摘要:

AbstractErythrocytes from adults and newborn infants (at term and premature) were separated by Percoll density gradient centrifugation into four fractions of increasing density. Glycolytic enzymes, especially the age‐dependent ones, hexokinase (EC 2.7.1.1, HK), pyruvate kinase (EC 2.7.1.40, PK), and glucose‐6‐phosphate dehydrogenase (EC 1.1.1.49, G6PD) were studied during reticulocyte maturation and further red cell senescence.Analysis of the fraction with lowest density showed an almost linear and steep decline of HK, PK, and G6PD activity with a decreasing number of reticulocytes. In the next three fractions of increasing density, the activity decline was far less. These data are therefore illustrative for a biphasic activity decay pattern of HK, PK, and G6PD during both adult and neonatal red cell aging.The strong decline in HK activity could not be ascribed to the disappearance of a particulate (mitochondrial) bound fraction of the enzyme during reticulocyte maturation. All hexokinase activity in human reticulocytes was found to be cytosolic in contrast with rabbit reticulocytes in which 70% of HK activity was partic

ISSN:0361-8609    

DOI:10.1002/ajh.2830200302

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

年代:1985

数据来源: WILEY

摘要:

AbstractConcomitant inheritance of α‐thalassemia in patients with β°‐thalassemia/hemoglobin (Hb) E disease was detected by restriction endonuclease DNA mapping. Among 42 patients with β°‐thalassemia/Hb E disease, seven were found to have an α‐thalassemia‐2 haplotype. Of these, five belonged to the rightward or 3.7‐kb type of α‐thalassemia‐2 and the remaining two the leftward or 4.2‐kb type. All the seven patients with α‐thalassemia‐2 haplotype had hemoglobin levels of 7.4 g/dl or above; those without detectable α‐thalassemia had hemoglobin levels both higher and lower than 7.4 g/dI. The latter attended the clinic regularly, the former did occasionally. These findings suggest that concomitant inheritance of α‐thalassemia can alleviate the severity of β°‐thalassemia/Hb E disease. Failure to find α‐thalassemia‐1 haplotype in these patients suggests that concomitant inheritance of α‐thalassemia‐1 with β°‐thalassemia/Hb E might lead to so mild a condition that the individuals do not present clinically. The fact that many patients without a detectable α‐thalassemia haplotype also had hemoglobin levels of 7.4 g/dl or higher suggests that there are additional factors res

ISSN:0361-8609    

DOI:10.1002/ajh.2830200303

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

年代:1985

数据来源: WILEY

摘要:

AbstractGranulopoiesis was investigated in five patients with congenital neutropenia (CN) (one Kostmann type, four benign forms). In semisolid agar culture, the marrow cells of all five patients produced normal numbers of CFU‐c (colony‐forming unit‐culture). The size and classification of colonies were normal. In suspension culture in vitro with exogenous colony‐stimulating factor (CSF) generated from omental‐conditioned medium (OMCM), the myeloid precursors of all patients could proliferate and differentiate into normal polymorphonuclear neutrophils (PMNs). But in the absence of exogenous CSF, myeloid precursors of the patient with Kostmann‐type CN did not proliferate or differentiate into PMNs at all. In the four patients with benign neutropenia, however, PMNs were found even without exogenous CSF similar to normal individuals. These results suggest that patients with CN may have normal granulopoietic stem cells with normal proliferative and differentiating capacity in response to exogenous CSF.When a small amount of normal human serum was added to normal marrow cultures stimulated by exogenous CSF, the colony growth increased in a superadditive manner. The enhancing activity of serum from neutropenic patients differed from that of normal serum. Especially, the addition of serum from the patient with Kostmann type CN to normal marrow cultures did not show this enhancement effect. The sera of patients with benign neutropenia had less enhancement effect than did normal control serum. These findings might be interpreted as showing an imbalance between CSF enhancer and inhibitors in the pati

ISSN:0361-8609    

DOI:10.1002/ajh.2830200304

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

年代:1985

数据来源: WILEY

摘要:

