摘要:

AbstractWhether the trimodality in the relative concentration of the hemoglobin variant Hb Leslie in heterozygotes (Huisman, Hemoglobin 1: 349–382, 1977) is due to a polymorphism of the α‐chain structural genes was investigated by conventional incubation of reticulocytes with14C‐leucine. In addition, an aliquot from each of the incubations was incubated under the same conditions but without isotope. Three Hb Leslie heterozygotes with presumably four, three (heterozygous α‐thalassemia‐2), and two (homozygous α‐thalassemia‐2) active α‐chain genes and with 33%, 22%, and 11% Hb Leslie respectively, and one patient with the Hb Leslie β0‐thalassemia condition with more than 85% Hb Leslie were studied. The data indicate that βLesliechains have a lower affinity for α chains than βAchains. A concomitant α‐chain deficiency results in a reduced incorporation of βLesliechains into the tetrameric Hb Leslie molecules, while the quantity of Hb Leslie produced correlates with the degree of α‐chain deficiency. Excess of βLeslie

ISSN:0361-8609    

DOI:10.1002/ajh.2830050102

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

年代:1978

数据来源: WILEY

摘要:

AbstractA case of congenital nonspherocytic haemolytic anaemia associated with a new abnormal glucosephosphate isomerase (GPI), GSH (reduced glutathione) deficiency, and instability and altered carbohydrate membrane composition is reported. The only functional abnormality of the mutant enzyme seems to be a marked instability to heat, urea, and guanidine‐HCl. Family studies suggest that the propositus is doubly heterozygous for a maternal gene producing an inactive enzyme and a paternal gene responsible for a structural alteration causing marked lability of the coded enzyme. Experiments of incubation of normal GPI and the propositus's GPI with oxidizing and reducing agents seem to indicate that the abnormality resides in the SH groups of the mutant GP

ISSN:0361-8609    

DOI:10.1002/ajh.2830050103

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

年代:1978

数据来源: WILEY

摘要:

AbstractIn five patients with idiopathic aplastic anemia, the colony‐forming unit (CFU‐c) assay was used to quantitate stem cells and test the possible presence of supressor cells inhibiting bone marrow differentiation. All five marrows failed to form CFU‐c. In one out of the five cases tested, coculture of the patient's marrow with normal marrow suppressed the latter to form CFU‐c. Removal of T‐cells from the patient's bone marrow freed the aplastic marrow to produce more colonies and, in the coculture study, abrogated its inhibitory activity on the normal bone marrow. Treatment of the patient with horse antihuman thymocyte globulin (ATG), improved the peripheral blood count and myelopoiesis. Furthermore, the aplastic marrow increased its colony forming capacity from<1% pre‐ATG to 46% post‐ATG of the normal control, and its inhibitory activity on the normal marrow was ablated. In selected cases of aplastic anemia, with evidence of T‐suppressor cell hyperactivity, treatment with ATG may offer an alternative approach to bone‐marro

ISSN:0361-8609    

DOI:10.1002/ajh.2830050104

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

年代:1978

数据来源: WILEY

摘要:

AbstractObservations indicating an enhancing effect of sodium on hemolysis of red cells suspended in dextrose solution prompted study of the mechanism of this effect. Osmotic fragility of red cells from normal subjects and from patients was measured in 5% dextrose solution (5D/W), 5% dextrose combined with various concentrations of sodium chloride (5D/NaCl), lithium chloride, and potassium chloride. The influence of ouabain on osmotic hemolysis in 5D/ NaCl was also studied, and the glucose content of ghost red cells was determined when incubated in 5% dextrose with 0.05% NaCl (5D/O.05NaCl), as compared with 5D/W.In the presence of minimal amounts of NaCl (0.05%) at pH 4.8, average hemolysis was greater than in the absence of NaCl, ie, 39% vs 24% in normal subjects (P<0.02) and 40% vs 31% in a group of unselected patients (P<0.05). When the pH was adjusted to 7.4 in the patient group, the results were 34% vs 21% (P<0.05). The sodium‐induced enhancement of hemolysis in dextrose solution was virtually duplicated when LiCl and KCl were substituted for NaCl (P<0.01 and<0.05, respectively). On the other hand, in the presence of ouabain, the sodium‐induced enhancement of hemolysis was abolished. The overall glucose content of ghost red cells incubated in 5D/0.05NaCl was 53% greater than in 5D/W (P<0.005), whereas with ghost red cells depleted of adenosine triphosphate (ATP), it was only 32% greater (P<0.01).These results suggest that glucose transport across the red cell membrane is enhanced by the sodium ion, presumably by triggering the membrane sodium‐potassium ATPase s

