摘要:

AbstractActivities of 21 red cell enzymes were measured in 15 units of CPD‐A1 anticoagulated blood before and after 35 days of storage. Paired t‐tests revealed that five of the enzyme activities decreased significantly after 35 days of storage (P<0.05) while four increased significantly after storage (P<0.05). The most striking change was in phosphofructokinase (PFK), which decreased an average of 33% (P<0.005). Mean lactate dehydrogenase activity increased to 128% of its zero‐day activity (P<0.005). Percent red cell survival, determined by reinfusion of a51Cr‐tagged aliquot of stored blood into the autologous donor, was highly correlated with 35‐day ATP levels (r = 0.97; P<0.001). No significant relationships between two measures of red cell survival and any of the nine significantly altered enzymes were found.Studies of reactivation of PFK in hemolysates using 4 mM concentrations of numerous compounds indicate that the compounds or combinations of compounds used which reactivate PFK significantly all contain at least one high‐energy phosphate group. These data suggest that loss of PFK activity during storage may be due to loss of organic phosphates. Whether the changes described have an important role in blood transfusion remains to be

ISSN:0361-8609    

DOI:10.1002/ajh.2830130102

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

年代:1982

数据来源: WILEY

摘要:

AbstractAragon et al [4] reported that rat erythrocytes can be cross‐linked and permeabilized without significant inactivation or alteration of several enzymes in the glycolytic pathway. If this is the case, in situ kinetic analysis of the red cell enzymes in normal human red cells and abnormal red cells associated with hemolytic problems could be performed. However, we found that the treatment of human red cells with several bifunctional cross‐linking reagents under various conditions always caused a certain extent of inactivation and a change in kinetic properties of glucose‐6‐phosphate dehydrogenase, 6‐phosphogluconate dehydrogenase, and lactate dehydrogenase. Thus, the cross‐linking and permeabilization method, as it stands, is not satisfactory for in situ kinetic analysis of red c

ISSN:0361-8609    

DOI:10.1002/ajh.2830130103

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

年代:1982

数据来源: WILEY

摘要:

AbstractTwo families with Hb E diseases are described. In the Laotian family S, three homozygous Hb E were found. In the Vietnamese family H, double heterozygous Hb E‐α‐thalassemia‐2 and Hb E‐Hb H diseases were found. Anemia or hemolysis was absent in Hb E carriers, unless complicated by iron deficiency, the presence of severe α‐thalassemia gene (Hb H disease), or oxidative drug (paraaminosalicylic acid). Moreover, iron deficiency or concurrent α‐thalassemia genes resulted in a decreased amount of Hb E in its heterozygous carriers. Mild microcytosis and hypochromia were observed in Hb E heterozygotes, whereas the microcytosis and hypochromia were more pronounced in Hb E homozygotes. Globin chain synthesis studies yielded unbalanced α/non‐α ratios in both heterozygotes and homozygotes (average ratios were 1.13 and 1.56, respectively). The unbalanced biosynthetic ratios with microcytosis and hypochromia in Hb E carriers represented a β‐thalassemia phenotype, which could be a result of reduced synthesis of βE‐globin mRNA, as suggested by rece

ISSN:0361-8609    

DOI:10.1002/ajh.2830130104

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

年代:1982

数据来源: WILEY

摘要:

AbstractAn entirely in vitro technique was employed to characterize hemoglobin‐iron metabolism by human macrophages obtained by culture of blood monocytes and pulmonary alveolar macrophages.Macrophages phagocytized about three times as many erythrocytes as monocytes and six times as many erythrocytes as pulmonary alveolar macrophages. The rate of subsequent release of59Fe to the extracellular transferrin pool was two‐ to fourfold greater for macrophages as compared to the other two cell types. The kinetics of59Fe‐transferrin release were characterized by a relatively rapid early phase (hours 1‐‐4) followed by a slow phase (hours 4–72) for all three cell types. Intracellular movement of iron was characterized by a rapid shift from hemoglobin to ferritin that was complete with the onset of the slow phase of extracellular release. A transient increase in59Fe associated with an intracellular protein eluting with transferrin was also observed within 1 hour after phagocytosis. The process of hemoglobin‐iron release to extracellular transferrin was inhibited at 4°C but was unaffected by inhibitors of protein synthesis, glycolysis, microtubule function, and microfilament function. These data emphasize the rapidity of macrophage hemoglobin iron metabolism, provide a model for characterization of this process in vitro, and in general confirm data obtained utilizing in vivo

ISSN:0361-8609    

DOI:10.1002/ajh.2830130105

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

年代:1982

数据来源: WILEY

摘要:

AbstractTwenty‐seven patients were seen and followed at our Sickle Cell Center over a period of seven years. Their clinical, hematological, and biochemical features were determined and compared to those of patients with sickle cell anemia who were concurrently investigated. The data indicate that the mild anemia of hemoglobin (Hb) SC disease is slightly microcytic and hyperchromatic. Parameters of hemolysis and the complications of chronic hemolytic anemia (cholelithiasis, leg ulcers, hepatomegaly, and cardiomegaly) are milder in Hb SC disease than in sickle cell anemia. Asplenia and its sequelae (increased platelet count and reduced serum IgM levels) are less frequent in Hb SC disease. Cerebrovascular accidents and the decreased leukocyte alkaline phosphatase scores are similar in both diseases. Thromboembolic complications, retinopathy, and renal papillary necrosis are more frequent in Hb SC diseas

