摘要:

AbstractIn recent studies, we observed a decrease of KMapp, an abnormally biphasic kinetics of the red cell membrane neutral phosphatase and an increased binding of hemoglobin to the membrane in various forms of β‐thalassemia. Since the gene encoding the β chain (βEchain) of hemoglobin E (HbE) is endowed with some thalassemic characteristics, we studied the erythrocyte membrane in 25 individuals with Hb E trait or disease. The apparent Michaelis‐Menten constant for p‐nitrophenylphosphate (the artificial substrate used) was significantly decreased, as in β‐thalassemia. However, the kinetics was monophasic in all the heterozygotes and in four of the homozygotes. It was biphasic only in the three other homozygotes. Vmaxwas also significantly reduced, a fact that is masked, when not reversed in β‐thalassemia, owing to the rejuvenation of the red cell population. In 5 mM phosphate buffer (pH 8.00), the binding of Hb E to the erythrocyte ghosts was increased in the homozygotes. In the heterozygotes, Hb A binding was also increased, as is the case in β‐thalassemia. This latter fact suggests that the membrane binding site(s) of hemoglobin is (are) altered. We found a highly significant increase of Hb F in EE subjects. The present study extends to the red cell membrane the β‐thalassemic phenotype associ

ISSN:0361-8609    

DOI:10.1002/ajh.2830160302

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

年代:1984

数据来源: WILEY

摘要:

AbstractBone marrow cells from three patients with idiopathic acquired sideroblastic anemia and one with X‐linked sideroblastic anemia were simultaneously cultured in plasma clot and methylcellulose cultures in order to evaluate their erythroid colony (CFUE) cloning potential in these two systems. In contrast to normals, sideroblastic anemia bone marrow cells demonstrated a marked ability to form erythropoietin (Epo)‐independent CFUEin methylcellulose culture, and were inhibited in their ability to generate CFUEin plasma clot culture even in the presence of Epo.Addition of citrated plasma to methylcellulose cultures inhibited Epo‐independent CFUEgrowth, but not Epo‐dependent growth, by both a normal and patients' bone marrow cells. These results demonstrate that bone marrow cells from some patients with sideroblastic anemia can undergo Epo‐independent and Epo‐dependent CFUEgrowth in vitro, and that there is a marked difference in CFUEgrowth depending on the type of clonal culture method used. It is suggested that the culture microenvironment, plasma, and sensitivity to Epo may be contributing factors which allow specific clones of sideroblastic anemia CFUE‐forming cells to prolife

ISSN:0361-8609    

DOI:10.1002/ajh.2830160303

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

年代:1984

数据来源: WILEY

摘要:

AbstractVigorous exercise is known to increase VIII:C and VIIIR:Ag levels transiently in normal individuals. Although exercise programs are frequently advocated in the management of hemophilia, the effects of exercise on coagulation parameters in these patients have not been well studied. Eleven hemophiliacs were exercised on a bicycle ergometer to maximum voluntary effort as evidenced by an increase in pulse, blood pressure, and plasma catecholamine (norephinephrine and epinephrine) levels. The effects of this exercise on coagulation parameters, including functional and antigenic components of the factor VIII molecule, were determined.The entire group demonstrated a decrease in mean prothrombin time (11.7 to 11.2 sec). Four mild hemophiliacs demonstrated an increase in mean VIII:C (14.5% to 17.3%), and VIII:CAg (12% to 17.8%). Changes in VIII:C and VIII:CAg were not noted in the seven severe hemophiliacs. Both severe and mild patients demonstrated significant changes in fibrinogen, factor II, and factor VII after exercise. This study indicates that submaximal exercise modifies coagulation parameters in patients with hemophilia.

ISSN:0361-8609    

DOI:10.1002/ajh.2830160304

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

年代:1984

数据来源: WILEY

摘要:

AbstractSimultaneous bone marrow scintigraphy with 99m Technetium colloids and 111 Indium transferrin was performed on 34 cases of preleukemic anemias and was shown to be of good prognostic value. Groups of different outcome were defined: for a normal and parallel uptake of the two markers, 90% of the patients died of acute leukemia; for a low Indium and high Technetium uptake, only 1 patient out of 15 died of leukemia (P<0.001). Standard clinical and hematological data were of no predictive value. Iron kinetic data and CFU/GM colony growth were correlated to the scintigraphic results. Taken together, these three kinetic parameters have a good sensitivity and specificity for the prognosis of preleukemic states.

ISSN:0361-8609    

DOI:10.1002/ajh.2830160305

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

年代:1984

数据来源: WILEY

摘要:

AbstractFunctional and enzyme activity of neutrophils in patients with agnogenic osteomyelofibrosis (AOM) is still a subject of controversy, probably owing to the heterogeneity of the abnormalities observed from patient to patient, and the absence of longitudinal studies performed during the course of the disease. For a better definition of these abnormalities, 40 patients with untreated AOM were studied from the time of diagnosis, and in 12 cases, neutrophil function and enzyme activity were reassessed at one‐year intervals for 3–5 years. The results showed that all abnormalities were serum‐independent. Mean ingestion rate in neutrophils from patients were similar to that of the controls but individual values were more varied, with some very high and some low ingestion rates. Histochemical latex ingestion and nitroblue tetrazolium reduction were lower in patients than in controls, as were cyanide‐insensitive O2consumption and superoxide anion and H2O2production. Mean iodination was also decreased and, in most patients, was related to a decrease in H2O2production. However, in eight patients H2O2production was normal, although an iodination deficiency was found. Myeloperoxidase activity was low in five of these cases. In the other three patients this discrepancy was not explained. The longitudinal study of 12 patients showed that defects observed in the early stages of the disease remained or were aggravated, and that new abnormalities appeared during the course of the

