摘要:

AbstractIn the USA, erythrocytic glutathione reductase (GSSG‐R) deficiency is significantly more common, and can be considerably more pronounced in hospitalized patients (118/3198) than in outpatients (37/1639) or in apparently healthy persons (12/849). Retrospective analysis of illnesses found in 118 inpatients with erythrocytic GSSG‐R deficiency revealed a striking and previously unsuspected association of the enzyme deficiency with a variety of chemotherapeutically treated hematological or nonhematological malignancies (51/118 patients, 43.2%, or 51/170 diagnoses, 30.0%). The prevalence of erythrocytic GSSG‐R deficiency also increased in malnutrition, liver disease, and sepsis. Drugs of the nitrosourea class, particularly BCNU [1, 3‐bis(2‐chloroethyl)‐1‐nitrosourea] are causally implicated in the association of GSSG‐R deficiency with malignancies. Severe or complete GSSG‐R deficiency may handicap host respo

ISSN:0361-8609    

DOI:10.1002/ajh.2830020402

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY

摘要:

AbstractThe first case of Hb Leiden (α2β26 or 7 Glu→0)‐β° thalassemia in a young patient with chronic severe hemolytic anemia, which improved after splenectomy, is described. His parents were Chinese. The patient's blood showed no Hb A or normal β chains when no blood transfusion was given. His mother was heterozygous for β° thalassemia, and his father and brother had the trait for the unstable Hb Leiden. The Hb Leiden level of the father was 22.6% and that of the brother was 19.3%. It is probable that the abnormal hemoglobin in this Chinese family resulted from an independent gene mutation, unrelated to the one found in 2 Caucasian families report

ISSN:0361-8609    

DOI:10.1002/ajh.2830020403

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY

摘要:

AbstractThe log P50of normal human blood at 37°, PCO2= 0, 21, 42, and 57 mm Hg, in the absence and in the presence of 2,3‐DPG and ATP, has been determined in the pH range 7.0 to 7.6. Similar data have been obtained for human hemoglobin isotonic solutions at different protein concentrations in the presence of various amounts of each of the cofactors which are known to affect hemoglobin oxygen affinity in blood. It has been found that the addition of KCl, organic phosphates, magnesium ions, and CO2confers to a 32% human hemoglobin solution the same oxygen affinity (over the entire physiological pH range) of whole blood. Thus there is no room for significant effects caused by some other unidentified molecules or io

ISSN:0361-8609    

DOI:10.1002/ajh.2830020404

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY

摘要:

AbstractThe effects of chloramphenicol (CAP) and thiamphenicol (TAP) on mouse and human in vitro erythroid (CFU‐E) and granulocytic (CFU‐C) colony forming units have been studied. Both drugs inhibited CFU‐E growth in a concentration‐dependent, stereospecific, manner. Complete inhibition of human CFU‐E growth was observed at a CAP concentration of 10 μg/ml while a concentration over 50 μ/ml was required to inhibit CFU‐C growth. Furthermore, whereas the inhibition of CFU‐C growth could be blocked in vitro by high colony stimulating factor concentrations, inhibition of CFU‐E growth was not affected by increased erythropoietin (ESF) levels. The lack of protection by ESF may account for the apparent vulnerability of erythroid cel

ISSN:0361-8609    

DOI:10.1002/ajh.2830020405

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY

摘要:

AbstractWe have studied the effects of splenectomy and glucocorticoids on the survival and sequestration of Heinz body‐containing red blood cells (RBC‐HZB). Mice were injected with phenylhydrazine damaged51Cr labeled isologous red blood cells (RBCs). The spleen removed 36% and the liver 19% of the injected dose after 120 hrs. Red cell survival (T½) fell from 180 hrs for undamaged red cells to 16 hrs for RBC‐HZB. Splenectomy resulted in an increase in hepatic uptake of damaged RBCs (36% of the injected dose) and a modest improvement in red cell survival (T½ 54 hrs). Treatment of non‐splenectomized mice with glucocorticoids reduced the splenic uptake to 16% and the hepatic uptake to 14% of the injected dose. The reduction of splenic uptake was associated with a decrease in splenic mass rather than a decrease in uptake per unit weight of splenic tissue, while the reduction in hepatic uptake was associated with both a decrease in hepatic mass and uptake per unit weight. A marked decrease was observed in hepatic uptake and in phagocytosis by Kupffer cells in glucocorticoid‐treated splenectomized mice. These data suggest that increased hepatic uptake may decrease the effectiveness of splenectomy in RBC‐HZB hemolytic anemia and that glucocorticoids may decrease the hepatic uptake by reducing phagocytosis by

