摘要:

AbstractA combination of 2 forms of thalassemia has been observed in a member of a South Carolina family. The proposita, a 34‐year‐old black female with hemolytic anemia, had over 50% fetal hemoglobin, an elevated level of hemoglobin A2, and in vitro imbalance in chain synthesis. Family studies revealed a δβ‐thalassemia heterozygosity in her mother and 2 sibs and a β‐thalassemia heterozygosity in her son. The fetal hemoglobin of the δβ heterozygotes was of theGγ type (i.e., the γ chain had glycine in position 136). Consequently, it may be concluded that the propositus hasGγ‐δβ‐thalassemia‐β+thalassemia; this is the first time that such a combination has been recognized. In 3 members of another family this same type ofGγ‐δβ‐thalassemia occurred in combination with Hb S, whereasGγ‐β°‐HPFH (hereditary persistence of fetal hemoglobin) and Hb S were present in 1 member of a third family. Clinical features and laboratory findings were specific for each condition and permitted a clear distinction betweenGγ‐δβ‐thalassemia andGγ‐β°‐HPFH. The discussion correlates these findings with data from 80 persons in 30 families

ISSN:0361-8609    

DOI:10.1002/ajh.2830030101

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

年代:1977

数据来源: WILEY

摘要:

AbstractThe prevalence of cholelithiasis in Jamaican adults with SS disease was studied by plain abdominal radiograph in 206 patients and by oral cholecystogram in 126 (61%) of these patients. Gallstones were found in 57 (28%) of patients, were more common in females than males, and increased with age and hemolytic rate. The majority of gallstones were visible on the plain abdominal radiograph, only 17% of patients with gallstones having only radiolucent stones. Nonfunctioning oral cholecystograms were common (10%) in agreement with observations by previous workers. Gallstones were noted in the common bile duct in 2 patients. In general there was no clear relationship between the presence of cholelithiasis and clinical symptomatology. Complications, such as pancreatitis and malignant change in the gall bladder, recognized to be associated with cholelithiasis in the general population, have not been clearly related to cholelithiasis in SS disease. More information is needed before a logical policy can be evolved for surgical intervention in cholelithiasis in SS disease.

ISSN:0361-8609    

DOI:10.1002/ajh.2830030102

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

年代:1977

数据来源: WILEY

摘要:

AbstractStarch gel electrophoretic patterns of 26 enzymes (corresponding to 36 gene loci) were examined in hemolysates of erythrocytes from 11 first‐trimester and midtrimester human fetuses (65‐138 gestation days). The zymograms of 16 enzymes were identical in fetal and control adult red cells. Six enzymes (enolase, guanylate kinase, lactate dehydrogenase, nucleoside Phosphorylase, phosphofructokinase, hexokinase) showed differences in the staining intensity of certain isozyme zones as compared with the controls. Also, the fetal red cell zymograms, in contrast to those of adults, contained the mitochondrial forms of isocitric dehydrogenase and glutamic oxaloacetic transaminase as well as more definite zones of phosphoglucomutase‐3. Finally, some of the isozymes of uridine diphosphate kinase in the fetal cells had slightly retarded mobility. These observed differences between fetal and adult red cells could reflect the expression of a different program of protein synthesis in red cells of the fetuses or the epigenetic modifications of isozymes in immature red

ISSN:0361-8609    

DOI:10.1002/ajh.2830030103

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

年代:1977

数据来源: WILEY

摘要:

AbstractTime course examination on human marrow erythxoid colony formation was carried out in methylcellulose culture by scoring colonies with clear signs of hemoglobinization. The colonies were arbitrarily divided into 3 categories: small colony, containing fewer than 50 cells; medium‐sized colony, containing 50‐500 cells; and large colony, containing more than 500 cells. The majority of the large colonies assumed the morphology of bursts consisting of several subcolonies. Small colonies became hemoglobinized, reached their peak on Day 4, and rapidly disappeared. Medium‐sized colonies gradually increased, reached their peak on Day 8, and then slowly declined. Large colonies became hemoglobinized beginning on Day 11 and continued to be present until Day 18.We then examined the cell sizes of the erythropoietic precursor using sedimentation velocity analysis. Bone marrow nucleated cells were separated in a Staput apparatus and fractions of equal volume were analyzed for their content of colony‐forming units (CFU). The mean of modal sedimentation velocities of late erythroid precursors, CFU‐E4(CFU‐E for 4‐day‐old small colonies), was 6.46 mm/hour, while that of CFU‐E14(CFU‐E for 14‐day‐old large colonies) was 5.17 mm/hour.These studies demonstrate the heterogeneity of human erythroid precursors assayable in culture and emphasize the necessity for clear standardizat

ISSN:0361-8609    

DOI:10.1002/ajh.2830030104

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

年代:1977

数据来源: WILEY

摘要:

AbstractTwo mechanisms are felt to be responsible for the production of anemia in patients with chronic diseases. The first is failure to produce adequate amounts of erythropoietin (EP), and the second is failure to deliver iron to the bone marrow in amounts sufficient to support normal erythropoiesis. In order to evaluate these hypotheses we studied urine and serum EP levels and levels of 2,3‐diphosphoglycerate in normal subjects, in patients with the anemia of chronic diseases, in patients with chronic liver disease, and in patients with a variety of other anemias. Based on the results, we propose first that insufficient production of EP is one of the major mechanisms responsible for anemia in patients with chronic diseases. Second, insufficient production of EP is, in part, responsible for anemia seen in patients with chronic liver disease. Third, serum and urine EP levels decrease with aging, and this correlates with the fall of hemoglobin levels seen in older normal subject

