摘要:

AbstractTo evaluate the possible role of inadequate food intake in the pathogenesis of the growth retardation of patients with sickle cell anemia, we determined the daily intake of calories and macronutrients and measured several anthropometric indices in 20 patients with sickle cell anemia aged 17–35 years and in 15 of their normal siblings of similar age. Compared to the control groups, the male patients, but not the females, had a significantly lower mean weight, body mass index, midarm circumference, and triceps and subscapular skinfold thicknesses. Also, while the male patients consumed significantly less total calories, proteins, carbohydrates, and fats per day than their control group, no difference was noted between the daily intake of calories or macronutrients in the female patients and their control group. However, when the intake of calories and macronutrients was corrected for body weight, there was no statistically significant difference between the intake of nutrients in the male patients and their control subjects or between the female patients and their control group. These results suggest a sex‐related difference in the somatic growth of adolescent and adult patients with sickle cell anemia and also suggest that, although an inadequate food intake may be partly responsible for the impaired somatic growth in sickle cell anemia, other factors are also probably important. © 1993 Wiley‐Lis

ISSN:0361-8609    

DOI:10.1002/ajh.2830440302

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

年代:1993

数据来源: WILEY

摘要:

AbstractWe studied the effects of recombinant α2‐b interferon (α2‐b IFN) in a dose of 3 × 106U intramuscularly three times a week for 1 year in 13 patients affected by polycythemia vera (PV) previously treated with phlebotomy only. Response to treatment was evaluated by reduction of the number of phlebotomies required to retain normal hematocrit value. Ten out of 13 patients (77%) responded to treatment; in 4 of them the exigency of phlebotomy was completely eliminated. In all responders a concomitant decrease of platelet count and splenomegaly was obtained. Treatment was well tolerated and side effects were easily controlled. We conclude that α‐IFN may represent an attractive therapeutic option in the management of the proliferative stages of PV. © 1993 Wiley

ISSN:0361-8609    

DOI:10.1002/ajh.2830440303

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

年代:1993

数据来源: WILEY

摘要:

AbstractWe describe an infant with hemoglobin D Ibadan‐β° thalassemia whose hemoglobinopathy was initially detected by neonatal screening. This previously undescribed condition was confirmed by family studies and by globin chain analysis by mass spectrometric techniques. The case illustrates the importance to neonatal screening programs of confirmatory testing and of linkage with reference laboratories capable of globin chain analysis. Hematologic studies at 36 months of age suggested that the presence of hemoglobin D Ibadan had no deleterious effect on this child with heterozygous β° thalassemia. © 1993 Wiley‐L

ISSN:0361-8609    

DOI:10.1002/ajh.2830440304

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

年代:1993

数据来源: WILEY

摘要:

Abstractα‐Thalassemia is usually due to deletions within the α‐globin gene cluster, leading to loss of function of one (‐α) or both [‐(α) or –] α‐globin genes. Nondeletion mutations (denoted ααTor αTα) are less frequent and in Greece are not well defined. We report the analysis of 16 nondeletion α‐thalassemia chromosomes using a polymerase chain reaction method to amplify specifically the α2‐globin gene, which was subsequently screened using ASO hybridization or restriction enzyme analysis for four mutations already characterized in other Mediterranean and Middle Eastern populations. Of the 16 nondeletion chromosomes, nine had the polyadenylation signal mutation (αPolyAα), two the IVSI 5′ pentanucleotide deletion (αHphα), two the Hb Icaria mutation (αicα), and one the initiation codon mutation (αNcoα). In two, the defects are still undefined. These findings show that nondeletion α‐thalassemia in Greece is heterogeneous and that the most frequent mutation (accounting for>50%) is the polyadenylation signal mutation, which to date was most commonly found in the Saudi Ara

ISSN:0361-8609    

DOI:10.1002/ajh.2830440305

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

年代:1993

数据来源: WILEY

摘要:

AbstractHemolysis caused by ABO antibodies after ABO‐compatible, nonidentical solid organ transplantation has been previously reported. The passenger B lymphocytes within the donor organ presumably generate an acute, primarily red cell‐directed graft vs. host (GVH) response. Graft survival may also be compromised. GVH Rh antibodies have also been described, primarily in renal transplants. Only three cases, two only in abstract form, have been reported thus far describing GVH Rh antibodies in liver transplant patients, to which we add a fourth. A 62‐year‐old blood group A Rho(D)‐positive woman with cirrhosis underwent orthotopic liver transplantation from a group A Rho(D)‐negative, previously Rho(D)‐sensitized donor and subsequently developed acute, self‐limited hemolysis requiring four units of packed red cells. Anti‐Rho(D) was identified in both serum and red cell eluate. An antibody detection test, identification, and assessment of the antibody reactivity score from the pretransplant donor specimen may identify patients at risk for hemolysis due to GVH Rh antibodies. © 19

ISSN:0361-8609    

DOI:10.1002/ajh.2830440306

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

年代:1993

数据来源: WILEY

摘要:

AbstractResults from this study show that a combination of heme and interleukin‐1 (IL‐1) treatment resulted in the most improved recovery of hematopoietic‐stromal regeneration after sublethal irradiation. Less pronounced effects were obtained when heme or IL‐1 were given singly. Sublethal irradiation of mice produced an initial (as early as day 1) intense depression of the hematopoietic system as evidenced by leukopenia. In vivo treatment of animals with heme in combination with IL‐1, accelerated hematopoietic and stromal regeneration as determined by hematopoietic spleen colony forming unit assay (CFU‐S), erythroid (BFU‐E), myeloid (CFU‐GM) clonal cultures, long‐term bone marrow cultures (LTBMC), and the ability to regenerate hematopoiesis by ectopic (renal) stromal hemopoietic transplantation. Sixteen days after irradiation, leukocyte levels in heme and IL‐1 treatment groups were higher than non‐treated animals and were near normal values by 27 days. One day after irradiation, the capacity of stromal progenitors to form new bone and hematopoietic cells (ectopic foci) was severaly impaired, but recovered after 2–4 weeks. This recovery process was accelerated in heme and IL‐1‐treated animals. BFU‐E, CFU‐GM, and CFU‐S capacity was also severely impaired in all animals 1–27 days after irradiation. CFU‐S was only 0.15% of control by day 1 and 5% of control by day 16. Treatment with heme or IL‐1 improved recovery by as much as 70% after 27 days of irradiation. A similar but enhanced recovery was seen for BFU‐E and CFU‐GM, with erythroid recovery the best. Total cellularity, adherent cell layer (ACL) formation, and donogenic capacity by LTBMCs (10 weeks) derived from irradiated animals was severely reduced, whereas the hematopoietic capacity by LTBMCs derived from heme‐ and IL‐1‐treated animals had recovery values similar to non‐irradiated controls. These results suggest therapeutic use of heme and IL‐1 after chemotherapy or bone marrow

ISSN:0361-8609    

DOI:10.1002/ajh.2830440307

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

年代:1993

数据来源: WILEY

摘要:

AbstractThe purpose of the study was to investigate the movement of iron and transferrin in the macrophage using light and electron microscopy. First, depicted here are the phagocytosis of antibody sensitized murine red cells by the murine bone marrow derived macrophage and the formation of red cell phagosomes. Second, we show the fusion of the lysosomes with the red cell phagosome to form a lysophagosome and the lysis of the red cell using acid phosphatase as a lysosome marker. Third, by autoradiography, the presence of55Fe silver grains in the phagocytosed red cells and its delivery to the organelles of the macrophage are demonstrated. Fourth a transferrin species is shown in red cells of all ages, in the phagocytosed as well as the non‐phagocytosed, and in the phagocytosed as well as the non‐phagocytosed, and in the macrophage itself. Transferrin was detected using immunogold and fluorescence labelling. These studies suggest that iron, using vesicles as means of transport, moves from the effete red cells inside the macrophage to the outside possibly bound to transferrin. © 1993 Wiley‐Lis

ISSN:0361-8609    

DOI:10.1002/ajh.2830440308

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

年代:1993

数据来源: WILEY

摘要:

AbstractWe studied whether or not an anti‐β2‐glycoprotein I antibody (aGPI) had lupus anticoagulant‐like activity, employing the diluted Russel viper venom time (dRVVT) and kaolin clotting time (KCT) as indices. aGPI prolonged the dRVVT and KCT of β2‐glycoprotein I (GPI)‐depleted normal plasma in the presence of extrinsic GPI. This prolongation of the dRVVT and KCT occurred immediately after the addition of aGPI and GPI, and was subsequently enhanced further in a time‐dependent manner. The GPI/aGPI combinaion was judged to have lupus anticoagulant‐like activity by the dRVVT‐platelet neutralization test, but this was not confirmed by a lupus anticoagulant‐specific test, i.e., the activated partial prothrombin time (APTT) using hexagonal phospholipid. From these findings, it can be concluded that aGPI has lupus anticoagulant‐like activity in the presence of GPI, but may be a partially or considerably different antiphospholipid antibody from lupus anticoagulant. Further investigations may be needed to clarify this point.

ISSN:0361-8609    

DOI:10.1002/ajh.2830440309

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

年代:1993

数据来源: WILEY

摘要:

AbstractHemoglobin Lepore is composed of two normal α‐globin chains and two δ‐β fusion globin chains that result from nonhomologous crossing over of genetic material during meiosis. The doubly heterozygous condition of sickle hemoglobin with Lepore hemoglobin (Hb S‐Lepore) is rare, having been described previously in only nine patients. We report two siblings with Hb S‐Lepore who have similar hematologic characteristics but a marked difference in clinical severity. © 1993 Wile

ISSN:0361-8609    

DOI:10.1002/ajh.2830440310

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

年代:1993

数据来源: WILEY

摘要:

AbstractPolycythemia vera (PCV) and multiple myeloma are both clonal disorders of hematopoietic stem cells. The simultaneous occurrence of these diseases in an individual patient is rare. A case of synchronous PCV and smoldering myeloma is presented and the literature is reviewed. The issues of clinical importance in this unusual case include the mechanisms of anemia in multiple myeloma, the difficulty in using anemia as a parameter on which to base the initiation of therapy for myeloma, and the risks of treatment‐induced leukemia and myelodysplasia. © 1993 Wiley‐Liss,

ISSN:0361-8609    

DOI:10.1002/ajh.2830440311

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

年代:1993

数据来源: WILEY