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

17 babies suffering from haemolytic disease or "hyperbilirubinaemia of prematurity" underwent exchange transfusion in the first 4 days of life. In 14 babies, including all those who had abnormally low pre-exchange serum vitamin E levels (<0.5 mg/100 ml), the vitamin E concentration rose as a result of exchange transfusion. In 3 babies the serum vitamin E concentration fell as result of exchange transfusion, but in no case was the post-exchange level below 0.5 mg/100 ml. The normal range of plasma vitamin E in stored ACD blood was 0.44--1.24 mg/100 ml. It was concluded that although vitamin E deficiency might be a factor in the aetiology of the anaemia of prematurity, prior subjection to exchange transfusion is unlikely to make a significant contribution to the vitamin E deficiency.
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PMID:Exchange transfusion and serum vitamin E (tocopherol) concentration in the newborn. 57 29

The ultrastructure of erythroblasts in a case of congenital dyserythropoietic anaemia type II with a negative serum-lysis test is described. The well known symptoms in electronmicroscopy in CDA type I and II are faced to our findings. Morphological changes of hypolemmal cisterns are described and their possible meaning is discussed.
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PMID:[Ultrastructure of erythroid cells in a case of congenital dyserythropoietic anaemia type II (author's transl)]. 59 16

The lipids derived from the erythrocytes of two siblings with clinical congenital dyserythropietic anemia Type II (CDA-II) and one sibling without clinical evidence of CDA-II underwent a detailed analysis. The total phospholipids, total cholesterol, and cholesterol: phosphate ratio were normal in all siblings. In the two clinically affected siblings there was a significant increase in the phosphatidyl choline with a concomitant decrease in the other phospholipids. The fatty acids of phosphatidyl choline from the CDA-II erythrocytes were normal. The total gylcosphingolipids were increased in all siblings. The glycosphingolipids, di- , tri-, and tetrahexosyl ceramides were increased 1.5- to 10-fold over controls in the clinically affected siblings. The increases were present but less striking in the clinically unaffected sibling. Gluococerebroside was not significantly increased in any of the siblings. The possible causes and significance of the lipid changes are discussed.
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PMID:Abnormal lipid composition of the red cell membrane in congenital dyserythropoietic anemia type II (HEMPAS). 114 28

The red blood cell membranes of patients with congenital dyserythropoietic anaemia Type II (CDA-II) were found to be abnormal. They had altered antigenic characteristics, decreased electrophoretic mobility, decreased sialic acid content, and abnormal filtration through polycarbonate filters. Proteins extracted from the CDA-II erythrocytes showed a different banding pattern on polyacrylamide gels compared to normal erythrocytes. Erythrocytes from clinically unaffected siblings and parents showed similar but less striking abnormalities of antigenic surface characteristics and banding patterns on polyacrylamide gels with nearly normal surface charge, sialic acid content, and filtration properties. These studies suggest a correlation between the degree of membrane abnormality and the clinical state of the CDA-II family member, demonstrate the erythrocyte in the heterozygote state is not normal, and support the concept of CDA-II as an autosomal recessive disease.
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PMID:The abnormal surface characteristics of the red blood cell membrane in congenital dyserythropoietic anaemia type II (HEMPAS). 120 Dec 21

ACD is probably the most common anemia among hospitalized medical patients. It is variably defined by its clinical and, particularly, its laboratory manifestations. The most consistent features are low serum iron and normal or increased serum ferritin levels, reflecting normal or increased iron stores and distinguishing ACD from iron deficiency anemia. ACD often coexists with iron deficiency and the anemia of renal insufficiency. Most patients have an underlying infectious, inflammatory, or neoplastic disease, but as many as one quarter of patients do not. Several mechanisms have been proposed, the most significant of which are a block in reutilization of hemoglobin iron for red cell production and relative deficiency of erythropoietin, but the pathogenesis and mediators involved remain uncertain. The anemia itself seldom requires treatment and is ameliorated by successful treatment of the underlying disease.
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PMID:Anemia of chronic disease. 157 57

In this study we determined the serum erythropoietin (Epo) levels of 97 anaemic rheumatoid (RD) patients (Hb less than 11 g/dl) of whom 44 had serum ferritin greater than 60 micrograms/l (anaemia of chronic disorders; ACD), 26 had ferritin 20-60 micrograms/l and 27 had ferritin less than 20 micrograms/l. These results were compared with the Epo levels of 36 iron deficient controls (Hb less than 11 g/dl), 33 non-anaemic RD patients and 33 normals. The serum Epo levels of anaemic subjects were significantly higher (P less than 0.05) than those of non-anaemic patients. Despite similar Hb, the Epo results (geometric mean (confidence limits] of the ACD group (38 (32,45) mU/ml) and of RD patients with ferritin of 20-60 micrograms/l (39 (33,46)mU/ml) were significantly lower (P less than 0.05) than those of iron deficient controls (65 (52,80)mU/ml). When the Hb fell to 10 g/dl or less, the serum Epo of 13 RD patients with ferritin less than 20 micrograms/l was 65 (47,89)mU/ml, significantly lower (P less than 0.05) than that of 17 iron deficient controls (104 (78,136)mU/ml). These results justify clinical trials of recombinant human Epo in RD patients with ACD.
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PMID:Evidence for impaired erythropoietin response to anaemia in rheumatoid disease. 154 Jul 97

