摘要:

AbstractRed cell morphology was studied after the induction of echinocytic transformation by metabolic depletion, Ca2+loading, and salicylate and stomatocytic transformation with chlorpromazine. The results indicate that the red cell has an energy‐dependent shape control mechanism that allows it to counteract shape‐changing stimuli such as metabolic depletion. Albumin was found to induce stomatocytic transformation, whereas γ‐globulins induced echinocytic transformation. Loading of the red cell with calcium resulted in polymorphous membrane damages such as submembranous, “blister‐like” lesions, and membrane disintegration; the red cell age had no influence on this process. Conversely, the stomatocyte‐echinocyte transformation induced by chlorpromazine and salicylate was shifted towards echinocytes in density‐separated old red cells. Sphero‐stomatocytes were capable of echinocytic transformation with spicule formation within the red cell vacuoles, whereas sphero‐echinocytes were unable to undergo stomatocytic transformation without hemolysis. These observations may help to unravel the complexity of echinocyte‐stomatocyte transformat

ISSN:0361-8609    

DOI:10.1002/ajh.2830240102

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

年代:1987

数据来源: WILEY

摘要:

AbstractThis study examined the effects of platelet‐derived serum factors from patients with idiopathic thrombocytopenic purpura (ITP) on human umbilical cord vascular endothelial cell growth and DNA synthesis. Vascular endothelial cell growth factors were identified in the platelet extracts, and platelet‐derived vascular endothelial cell growth factors with molecular weights of less than 10,000 were also identified in the sera. These growth factors were stable even after 30 minutes of heat treatment at 56°C and acid treatment.When ITP patient serum was added to vascular endothelial cells, their growth capacity and DNA synthesis capacity were lower than when serum from normal subjects or patients with ITP in remission was added. This indicated that in ITP, impairment of vascular integrity and abnormal repair of the vascular walls that accompany thrombocytopenia may be causes of the predisposition to hemorrhage. The decrease in ITP of platelet‐derived vascular endothelial cell growth factors with molecular weights of less than 10,000 was hypothesized to be a contributory

ISSN:0361-8609    

DOI:10.1002/ajh.2830240103

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

年代:1987

数据来源: WILEY

摘要:

AbstractFor the purpose of examining the mechanisms for the overproduction of megakaryocytes and platelets in essential thrombocythemia (ET), marrow megakaryocytic colony‐forming units (CFU‐Meg) were characterized using a methylcellulose culture system in nine patients with ET, and they were compared with those of 16 control subjects and four patients with secondary thrombocytosis (ST). The number of CFUMeg per 105cells in ET was significantly higher (p<0.001) than that in the controls (5.8 times of the controls) or that in ST. ET showed endogenous CFU‐Meg (58 ± 16% of the total CFU‐Meg) that could form megakaryocytic colonies without phytohemagglutinin‐stimulated leukocyte‐conditioned medium (PHA‐LCM) as a source of Meg‐CSA. The controls and the patients with ST revealed no endogenous CFU‐Meg. The dose‐response studies with PHA‐LCM revealed that CFU‐Meg from ET are highly sensitive to low concentrations of PHA‐LCM compared to CFU‐Meg from the control and ST.Erythropoietic burst‐forming units (BFU‐E) were monitored as a control progenitor, because the patients with ET studied did not have erythrocytosis. Although five out of nine patients with ET had endogenous BFU‐E, the percentages of endogenous BFU‐E were small (4–10%). The number of BFU‐E in ET was not different from the control value. Thus, ET is associated with an enlarged CFU‐Meg compartment that shows abnormal growth patterns when cultured in vitro, while the growth abnormality of BFU‐E is small, which may underlie the hematological features of ET. The culture of CFU‐Meg should be cl

ISSN:0361-8609    

DOI:10.1002/ajh.2830240104

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

年代:1987

数据来源: WILEY

摘要:

AbstractSickle/β thalassemia is a sickling disorder of varying severity which results from compound heterozygosity for sickle cell trait and β‐thalassemia trait. Clinical and genetic studies have shown an inverse correlation between the level of hemoglobin A and the severity of the disease. It has been suggested that the level of hemoglobin A may be a function of the severity of the β‐thalassemia defect. In this study, we use molecular biological techniques to test this hypothesis. We show that the interaction of the mildest of the β+‐thalassemia genes with the sickle gene results in a high level of hemoglobin A. However, the interaction in this case resulted in a severe sickling disorder in the absence of significant anemia. We hypothesize that a mild β+‐ thalassemia gene may have two opposite effects on the clinical course of sickle/β+thalassemia: (1) A high level of hemoglobin A which probably confers a favorable antisickling effect and (2) decreased hemolysis leading to increased numbers of total circulating red cells, thereby increasing the blood viscosity and the propensity for sickling. The inheritance of heterozygous α thalassemia 2 in conjunction with the mild β+‐thalassemia gene and sickle gene in this patient may have further enhanced the latter effect and resulted in a severe

ISSN:0361-8609    

DOI:10.1002/ajh.2830240105

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

年代:1987

数据来源: WILEY

摘要:

AbstractTwenty‐one asymptomatic individuals with a mildly prolonged prothrombin time (>2 SD from the prothrombin time of the reference plasma) were found to have a mild isolated factor VII (F VII) defect (x38.8 U/dl; SD 13.2). Factor VII antigen levels were also found to be reduced (x45.5 U/dl; SD 7.8) in 13 of them. These figures were compared with those of 50 normals and 28 obligatory heterozygotes for F VII deficiency. The phenotypical behaviors in the propositi were found to be equal to those of the F VII congenital deficiency heterozygotes: the discrepant one (VII+) and the nondiscrepant one (VII−/R).Fifteen families of the propositi could also be studied, totalling 55 additional individuals; in 25 of them (ten pedigrees) a mild F VII deficiency was found showing the same phenotypical features of the corresponding propositi.We therefore believe that these individuals with mild F VII deficiency can be considered as heterozygotes for the defect, since (1) the other vitamin K‐dependent clotting factors were normal; (2) the defect is transmitted throughout the kindred with the same mode of inheritance as F VII congenital deficiency; and (3) F VII: C and F VII:Ag levels are highly comparable with those of known obligatory heterozygotes for F VII deficiency.On the grounds of a careful statistical analysis we propose a formula which allows a discrimination between the two phenotypes of the heterozygotes for F VII congenital deficiency.In addition it is suggested that sensitive tissue thromboplastins should be used to pick up these mild de

ISSN:0361-8609    

DOI:10.1002/ajh.2830240106

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe intravascular pool of human polymorphonuclear leukocytes (PMN) is composed of one compartment which is circulating and another that is marginated to the vascular endothelium. Administration of B‐adrenergic agonists leads to a rapid demargination with an increase in the circulating PMN pool. The marginating PMN has previously been stated to represent an older PMN based on a higher cytochemical alkaline phosphatase activity. With the understanding that circulating PMN are heterogeneous with respect to function and size we undertook the present study to evaluate the contribution of the marginating PMN to functional and volume‐dependent heterogeneity. We found that PMN isolated 7 min after epinephrine administration, presumably enriched by marginating PMN, were not different in volume, biochemically measured alkaline phosphatase activity, stimulated superoxide anion release, degranulation, or phagocytosis. These data suggest that the circulating and marginating pools of PMN are interchangeable and that the marginating PMN are not enriched by a particular subpopulation of

ISSN:0361-8609    

DOI:10.1002/ajh.2830240107

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

年代:1987

数据来源: WILEY

摘要:

AbstractHospitalized patients were studied prospectively in an attempt to determine whether idiopathic eosinopenia in the absence of other major changes in blood cells is rare or is more frequent than is commonly recognized. Strict criteria for eosinopenia were used; patients with more than 10.0 × 109/liter total leukocyte count, or less than 4.0, as well as those with any form of hematologic cancer, or those receiving any form of cancer chemotherapy, were excluded from the study. With those criteria and exclusions, only 24 patients with eosinopenia were found among 24,300. Twenty were receiving some form of adrenal glucocorticosteriod (steroid) and of the other four, three had serious organic diseases for which they were receiving various drugs. The remaining patient, whose primary problem was depression, could have had druginduced eosinopenia. Thus, unexpected eosinopenia appears to be a very rare event or syndrome.A normal range for eosinophils was defined from studies of 740 medical students, which was 0.015 to 0.65 × 109/liter and was quite similar to previously reported values. The effect of acute or chronic steroid administration on eosinophils in normal human subjects was studied. Confirming studies reported for man and many other mammals, eosinopenia developed promptly, but disappeared within hours unless repeated doses were given. Literature on various types of eosinopenia was reviewe