ISSN:0361-8609    

DOI:10.1002/ajh.2830050105

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

年代:1978

数据来源: WILEY

摘要:

AbstractBecause unexplained megaloblastic erythroid maturation occurs in patients with primary refractory sideroblastic anemia (PRSA), deoxyuridine (dU) suppression tests using125I‐UdR were performed on bone marrow from five patients with PRSA. All patients had megaloblastic alterations in the marrow erythroid precursors (with normal serum folate and B12levels) and numerous ringed sideroblasts, and most had marrow iron overload. Results of the dU suppression tests were normal in all five, both with and without incubation with pharmacologic amounts of folate, B12, and pyridoxal phosphate with or without 1‐serine.Despite occasional hematologic improvement subsequent to folate, pyridoxine, or pyridoxal phosphate (PLP) administration in some patients with PRSA, normal dU suppression implies that in our group the availability of B12and folate (and possibly PLP) was not limiting in the utilization of folate for DNA‐thymine synthesis. The marrow erythroid maturation defect remains unexpl

ISSN:0361-8609    

DOI:10.1002/ajh.2830050106

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

年代:1978

数据来源: WILEY

摘要:

AbstractFalsely high microhematocrits are found when compared to Coulter S hematocrit levels. This increase is exaggerated when hematocrit levels are increased and the cells are hypochromic and microcytic. Polycythemic patients monitored with microhematocrits may be phlebotomized incorrectly because of this abnormality. We suggest the use of Coulter S hematocrits or spectrophotometric hemoglobin levels to monitor polycythemic patients.

ISSN:0361-8609    

DOI:10.1002/ajh.2830050107

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

年代:1978

数据来源: WILEY

摘要:

AbstractA patient presented with lymphopenia, anergy, hypogammaglobulinemia and hypersplenism. Histologic examination of the spleen and lymph node revealed noncaseating sarcoid‐like granulomas. Despite a significant rise in circulating lymphocytes after splenectomy there was in vivo and in vitro evidence of B‐ and T‐lymphocyte dysfunction. A histologic picture mimicking sarcoidosis may occur in patients with immune deficiency. The granulomatous proliferation may represent an altered host response to an

ISSN:0361-8609    

DOI:10.1002/ajh.2830050108

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

年代:1978

数据来源: WILEY

摘要:

AbstractA patient with familial erythrocytosis associated with Hemoglobin Rainier, and previously treated with32P, developed myelofibrosis with a hyperdiploid chromosome clone in the myeloid cells (51, XX, +1, 2q‐(q33), +6, +9, +11, ‐19, +20q +,+ mar 1). This transformation from a benign disorder of differentiated erythrocytes to a malignant disorder may have been secondary to radiophosphorus ther

ISSN:0361-8609    

DOI:10.1002/ajh.2830050109

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

年代:1978

数据来源: WILEY

摘要:

AbstractA teenage boy with mixed‐cellularity Hodgkin disease presented with severe anemia secondary to pure red cell aplasia of marrow without evidence of lymphomatous infiltration or hemolysis. In vitro studies of the patient's serum demonstrated an inhibitor of erythropoietin activity which appeared to be an IgG but which did not directly bind erythropoietin. The patient's anemia resolved and the inhibitor disappeared following chemotherapy for Hodgkin disease. Presumably, the inhibitor was directed at a very early stage of red blood cell production. This phenomenon may be related to other autoimmune manifestations occasionally seen in patients with lymphomas. The case is presented to bring attention to the unusual occurrence of red cell aplasia in Hodgkin disease. Several hypotheses concerning significance and etiology of the anemia are detaile

ISSN:0361-8609    

DOI:10.1002/ajh.2830050110

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

年代:1978

数据来源: WILEY

ISSN:0361-8609    

DOI:10.1002/ajh.2830050111

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

年代:1978

数据来源: WILEY