies on the erythrocyte membrane results in haemolysis through an immune process, but does it not alter the rheological properties of red blood cells (RBC), thus adding a mechanical factor to haemolysis? This study was designed to examine the rheological properties of erythrocytes sensitized with IgG-type antibodies. The study involved 20 patients with anaemia and positive direct antiglobulin test, including 12 with straightforward haemolysis, 10 samples sensitized in vitro, and 20 controls. The following haemorheological parameters were studied: erythrocyte filtration, blood and plasma viscosities, titration of adenosine triphosphate (ATP) and 2-3-DPG, erythrocyte morphology under scanning electron microscopy. The results showed increased erythrocyte rigidity (P less than 0.025) as well as higher blood viscosity compared to controls with similar haematocrit values, and unaltered ATP and 2-3-DPG (consistently with scanning electron microscope observations). These haemorheological disorders were more noticeable in patients with clear-cut haemolysis, and there was a correlation between the increase in erythrocyte rigidity indices and the haemolytic parameters, especially haptoglobin (P less than 0.001). The in vitro study confirmed the results obtained ex vivo. To conclude, the mechanical properties of antibody-coated erythrocytes are impaired, which may promote the immunological mechanism favouring haemolysis in the spleen.
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PMID:Is there a mechanical factor of haemolysis in patients with positive IgG-type direct antiglobulin test? 153 15

We report herein a 61-year-old man with chronic cold agglutinin disease which occurred after a diagnosis of aplastic anemia. The patient's pancytopenia was recognized upon visiting a local doctor because of high fever and cough on December 21, 1985. He was subsequently admitted to our hospital because of anasarca on January 31, 1986, and was diagnosed as having aplastic anemia. He was treated with prednisolone, and was discharged after his anemia improved. He was readmitted on October 23, 1988, because of icterus. Laboratory data on the patients second admission revealed increased reticulocyte count, hyperplastic bone marrow with a predominance of erythroblasts, increased serum indirect bilirubin, increased serum LDH1 value and decreased serum haptoglobin. Moreover, cold agglutinin titer was increased, anti-IF antibody was positive, and anti-IgM antibody was recognized with direct anti-globulin test. There was no precedent infection such as mycoplasma pneumonia or infectious mononucleosis. Hence, this patient was diagnosed as having chronic cold agglutinin disease.
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PMID:[Chronic cold agglutinin disease occurring after a diagnosis of aplastic anemia]. 154 14

Hemolysis can be induced by two general mechanisms. In the first one, erythrocytes lyse intravascularly due to complement fixation, trauma, or other extrinsic factors. In the second mechanism, which is the most common, the red cells are removed from the circulation by the mononuclear-phagocytic system either because they are intrinsically defective or because of the presence of bound immunoglobulins to their surfaces. The diagnosis of hemolysis is not difficult to establish and is based on the presence of anemia with sustained reticulocytosis in the absence of blood loss. Additional findings can include marrow erythroid hyperplasia; increased unconjugated bilirubin, LDH, and free hemoglobin; decreased haptoglobin and hemopexin; hemoglobinuria and hemosiderinuria; and decreased 51Cr red cell half-life. Hemoglobinemia, hemoglobinuria, and hemosiderinuria occur only in the setting of severe and rapid intravascular hemolysis. Conditions associated with significant lysis of red cells in the circulation include incompatible transfusion, G6PD deficiency, PNH, severe burns, and certain infections. The morphology of the red cell is abnormal in almost all cases of hemolytic anemia. However, the morphologic abnormality can be, in certain cases, diagnostic of the underlying condition. Treatment is usually supportive, with effective therapy directed to treat the underlying cause of hemolysis.
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PMID:Hemolytic anemias. Diagnosis and management. 157 62

A retrospective study of the 99 surviving heart and lung transplant (HLT) recipients at one center showed that 31% had significant anemia (hemoglobin less than 100g/L) six months after transplantation. Chronic anemia persisted in 18% of HLT recipients two years posttransplantation. A similar study of 100 heart transplant recipients showed no unexplained anemic patients. The prevalence of anemia after HLT was unrelated to the original diagnosis, immunosuppression, or acute rejection. All HLT recipients appeared to be unduly sensitive to the myelosuppressive effects of azathioprine. Detailed studies in 16 representative patients showed a normochromic, anisocytotic anemia with normal reticulocyte counts, B12 and folate levels, and haptoglobin levels and appropriate erythropoietin levels--but increased ESRs, low/normal iron levels and low/normal total iron binding capacity, normal or raised ferritin levels, and autoantibodies in 4 (25%). Bone marrow aspirates in 10 patients showed dyshemopoiesis out of proportion to the degree of anemia and colonies of activated lymphoid cells. The cause for this anemia appears to be a combination of anemia of chronic disease and dyshemopoiesis, both of uncertain etiology.
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PMID:The prevalence, course, and characteristics of chronic anemia after heart and lung transplantation. 160 80

It is generally considered that abnormality of the erythrocyte membrane skeleton co elliptocytes. There are, however, few reports of beta spectrin variants. We found a new variant of beta spectrin in a child and her mother. This report is the first case of abnormality of beta spectrin in Japan. The propositus was an 8 month-old girl who was first examined by us in 1988. On laboratory findings, she showed anemia, increased reticulocyte count and decreased haptoglobin concentration. Both peripheral blood smears of patient and her mother showed typical elliptocytosis and they were diagnosed as hereditary elliptocytosis. SDS-PAGE patterns of the red cell membranes of the propositus and her mother were characterized by the presence of an abnormal component migrating immediately below the spectrin chains. We confirmed that the abnormal spectrin appeared clearly at the expense of normal beta chain. The abnormal spectrin (M.W. 216,000d) makes up 16% of the total beta chain. The inheritance of our case was autosomal dominant. The present case is considered as a new spectrin variant.
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PMID:[Abnormalities of beta spectrin with hereditary elliptocytosis in mother and child]. 163 63


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