摘要:

AbstractPeripheral blood mononuclear cells from normal adults, normal newborn infants, and from newborn and adult subjects with one of three γ chain variants (GγF‐Malta I,GγF‐Port Royal,AγF‐Hull) were cultured in vitro with erythropoietin. The35S‐methionine‐labelled hemoglobin from 13‐ to 15‐day‐old BFUe‐derived colonies was studied by chromatography on columns of DEAE‐cellulose and the quantities of Hbs A2, Fx, and F0determined. The percentages ofGγ andAγ chains in isolated Hb Fxand Hb F0were determined using high‐performance liquid chromatography (HPLC) of the tryptic peptides of these proteins. Calculation of these percentages was based on the total activities of theGγT‐15 andAγT‐15 peptides which contain one (35S‐labelled) methionyl residue each and can be separated by the HPLC procedure.The data show an increased synthesis of Hb F in the „adult”︁ colonies and a decreased synthesis in the „newborn”︁ colonies. TheGγ toAγ ratio of the Hb F from adult colonies varied greatly. The percentages ofGγ andAγ chains in the Hb F from adult colonies correlated with the percentages in the Hb F isolated from the Hb F of circulating red blood cells. TheGγ toAγ ratio in the Hb F from newborn colonies was high as in the Hb F from cord blood samples.Gγ andAγ chain abnormal Hb F variants were readily detectable in colonies from both adults and newborn. TheGγ toAγ ratio in the Hb F of colonies of adult Hb F‐Malta I and Hb F‐Hull heterozygotes approached 1, but that of adult Hb F‐Port Royal heterozygotes remained about as high as in colonies from newborn heterozygotes. The percent Hb F‐Port Royal in the Hb F of adult colonies was twice that in the Hb F of newborn colonies.These results are discussed in the light of information from recent detailed studies of genomic DNA assuming controlling functions for the segments

ISSN:0361-8609    

DOI:10.1002/ajh.2830090202

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

年代:1980

数据来源: WILEY

摘要:

AbstractA new slow‐moving hemoglobin was found in low proportion in an asymptomatic black woman. Isoelectric focusing helped to distinguish it from other hemoglobins with similar electrophoretic mobility, and amino acid analysis showed that aspartic acid α6 (A4) had been replaced by asparagine. Oxygen affinity was increased, but the Bohr and DPG effects were normal. Stability of the purified hemoglobin was decreased, but that of hemolysates was normal. Abnormal oxygen affinity of this variant, and that of hemoglobin Sawara (α6(A4)Asp→Ala), may reflect loss of a salt bridge between Asp α6 and Lys‐α127(H10) which would tend to favor the high‐affinity R conformation of t

ISSN:0361-8609    

DOI:10.1002/ajh.2830090203

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

年代:1980

数据来源: WILEY

摘要:

AbstractIn order to investigate whether a common control mechanism is involved in the diminution of i antigen expression and that of Hb F content in human erythrocytes during the postnatal period, we compared changes in 72 normal infants aged from 0 to 12 months. The proportion of hemoglobins (Hb F, Hb A, Hb A2) and the quantitation of „i”︁ antigen were determined on the total population of red blood cells. In addition, the percentage of individual cells containing Hb F or „i”︁ antigen or both (F cells, „i”︁ cells, and F + „i”︁ cells) were evaluated by using a rhodamine‐conjugated anti‐Hb F and a fluorescein conjugated anti‐i system on the same smear preparation, The results provided by the two most sensitive techniques (F cell counting and „i”︁ agglutinability) indicated that the curves of disappearance of Hb F and „i”︁ antigen along the 12 first months after birth were identical. A strong correlation (r = 0.97, P<0.0001) existed between the percentage of F cells and „i”︁ antigen expression. In addition, the progressive increase in Hb A2concentration was inversely correlated firstly with the proportion of Hb F and second with the expression of the „i”︁ antigen.These results suggest that the switch from fetal to adult hemoglobin and the transformation of „i”︁ antigen expression occurring during the first year