ISSN:0361-8609    

DOI:10.1002/ajh.2830160306

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

年代:1984

数据来源: WILEY

摘要:

AbstractFifty‐two consecutive and previously untreated adults with nonlymphoblastic leukaemia underwent remission induction chemotherapy with a combination of the epipodophyllotoxin VP16‐213, cytosine arabinoside, and doxorubicin (Adriamycin). Complete remission was achieved in 23 of the 52 patients (44%) by means of a single course of therapy in 20 individuals, two courses in two, and three courses in one; median duration of complete remission was 48 weeks. Failure to achieve remission status was due to primary drug resistance in 13 patients (25%), and adequate trial of therapy was not possible in 16 patients (31%) owing to their late referral and accounts for the low remission rate. Individuals achieving complete remission were randomly assigned to receive either 6 (n = 8) or 15 months (n = 13) of maintenance therapy; respective median survival was 95 and 78 weeks (P>0.10). These data confirm previously reported results for complete remission induction with this three‐drug combination and fail to show any difference between short (6 months) and long (15 months) maintenance chemoth

ISSN:0361-8609    

DOI:10.1002/ajh.2830160307

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

年代:1984

数据来源: WILEY

摘要:

AbstractLymphocyte subsets were enumerated in a group of 31 patients with aplastic anemia. Abnormal numbers of immunoregulatory T‐cells were found in some patients: 26% of them showed a reversed helper/suppressor ratio. Seven of 18 patients showed significantly decreased proliferation in response to PWM; this hyporesponsiveness was present in 75% of patients with a reversed helper/suppressor ratio and in 10% of those with a normal helper/suppressor ratio (R = 0.66, P = 0.008). Eight of 18 patients showed suppressor activity over PWM‐induced allogeneic cell proliferation. This suppressive activity did not correlate with T‐cell phenotype. Of the patients with a low number of T‐cells, 73% had responded to treatment, whereas of those patients with a normal number of T‐cells, 26% had responded (P = 0.016). The results are consistent with abnormal immune response in selected patients with aplastic anemia, and suggest a possible influence of T‐cells on dise

ISSN:0361-8609    

DOI:10.1002/ajh.2830160308

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

年代:1984

数据来源: WILEY

摘要:

AbstractThe total number of nucleated cells in the long bone of a mouse can be determined with some accuracy. Thus, in this species, values for marrow cells can be expressed as a total cell count per bone, a more meaningful number than values expressed as concentration as is done in most studies of other species. If the percent of total marrow in the skeleton that is contained in that bone is known, values can be expressed as “per mouse” (total marrow mass)–‐a still more meaningful value than values per bone. The total marrow mass of mice has been calculated previously on the basis of nucleated cells per humerus or per femur and the percent of the total marrow contained in that bone. However, that percent was based on rather tedious dissection of the entire skeleton and determining the amount of59Fe that had been taken up by each bone. In the present study, mice were injected with59Fe, skinned, and eviscerated. The carcass was then either cooked and all bones dissected out or simply cut into pieces containing various bones or bone groups. The percent of59Fe taken up by various bones or bone groups as measured by the two techniques was virtually identical. The percent distribution between various bones was found to be fairly constant between 4 and 18 h after59Fe injection and the same in mice aged 3 or 12 months. This simplified technique makes the measurement of total marrow mass a practical addition in studies of murine hemato

ISSN:0361-8609    

DOI:10.1002/ajh.2830160309

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

年代:1984

数据来源: WILEY

摘要:

AbstractA 21‐year‐old primigravida in her 20th week of pregnancy developed TTP. She was managed with weekly or semiweekly plasma infusions and delivered a healthy 1.6 kg baby 2 weeks prior to the expected date of delivery. After delivery she continued to have active TTP with thrombocytopenia which responded repeatedly to plasma infusion though their frequency gradually decreased to about one unit every 3–4 weeks. Three years after the birth of the first child she conceived again and was easily managed with repeated plasma infusions although the frequency and amount of plasma required to prevent thrombocytopenia were increased. She delivered a normal 3.4 kg term baby after which she again had a decreased plasma requirement.This is the first report of a woman with 5 years of active TTP managed with plasma alone. She experienced two pregnancies in which both mother and infant survived. We believe that the use of plasma in the management of TTP during pregnancy will improve the survival rate of both mother and infant.At the time of second birth, the platelet count was low in the mother but normal in the baby. This suggests that the platelet depressing factor of this patient does not cross the placental ba

ISSN:0361-8609    

DOI:10.1002/ajh.2830160310

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

年代:1984

数据来源: WILEY

摘要:

AbstractPeripheral blood cells from a female infant with Down syndrome and over 60% circulating myeloblasts were cultured in soft agar. Growth was virtually restricted to cluster formation, and cluster‐forming cells resided almost exclusively in the very light density fraction (SG<1.062). Morphological assessment of clusters revealed no evidence of cellular differentiation beyond the blast cell stage. Despite receiving no specific chemotherapy, the peripheral blood normalized within 2 months, and there was no evidence of leukemia when the patient died aged 1 year from cardiac pathology. The findings indicate that caution should be exercised when assessing prognosis on the basis of in vitro growth characteristics in such patient

ISSN:0361-8609    

DOI:10.1002/ajh.2830160311

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

年代:1984

数据来源: WILEY