Serum thymidine kinase (TK) was determined in a family with congenital dyserythropoietic anaemia type III (CDA, type III). 20 patients and 10 of their healthy siblings were investigated. Elevated TK was found in all 20 patients (median 56.2 U) but their healthy siblings had normal values (median 2.65 U). We suggest that determination of TK should be used for discrimination between healthy siblings and individuals affected by CDA type III when bone marrow examination is not suitable.
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PMID:Serum thymidine kinase in congenital dyserythropoietic anaemia type III. 799 14

In a 48-year-old woman investigated on account of congenital dyserythropoietic anaemia type II (HEMPAS) on the X-ray of the chest a polycystic sharply defined shadow in the posterior mediastinum was detected and extramedullary haematopoiesis was suspected. The diagnosis was confirmed cytologically. According to the authors' knowledge this is the second case of this rare complication in CDA-II in the world and the second case of EMH in the mediastinum in this country. In the discussion the authors summarize the diagnostics of congenital dyserythropoietic anaemias. They discuss also the pathogenesis of extramedullary haematopoiesis, diagnosis and differential diagnosis of EMH in the posterior mediastinum, possible complications and treatment.
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PMID:[Congenital dyserythropoietic type II anemia complicated by extramedullary hematopoiesis in the posterior mediastinum]. 857 4

Anemia is common in cancer patients, especially in those with more advanced stages of progressive tumor growth, the frequency varying on type of cancer, stage and chemotherapy or radiation therapy used. The pathophysiology is multifactorial. However the most common anemia is the anemia whose features are similar to those seen in other chronic diseases (anemia of chronic disease--ACD). The pathophysiological mechanisms are: a mild decrease in red blood cells survival, a decreased re-utilization of bone marrow iron stores and an inadequate erythropoietin response to the degree of anemia. When anemia cannot be corrected through the administration of hematinics and anemia is severe enough to significantly restrict physical activity and quality of life, blood transfusion is requested. It has been reported that the percentage of patients requiring transfusion ranges from 20 to 50%. The transfusion of allogeneic blood exposes the recipient to immunological and infectious risks. There is evidence that allogeneic blood transfusions can have immunologic consequences and some argue that these immune changes can adversely affect the prognosis in cancer patient. Although this is still controversial, until it can be shown that blood transfusion is not harmful in the long term to patients with cancer, it seems reasonable to avoid it whenever possible. Recently the availability of recombinant DNA technology permitted large scale production of recombinant human erythropoietin (rHuEPO). To date several clinical trials employed rHuEPO in anemic cancer patients with various solid tumors both on and off chemotherapy. All these studies have reported a significantly increase in Hct than placebo in more than 50% of the treated patients. The problem of correcting anemia and of blood transfusion is even more important when cancer patients become candidate to major surgery. In such situation, the transfusion of a consistent number of units is generally required to cover the surgical blood loss. The use of homologous blood in surgery can be substantially reduced by the introduction of autologous blood transfusion (ABT) programmes in association with rHuEPO. A number of experimental and clinical studies on the effects of rHuEPO on AB donation and on erythropoiesis in the peri-operative period have demonstrated that it resulted to be effective in stimulating erythropoiesis, with a consequent increase in the volume of red cells produced during the course of treatment and in the number of units predeposited. It was also effective in correcting anemia induced by collection of blood units. The efficacy of rHuEPO in increasing the volume of autologous blood the patient can predeposit before surgery has been demonstrated also in patients with ACD and cancer. No significant adverse effects of rHuEPO administration have been reported in any of the studies published to date. It can be concluded that rHuEPO therapy may be safe and effective in selected surgical patients, in stimulating erythropoiesis, in expanding the circulating RBCs mass, in increasing the volume of AB that can be collected pre-operatively and, consequently, in reducing the exposure to homologous blood. Therapy with rHuEPO may prove to be a useful addition to existing strategies of blood conservation to minimize exposure to HB.
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PMID:[The role of human recombinant erythropoietin in oncologic surgery]. 948 79

Congenital dyserythropoietic anemia type II (CDA-type II) (HEMPAS) was reported in three siblings. CDA-type II was associated with marked hepatosplenomegaly and siderosis of both organs. All three sibling developed cholelithiasis with choledocholithiasis and obstructive jaundice in two of them. Anemia showing hemolytic component with sequestration of erythrocytes in the spleen was corrected after splenectomy without the need of blood transfusions during the follow up period of 20, 11 and 11 years. Ultrastructural investigation of the erythrocytes after splenectomy revealed increased number of erythrocytes showing the double membrane phenomenon.
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PMID:[Favorable effect of splenectomy on anemia in 3 siblings with type II congenital dyserythropoietic anemia (HEMPAS). (Ultrastructural changes in erythrocytes after splenectomy)]. 960 75


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