ISSN:0361-8609    

DOI:10.1002/ajh.2830090204

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

年代:1980

数据来源: WILEY

摘要:

AbstractFive of 39 (13%) women treated with adjuvant combination chemotherapy plus levamisole immunotherapy after mastectomy for Stage II or III breast cancer developed levamisole‐induced granulocytopenia. This complication occurred in each of the women between six and ten weeks after the completion of six months of combination chemoimmunotherapy when they were taking levamisole alone. Although none of the patients had an HLA B‐27 locus and leukoagglutinins could not be demonstrated, complement‐dependent, IgM mediated, peripheral destruction of granulocytes was documented using a microgranulocytotoxicity assay. In addition, a factor(s) present in serum from patients developing levamisole‐induced granulocytopenia caused suppression of bone marrow granulocyte progenitor cells (CFU‐C). The possible relationships between levamisole‐induced peripheral granulocyte destruction and bone marrow CFU‐C suppression

ISSN:0361-8609    

DOI:10.1002/ajh.2830090205

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

年代:1980

数据来源: WILEY

摘要:

AbstractTo examine the diurnal changes in circulating myeloid progenitor cells (CFU‐Cs), 15 normal human volunteers were studied. Total and differential peripheral blood leukocyte counts and CFU‐C levels, using the in vitro agar culture method, were obtained at 8 AM, 11 AM, 3 PM, and 8 AM the following morning (in nine subjects) or only at 8 AM and 3 PM (in six subjects). On the average, an approximate twofold rise in circulating CFU‐C levels (per ml of blood) was found at 3 PM as compared to the 8 AM levels (P 0.002), and a smaller rise at 11 AM was found than at 8 AM levels (P 0.03). The ratios of CFU‐C per ml of blood to mononuclear cells per ml of blood and neutrophils per ml of blood, respectively, were significantly higher at 3 PM than at 8 AM (P 0.002 and 0.004, respectively). The rise in CFU‐C levels is usually significantly higher when compared to that in peripheral leukocy

ISSN:0361-8609    

DOI:10.1002/ajh.2830090206

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

年代:1980

数据来源: WILEY

摘要:

AbstractGranulopoietic assessment was made in 14 patients with chronic idiopathic neutropenia (CIN) whose neutrophils were consistently less than 1.5 × 109/ liter and in whom there was absence of splenomegaly and recent drug ingestion. Granulopoiesis was studied using a combination of bone marrow culture in nutrient agar and granulocyte kinetics. Agar colony growth assessed bone marrow concentration of granulocyte progenitor cells (GPC) and the proportion of GPC in DNA synthesis by in vitro3HTdR suicide. Granulocyte kinetics with in vitro DF32P labelling of patient granulocytes measured granulocyte half‐life (T1/2), turnover rate (GTR), and the circulating, marginated, and total blood granulocyte pools. The results indicated that either GPC concentration or the proportion of GPC in DNA synthesis was outside the normal range in all but one patient and decreased in ten out of 14 patients. CIN was also characterized by reduced total, circulating, and marginated blood granulocyte pools, reduced GTR, and normal granulocyte half‐life. The neutropenia appeared to be due to a variety of intra‐marrow causes, including either a reduction in the GPC compartment, a reduction in GPC proliferation, a maturation arrest, or a reduced amplication during granulopoiesis. Increased granulocyte utilization, intra‐vascular destruction or excessive margination could be excluded as possible causes of CIN in this series. Although GPC parameters tended to be reduced, suggesting a production defect, there were signs in nine patients that the bone marrow was attempting to compensate for the peripheral neutropenia. It is suggested that for a complete assessment of granulopoiesis in man, granulocyte kinetic studies need to be combined with quantitative studies of the bone marrow granulocyte progenitor compartment using the agar colon

ISSN:0361-8609    

DOI:10.1002/ajh.2830090207

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

年代:1980

数据来源: WILEY

摘要:

AbstractWaldenstrom macroglobulinemia (WMG), a proliferation of malignant monoclonal IgM secreting plasmacytoid lymphocytes in lymph nodes, spleen, and marrow, usually pursues a chronic clinical course. A patient with WMG for five years who developed pulmonary tumors consisting of plasmacytoid lymphocytes prompted a review of the literature for pulmonary manifestations of WMG.Twenty‐six males and 18 females, ranging in age from 33 to 84 years, have been reported with histologically proven pulmonary involvement by WMG. The x‐ray findings, evident in most patients when first seen, consisted of masses (22 patients), infiltrates (31 patients), and pleural effusions (19 patients). Most patients (24) had two or more of these manifestations but only five, in addition to our patient, had isolated pulmonary nodules. Isolated pulmonary infiltrates were found in ten patients and isolated pleural effusions in only four. Symptoms at the onset of pulmonary involvement included dyspnea (54%), nonproductive cough (33%), and chest pain (7%); 15% were asymptomatic. Pulmonary manifestations, like other features of WMG, respond to alkylating agents or irradiation and do not appear to affect prognosis adversely. Pulmonary involvement should be suspected in any patient with WMG who develops an abnormal chest x

ISSN:0361-8609    

DOI:10.1002/ajh.2830090208

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

年代:1980

数据来源: WILEY

摘要:

AbstractAcute lymphoblastic leukemia (ALL) developing in myelofibrosis (MF) and myeloid metaplasia (MM) is reported in two patients. In both cases, the clinical course of the „blastic crisis”︁ was rapidly progressive with little response to chemotherapy. The circulating cells were readily identified as lymphoblasts on the basis of cytology, cytochemistry, immunologic studies, and ultrastructure. In one of the cases, 40% of cells had T‐cell markers and all cells contained paranuclear acid phosphatase. In the second case, cells had a „Burkitt‐like”︁ appearance, contained multiple cytoplasmic vacuoles positive for oil red O, and one‐third of them had B‐cell markers. The development of lymphoblastic crisis in MF and MM occurs rarely, is analogous to blastic transformation in chronic granulocytic leukemia, and supports the hypothesis that myeloproliferative disorders originate from pluripotent hemat

ISSN:0361-8609    

DOI:10.1002/ajh.2830090209

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

年代:1980

数据来源: WILEY

摘要:

AbstractTwo patients with macroglobulinemia refractory to alkylating agent therapy did have an objective response to therapy with doxorubicin. For patients refractory to alkylating agents, doxorubicin is an alternative to other alkylating agents or procarbazine.

ISSN:0361-8609    

DOI:10.1002/ajh.2830090210

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

年代:1980

数据来源: WILEY

摘要:

AbstractThree types of γ chain of human hemoglobin (Hb) F have been discovered. The γ andAγ chains differ at position 136 (glycine inGγ; alanine inAγ) and are the products of nonallelic genes. TheTγ chain is found in rather high frequency in certain world populations, and is characterized by a threonyl residue at position 75 replacing the commonly occurring isoleucyl residue. Convincing evidence is present that theTγ chain is the product of an allele of theAγ chain gene, and should be renamedAγTchain. Thus, the three γ chains areGγI,AγI, andAγT.Until recently, the presence and the quantity of these chains were determined by chemical analyses of the tryptic peptides of the isolated Hb F. These procedures involve chromatographic separations or fingerprinting methods. The introduction of high pressure liquid chromatography (HPLC) has greatly facilitated the separation of these peptides. Since minute quantities are required, the latter method is ideally suited for the identification of the Hb F produced in in vitro cell colonies.Electrophoretic and chromatographic (HPLC) methods have been developed which make it possible to separate theGγ andAγ chains in small amounts of Hb F (less than 0.5 mg). These procedures allow accurate quantitation of theGγ/Aγ ratio in the Hb F and may also be suitable for in vitro biosynthetic analyses. One of the HPLC procedures has been modified so that the three types of γ chain can be quantitated in one single chromatographic experiment. Examples of separations are provided, details of some of the methods are discussed, and their applicab

ISSN:0361-8609    

DOI:10.1002/ajh.2830090211

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

年代:1980

数据来源: WILEY