ISSN:0361-8609    

DOI:10.1002/ajh.2830240108

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe cell cycle characteristics of a newly diagnosed patient with acute nonlymphocytic leukemia (ANLL) were studied by using intravenous bromodeoxyuridine (BrdU) and our previously described “double‐label” technique. The percentage of S‐phase cells in the bone marrow (BM) biopsy were 25 % compared to 7 % from the simultaneously obtained BM aspirate. The duration of S‐phase (Ts) was determined to be 4 hr and the total cell cycle time (Tc) was 16 hr. We demonstrated that the actual clinical course of this patient's illness corresponded well with the course predicted on the basis of these cell cycle measurements. Although he achieved aplasia in response to two successive courses of induction chemotherapy, leukemic cells repopulated the marrow, producing a rapidly rising PB white blood cell (WBC) count with a T1/2 of approximately 20 hr each time. It is likely that the resistance of this patient's leukemia to therapy was a result of the rapid proliferative rate of his leukemic cells and not due to the inability of chemotherapeutic agents to kill a large number of cells. Since the measurements can be completed within 48 hr, measuring Tsand Tcwill provide a better understanding of the biological differences that exist between patients

ISSN:0361-8609    

DOI:10.1002/ajh.2830240109

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

年代:1987

数据来源: WILEY

摘要:

AbstractWe have postulated that the p‐NO2group of chloramphenicol (CAP) is the structural feature underlying aplastic anemia from this drug. In a series of studies to examine this hypothesis we have demonstrated the toxic nature of the CAP‐reduction intermediate nitroso CAP (NO‐CAP) and its damaging effect on isolated DNA in vitro. The present study was designed to examine the comparative effects of CAP, NO‐CAP, and thiamphenicol (TAP) on the integrity of DNA in intact cells. By using the alkaline elution technique of Kohn, DNA damage in the form of single strand breaks could be readily demonstrated in cultured Raji cells and in PHA‐stimulated normal human lymphocytes by small concentrations of NO‐CAP (0.05‐0.1 mM). A small but reproducible effect was observed from large concentrations of CAP (2 mM). In contrast, TAP, lacking the p‐NO2group, was

ISSN:0361-8609    

DOI:10.1002/ajh.2830240110

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe development of monoclonal antibodies to cell‐surface antigens has provided method for characterizing distinct subpopulations of T‐cells. In the present study we have quantified peripheral blood T‐cell subpopulations in premature infants born weighing less than 1,500 g (1123 ± 223 g) and ranging in gestational age from 25 to 32 weeks. The relative proportion of T4 cells in the very low birth weight (VLBW) infants was markedly higher at l week and 1 month of age (mean ± SEM; 67.5 ± 4.1 and 59.2 ± 1.6) than in adult controls (47.2 ± 1.5). The percentage of T4 cells remained elevated until 6 months of age, when it decreased to a level comparable to that in adults. In contrast, the proportion of T8 cells was significantly lower than the adult level at 1 week and 1 month of age. The T4/T8 ratio in the VLBW infants was higher at 1 week (4.3 ± 0.5) and 1 month (3.5 ± 0.2) than in adult controls (2.0 ± 0.1). Thereafter, the T4/T8 ratio decreased but was still significantly higher than that in adult controls at 6 months of age (2.6 ± 0.2). The absolute numbers of total T‐cells (T3) and T8 and T4 cells were significantly higher in VLBW infants. The numbers of T8 cells were significantly lower in the first month of life than at 3‐6 months of age. These alterations in the T‐cell subsets in the first 6 months of life suggest that postnatal T‐cell phenotypic changes in VLBW infants may parallel the T‐cell ontogenetic process which occurs during the last trimester of pregn

ISSN:0361-8609    

DOI:10.1002/ajh.2830240111

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

年代:1987

数据来源: WILEY