AbstractPolymorphonuclear leukocytes (PMN) demonstrate altered function during acute infections and after administration of corticosteroids. We questioned whether or not such changes are due to population shifts from functionally different compartments of the granulocyte pool. Volunteers were given epinephrine to induce demargination or hydrocortisone (HC) to promote egress of PMN from the bone marrow. PMN obtained before and after drug administration were compared for adherence, chemotaxis, luminol‐enhanced chemiluminescence, and total content and release of lactoferrin (LF), myeloperoxidase (MPO), and β‐glucuronidase (β‐glu). Epinephrine induced a significant neutrophilia of mature PMN (segmented neutrophils), but there were no changes in function or granule protein content. HC induced a significant neutrophilia with segmented neutrophils and immature PMN (bands). Circulating PMN obtained 4 hr after HC administration demonstrated less adherence, increased chemiluminescence, increased MPO release, and decreased MPO content. Band neutrophils, however, were more adherent than segmented PMN and showed a similar decrease in adherence following HC in vivo. Thus alteration of PMN adherence following intravenous corticosteroids is not due to an influx of immature neutrophils. On the other hand, it is possible that MPO content and release and capacity for oxidative metabolism change as PMN

ISSN:0361-8609    

DOI:10.1002/ajh.2830200305

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

年代:1985

数据来源: WILEY

摘要:

AbstractSeven normal human peripheral blood cell fractions (buffy coat, mononuclear cells, non‐T, T, Fc‐IgM receptor‐depleted T‐lymphocyte, Fc‐IgG receptor‐depleted T‐lymphocyte, and autologous rosette‐forming T‐cell‐depleted T‐lymphocyte subpopulations) treated with phytohemagglutinin (PHA) were examined for the production of granulocyte‐macrophage colony‐stimulating activity (CSA). It was found that medium conditioned by a T‐lymphocyte subpopulation depleted of autologous rosette‐forming T‐cells (Tar cells, a postthymic precursor subpopulation that inhibits Ig synthesis) stimulated colony‐forming units of granulocyte and macrophages (CFU‐GM) to a greater extent than did the other conditioned media (CM) analyzed. Based on this finding, CM from an enriched Tar subpopulation was prepared and thus showed that PHA‐treated Tar cells release a factor capable of inhibiting CFU‐GM growth. The inhibitory activity of this factor persisted after heat inactivation, suggesting that cause of the colony‐inhibiting activity (CIA) is other than interferon. Further studies revealed that Tar‐derived inhibitory factor acts either directly upon CFU‐GM or via monocytes/macrophages (Mϕ/Ma), enhancing CIA, and not the level of CSA production by Mϕ/Ma. The overall data are interpreted as demonstrating the presence of CIA in a specific T‐lymphocyte subpopulation that may represent a new relationship betwee

ISSN:0361-8609    

DOI:10.1002/ajh.2830200306

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

年代:1985

数据来源: WILEY

摘要:

AbstractDisseminated intravascular coagulation (DIC) is a frequent occurrence in acute promyelocytic leukemia (APL), especially after onset of chemotherapy. We have used a human promyelocytic leukemic established cell line (HL‐60) and various other human leukemic cells to investigate the effect of cytotoxic drugs on generation of procoagulant activity (PCA). The results indicate that, unlike normal human peripheral blood monocytes and certain other cell types where PCA induction requires active mRNA and protein synthesis, in HL‐60 cells, compounds such as actinomycin D, puromycin, and cytosine arabinoside and a variety of other cytotoxic agents, induced generation of a potent PCA.Although different in its mechanism of induction, this HL‐60 cell PCA was similar, and may be identical, to mononuclear cell tissue factor. The PCA induction was rapid and preceded the lytic effect of the drugs. It was first detected on the outer cell surface but, following prolonged exposure to the drugs, upon lysis of the cells, it was also found in the extracellular medium.This in vitro effect mimics the development of DIC in patients with APL. The system may, therefore, serve as a model for the study of the cellular and molecular events associated with PCA generation by malignant promyelocytes and DIC occurrence in patients with APL and other maligna

ISSN:0361-8609    

DOI:10.1002/ajh.2830200307

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

年代:1985

数据来源: WILEY

摘要:

AbstractT‐cell subsets defined by monoclonal antibodies (OKT3, OKT4, and OKT8) were analyzed in 117 patients with monoclonal gammopathies—69 multiple myeloma (MM) (30 untreated and 39 treated), 14 Waldenström's macroglobulinaemia (WM), and 34 essential monoclonal gammopathy (EMG) patients. The percentage and absolute numbers of total T‐lymphocytes (E+, OKT3+cells) were within the normal range in all groups except for the treated MM patients, in which a decrease in the absolute number could be observed. The percentages of OKT4+cells were significantly lower in MM (35 ± 1.7) than in EMG patients (43 ± 2) and controls (50 ± 2). In contrast, OKT8 cells correspondingly increased in MM (38 ± 1.6) compared with EMG patients (29 ± 1) and controls (27 ± 1). The OKT4/0KT8 ratio was lower in MM than that in EMG patients and controls (p<0.01) and was shown to be one of the four most significant variables in a linear discriminant analysis used to distinguish between MM and EMG groups. The MM patients in clinical stage III as well as Bence‐Jones myeloma patients showed a more pronounced OKT4/0KT8 imbalance. The treatment did not influence the percent distribution of T‐cell Subpopulations.The patients with WM exhibit an alteration in the distribution of the T‐cell subsets similar to the MM patients with a T4/T8 ratio of 1.1±0.1. This imbalance was more pronounced in WM patients with monoclonal B‐lymphocytes in peripheral blood (leukaemic phase of WM).The functional significance of the altered T‐cell subsets in MM and WM patients remains to be established, though it is probable that such an imbalance plays an important role in regulating thes

ISSN:0361-8609    

DOI:10.1002/ajh.2830200308

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

年代:1985

数据来源: WILEY

摘要:

AbstractThe neoplastic cells from a patient with B‐cell prolymphocytic leukemia exhibited sheep red blood cell (SRBC) rosette formation. Immunochemistries revealed no reactivity with T‐cell monoclonal antibodies OKT 3, 4, 8, and 11. However, the neoplastic cells expressed characteristics of B‐cells including surface IgM and IgD associated with lambda light chains, Ia‐like antigenicity, and reactivity to monoclonal antibodies against B‐cell antigens B1, B2, and B4. Inhibition procedures revealed that SRBC rosette formation was the result of binding activity of surface immunoglobulin to SRBC. Pretreatment of the leukemic cells with antihuman IgM or lambda antisera, or pronase or trypsin blocked rosette formation with SRBC. Circulating antibody to SRBC was also suspected by the fact that preincubation of SRBC with heat‐inactivated patient's serum resulted in agglutination

ISSN:0361-8609    

DOI:10.1002/ajh.2830200309

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

年代:1985

数据来源: WILEY

摘要:

AbstractThe Passovoy defect is a recently characterized hemorrhagic diathesis. We describe a patient with a febrile illness, possibly from Epstein‐Barr (EB) virus, who acquired this defect transiently. Prothrombin time; assays for factors VIII, IX, XI, XII; and Fletcher (prekallikrein) and Fitzgerald (high molecular weight kininogen) factors were normal. No definite circulating inhibitor could be demonstrated. The transient Passovoy defect could possibly be ascribed to the infectious process or sulfisoxazole, which the patient had receive

ISSN:0361-8609    

DOI:10.1002/ajh.2830200310

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

年代:1985

数据来源: WILEY

摘要:

AbstractTritiated thymidine labeling indices (LI), although useful in diagnosis and prognosis of multiple myeloma, have not found wide‐spread application because autoradiographic analysis is diffucult and time consuming. Using a monoclonal antibody (BU‐1) reactive with 5‐bromo‐2‐deoxyuridine (BrdUrd), we have developed an immuno‐fluorescent procedure that allows DNA S‐phase measurements to be determined in 4 hr. Plasma cells are easily identified by reactivity with a fluorescein isothiocya‐nate‐conjugated antihuman immunoglobulin, and cells in DNA S phase are detected via BU‐1 and a rhodamine‐conjugated antimouse immunoglobulin. Results using this method on 12 patients with multiple myeloma compare favorably (correlation coefficient 0.84), with those obtained by tritiated thymidine. This immunofluorescent slide method will facilitate application of labeling indices as a clinical test to measure disease activity in patients with multiple myeloma and other

ISSN:0361-8609    

DOI:10.1002/ajh.2830200311

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

年代:1985

数据来源: WILEY