ISSN:0361-8609    

DOI:10.1002/ajh.2830020406

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY

摘要:

AbstractWe have identified partial trisomy 1q in 2 patients with different hematologic disorders. The first patient was a 55‐year‐old female with myelosclerosis and myeloid metaplasia diagnosed at age 38 years presenting with anemia, fatigue, bruising, fever, and splenomegaly. At age 56, she had 50–95% myeloblast cells and 95–100 nucleated RBC precursors per 100 WBC. Chromosome analysis of unstimulated leukocytes with Q, G, and C banding showed 46,XX,‐6,+t(1;6) (q25;p22) in all metaphase cells. In vitro incorporation of Fe55was demonstrated in 90% of metaphases by autoradiography. The second patient, a 49‐year‐old male, was diagnosed as having polycythemia vera at age 30 during a regular checkup. He since developed hepatosplenomegaly. Chromosome analysis from a direct bone marrow preparation at age 44 and 45 showed grossly normal karyotypes. At age 49, his marrow by Q and G banding showed almost 100% of cells with 46,XY,–13,+t(1;13) (q12;p12). Eleven cases of trisomy of 1q have been reported in various hematologic disorders. It is apparent that partial trisomy 1q represents another nonrandom chromosomal abnormality, in addition to the most common nonrandom chromosomal aberrations, such as the Philadelphia chromosome, trisomy 8, trisomy 9, and monosomy 7 in hemato

ISSN:0361-8609    

DOI:10.1002/ajh.2830020407

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY

摘要:

AbstractA patient with inherited combined deficiency of factor V and factor VIII is reported, who demonstrated normal levels of factor VIII antigen and plasma co‐factor for ristocetin‐induced platelet aggregation. The relationship of this condition to classical hemophilia and von Willebrand's disease is discussed. The data presented suggest that multiple loci on at least 2 chromosomes are necessary for the normal expression of factor VIII activ

ISSN:0361-8609    

DOI:10.1002/ajh.2830020408

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY

摘要:

AbstractA case of Kaposi's sarcoma which presented as a warm type of immune hemolytic anemia is described. The malignancy was discovered at the time of splenectomy, which was required for control of the hemolytic anemia. Three other cases of immune hemolytic anemia in patients with Kaposi's sarcoma have been reported. An association between Kaposi's sarcoma and immune hemolytic anemia is suggested. Careful examination of the skin for Kaposi's sarcoma seems appropriate in cases of immune hemolytic anemia.

ISSN:0361-8609    

DOI:10.1002/ajh.2830020409

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY

摘要:

AbstractAlthough patients with hemolytic hemoglobinopathies characteristically are over‐producers of urate, and hyperuricemia is frequently recognized, clinical gout has rarely been reported in such patients. Our evaluation of 2 premenopausal women with gout led to the diagnosis of previously unrecognized hemoglobinopathies (SC disease and CC disease). Investigation of these 2 patients and review of the reported cases of gout in patients with hemoglobin S or C disorders suggest that relatively minor abnormalities of renal function in these patients may lead to early development of significant hyperuricemia. With increasing lifespan of patients with hemolytic hemoglobinopathies and the likelihood of increased occurrence of renal function abnormalities, it is anticipated that gout will more frequently be responsible for joint symptoms in such patient

ISSN:0361-8609    

DOI:10.1002/ajh.2830020410

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY

摘要:

AbstractA simple and accurate micromethod for the determination of erythrocyte osmotic fragility is introduced. The method uses a laminar parabolic flow pattern, together with gravity, to retain cells in a long, small‐diameter tube while a solution with decreasing osmolarity is passed through the tube. As the cells hemolyze, hemoglobin released from the cells is quickly removed by the axial flow pattern and monitored with a 547 nm optical detector for recording the hemolysis curve. Consequently, a continuous curve is obtained, with a peak occurring at the salt concentration that produces the maximum hemolysis rate. The advantages of this method are simplicity, accuracy, and small sample size (2 microliters of whole blood). The small sample size is of particular importance for infants.A comparison is made with the Parpart method using samples from 18 normal adults. Results are also given for a few abnormal adults and for a series of 26 normal newborn

ISSN:0361-8609    

DOI:10.1002/ajh.2830020411

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1977

数据来源: WILEY