ISSN:0361-8609    

DOI:10.1002/ajh.2830130106

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

年代:1982

数据来源: WILEY

摘要:

AbstractTwo separate processes of putative red‐cell destruction in penicillin‐induced immune hemolysis were measured simultaneously by a rapid (3 hour) assay utilizing51Cr‐labelled red blood cells (RBC). Antibody‐dependent, cell‐mediated cytotoxicity (ADCC) was estimated by release of51Cr; and antibody‐dependent phagocytosis (ADPh) by quantitation of51Cr uptake into mononuclear phagocytes as well as by counts of engulfed RBC. Attacking cells were obtained by Ficoll‐Hypaque separation of peripheral blood from normal donors. Phagocytosis as well as lysis were proportional to anti‐penicillin antiserum concentration, to incubation time, and to the concentration of the attacking cells. Enrichment of mononuclear phagocytes in the attacking cell population by albumin gradient separation led to an increase in phagocytosis as well as in cytotoxicity. Depletion of mononuclear phagocytes resulted in a decline in both processes. Dilution of antiserum abolished ADCC but affected ADPh only slightly. Iodoacetate as well as colchicine inhibited both activities. These results indicate that both processes may be operative in the immune destruction

ISSN:0361-8609    

DOI:10.1002/ajh.2830130107

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

年代:1982

数据来源: WILEY

摘要:

AbstractThe complete remission (CR) rate in children with acute lymphoblastic leukemia (ALL) who received vincristine (VCR) plus prednisone (PRED) alone for induction is>90%, and has not improved with the use of more intensive regimens. In adults with ALL, using the same regimen, a CR rate of only 50% is frequently quoted, and is apparently based mainly on unpublished data on only 13 patients from an early Acute Leukemia Group B protocol. We reviewed the case records of 43 adults with previously untreated ALL seen between July 1, 1966 and December 31, 1978 who received VCR + PRED alone for induction. A CR rate of 66% was achieved and compares to an overall figure of 61% from 162 patients collected from the literature. The CR rate was age dependent with 21/29 (76%)<50 years of age achieving a CR compared to 4/19 (29%) who were<50 years of age (P<0.02). The median duration of CR was 8.3 months. For those who achieved a CR, the median survival was 19 months compared to five months for those who either failed to achieve a CR or who achieved only a partial remission (P = 0.004). All patients who achieved a CR received maintenance therapy, and approximately one‐half also received CNS prophylaxis with intrathecal methotrexate. The incidence of isolated CNS relapse was 11% in those who received CNS prophylaxis compared to 20% in those who did not.From these data and a review of the literature, we conclude that approximately two‐thirds of adults with ALL will achieve a CR with the use of VCR + PRED alone. The median duration of CR, however, is

ISSN:0361-8609    

DOI:10.1002/ajh.2830130108

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

年代:1982

数据来源: WILEY

摘要:

AbstractA patient with periodic neutropenia exhibited simultaneous monocytopenia, and epinephrine infusion revealed no monocytes in the marginating pool during neutropenia. Lymphocytes, eosinophils, and platelets also fluctuated periodically, but serial bone marrow studies and epinephrine infusion data indicate these fluctuations could have represented epiphenomena rather than a more global form of periodic hematopoiesis. Bone marrow descriptions of most cases of periodic neutropenia have indicated a “maturation arrest” at the promyelocyte or myelocyte stage prior to development of neutropenia; peripheral blood monocytes are usually normal or fluctuate out of phase with neutrophils. In the present case, “maturation arrest” occurred at the myeloblast stage, and neutrophils and monocytes cycled together. Morphologically normal eosinophilopoiesis with a mean eosinophil to erythroid ratio in the marrow of 0.27 ± 0.10 (SD) persisted despite a sustained disappearance of promy

ISSN:0361-8609    

DOI:10.1002/ajh.2830130109

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

年代:1982

数据来源: WILEY

摘要:

AbstractA previously healthy woman developed severe, periodic neutropenia after ingestion of phenylbutazone. Oscillations in the monocyte count and hemoglobin concentrations also occurred. The neutropenic episodes were associated with severe bacterial infections requiring hospitalization. Lithium induced a transient interruption in the neutropenia, but continued use was ineffective. Prednisone in a dosage of 100 mg daily successfully interrupted the neutrophil cycling and prevented infection. The patient has remained in remission on 10 mg of prednisone on alternate days.

ISSN:0361-8609    

DOI:10.1002/ajh.2830130110

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

年代:1982

数据来源: WILEY

摘要:

AbstractBy their nature, antibody molecules exhibit a wide range of binding specificities. The antigen‐binding properties of the antibody reside entirely in the amino‐terminal portion of the molecule, termed the variable domain. Structurally, the combining site specificity is determined by the amino‐acid residues within 6 short lengths, 3 each in the heavy and light chains, of unusually variable sequence. The hypervariability of 2 of these lengths arises from the somatic recombination of short gene segments into a single stretch of mRNA which encodes the entire variable region of 1 polypeptide chain. For example, a V gene segment that codes for most of the variable portion of a light chain, can combine with one of a number of much shorter J gene segments to create the complete variable region gene. In heavy chain genes, a third element, the D gene segment, increases the potential for diversity even further. A mechanism has been proposed by which variability occurs at the point where 2 gene segments join. Thus, a large part of the generation of antibody diversity occurs in the somatic recombination of small genetic ele

ISSN:0361-8609    

DOI:10.1002/ajh.2830130111

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

年代:1982

数据来源: WILEY