ISSN:0361-8609    

DOI:10.1002/ajh.2830030105

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

年代:1977

数据来源: WILEY

摘要:

AbstractSerum from patients with chronic renal failure (CRF serum) contains a substance inhibitory to erythropoiesis in vitro. This paper explores the mechanism of the inhibition. Four experiments were performed to evaluate the effects of CRF serum on erythropoietin (EP). In the first 2 experiments, the effect of exposure of EP solutions to CRF serum was evaluated using the plethoric mouse EP assay system and a tissue culture system containing normal dog marrow cells. In the third study, dog marrow cells were preincubated with CRF serum before being stimulated with EP. Finally, EP‐dose response curves were constructed in the dog marrow tissue culture system and analyzed using an enzyme kinetic model. The results show no evidence of inhibition or inactivation of EP by CRF serum, although in vitro heme synthesis is clearly depressed in the presence of CRF serum. We conclude that CRF serum inhibits erythropoiesis by directly, although reversibly, impairing the ability of erythroblasts to synthesize hem

ISSN:0361-8609    

DOI:10.1002/ajh.2830030106

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

年代:1977

数据来源: WILEY

摘要:

AbstractThe effect of amphotericin B on human and murine hematopoiesis was studied in vitro using assays for erythroid and myeloid colony‐forming cells. Amphotericin consistently inhibited colony formation by erythroid and granulocyte‐monocyte progenitor cells. Clear effects were observable at amphotericin concentrations of 1.0 μg/ml, and concentrations of 2.0 μg/ml caused approximately 50‐60% inhibition of cloning of both murine and normal human bone marrow. These data suggest that amphotericin, in concentrations achieved in therapy, can impair hematopoiesis by a direct effect on precurso

ISSN:0361-8609    

DOI:10.1002/ajh.2830030107

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

年代:1977

数据来源: WILEY

摘要:

AbstractGlycoproteins have been discovered to be important to platelet function both in normal and pathological states. We have studied membrane glycoprotein patterns in 16 patients with various myeloproliferative disorders. There was an abnormal ratio of glycoprotein I glycoprotein IV in patients with myeloproliferative disease compared with controls. There was no discernible correlation between glycoprotein pattern and aggregation response or platelet count, but patients with megathrombocytes had higher values for glycoprotein IV than those without megathrombocytes. These experiments suggest that patients with myeloproliferative disorders may have alterations in membrane glycoproteins that could alter platelet function.

ISSN:0361-8609    

DOI:10.1002/ajh.2830030108

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

年代:1977

数据来源: WILEY

摘要:

AbstractThree successive patients with thrombotic thrombocytopenic purpura (TTP) were treated by exchange transfusion on a total of 5 occasions in addition to receiving more conventional therapy such as corticosteroids, platelet inhibitors, heparin, and splenectomy. Dramatic relief of symptoms and objective improvement in hematologic values occurred within 24 hours of each exchange. One patient subsequently died of complications resulting from a massive hemorrhage, but in hematologic remission. The remaining 2 are now apparently well. These observations appear to confirm recent reports of a beneficial effect of exchange transfusion in TTP. Although the reason for its effectiveness is not yet known, exchange transfusion may be a highly useful adjunct in the treatment of this disorder and further evaluation of this form of therapy appears warranted.

ISSN:0361-8609    

DOI:10.1002/ajh.2830030109

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

年代:1977

数据来源: WILEY

摘要:

AbstractIncreased numbers of committed leukocyte precursor cells were observed in the peripheral blood of children with hereditary spherocytosis in a study that made use of the in vitro leukocyte colony‐forming technique. Five children with hereditary spherocytosis had increased numbers of peripheral blood colony‐forming unit cells (CFUc) before splenectomy (range 8.7‐27.2 CFUc/106cells/plate; normal range 0‐5 CFUc/106cells/plate), but no CFUc were found 24‐120 hours after splenectomy. Six additional cases of hereditary spherocytosis (presplenectomy) had increased CFUc in their peripheral blood (range 7.5‐61.3 CFUc/106cells/plate). Three patients with other hemolytic anemias associated with increased hematopoietic activity also had increased peripheral blood CFUc values. Five cases of hereditary spherocytosis were studied two or more months after splenectomy and generally had normal numbers of CFUc in their blood (range 0‐6.4 CFUc per 106cells/plate). In all spleens there was evidence of extramedullary hematopoiesis. Four patients with elliptocytosis and one patient with hereditary spherocytosis, all with intact spleens, had normal numbers of peripheral blood CFUc. These studies document increased peripheral blood CFUc in hereditary spherocytosis with hemolytic anemia of moderate severity and implicate the degree of hematopoietic stress as a factor in the regulation of increased CFUc emerging from the bone marrow in these patients. As an indicator of hematopoietic stress, an elevated reticulocyte count may reflect to some degree the presence of increased CFUc circulating in the peripheral blood of unsplenectomized hereditary spherocyt

ISSN:0361-8609    

DOI:10.1002/ajh.2830030110

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

年代:1977

数据来源: WILEY