摘要:

AbstractThe objective of this study was to assess the effect of protein A immunoabsorption in terms of response rate and toxicities in patients with classical thrombotic thrombocytopenic purpura (TTP) refractory to therapeutic plasma exchange. The study included nine females and one male with a diagnosis of classical TTP treated at multiple university hospital centers with protein A immunoabsorption (PAI) after having failed plasma exchange. The 10 patients had an age range 17‐62 years. Prior to PAI, the patients had failed to respond to a mean of 15 (range 6‐39) therapeutic plasma exchanges. Three patients had previous episodes of TTP.Evaluation for response to PAI included serial measurements of serum creatinine, lactate dehydrogenase (LDH), hemoglobin, hematocrit, and platelet count before, during, and up to 18 months post‐PAI treatment.Seven of 10 study patients had resolution of their TTP. Six of the patients required six or fewer therapeutic PAIs and one required 12 treatments. All responding patients had evidence of improvement by the third PAI treatment. Three patients demonstrated no response to PAI, with two patients expiring from complications of TTP and one patient demonstrating a complete response to a subsequent therapy. No significant toxicity was noted with the use of PAI in this setting.Protein A immunoabsorption in patients with classical TTP refractory to plasma exchange can produce durable complete remissions and warrants comparative studies. Am. J. Hematol. 55:55‐58, 1997. © 1997 Wiley

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<55::AID-AJH1>3.0.CO;2-1

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

年代:1997

数据来源: WILEY

摘要:

AbstractFour hundred and ninety‐three consecutive patients referred for arterial or venous thrombosis were screened for congenital and acquired abnormalities of blood coagulation predisposing to thrombosis, and were compared to 341 age‐ and sex‐matched controls. The aim of the study was to determine the prevalence and clinical characteristics of resistance to activated protein C (APC), a defect shown to have different prevalences in different ethnic groups and to be associated with an increased risk of thrombosis. Seventy‐three (15%) patients had both APC resistance and the 1691 G to A Factor V gene mutation, compared to 6/341 (2%) controls. Seven patients had antithrombin deficiency (1.4%), 11 had protein C deficiency (2.2%), and 4 had protein S deficiency (0.8%). The relative risk of thrombosis in APC‐resistant patients was 9.4. Resistance to APC was associated mainly with venous thrombosis, the most frequent being deep‐vein thrombosis of the lower limbs. Fifty‐eight percent of APC‐resistant patients had an associated risk factor at the first thrombotic event: pregnancy and oral contraceptive intake were associated with the first thrombotic episode in 35% and 30% of women, respectively. APC resistance is the most frequent defect of blood coagulation in the general population and in the unselected thrombotic population studied by us. Am. J. Hematol. 55:59‐64, 1997. © 19

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<59::AID-AJH2>3.0.CO;2-1

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

年代:1997

数据来源: WILEY

摘要:

AbstractThere is a high prevalence of thalassemia in the Taiwan area. Prenatal diagnosis of severe forms of thalassemia is important for the prevention of this disease. We performed prenatal diagnosis in 167 cases, of which 59 cases were diagnosed by chorionic villi biopsy, 91 cases by amniotic fluid analysis, and 17 cases by cord blood analysis. Hb Bart's hydrops was detected by amplifying the break junction area of the α‐thalassemia‐1 Southeast Asia (SEA)‐type gene, and β‐thalassemia major was detected by using naturally occurring restriction sites and the amplified created restriction sites (ACRS) method. Screening for hemoglobin (Hg) Bart's hydrops revealed 26 cases of Hb Bart's hydrops, 67 cases of α‐thalassemia‐1 (including 6 Hb Bart's hydrops falsely diagnosed as α‐thalassemia‐1 from chorionic villi samples), and 38 normal cases. Screening for β‐thalassemia major revealed 8 cases of β‐thalassemia major, 17 cases of β‐thalassemia minor, and 11 normal cases. In cases of α‐thalassemia, maternal tissue contamination in the chorionic villi samples occurred in the diagnosis of the carrier state and further amniotic fluid analysis will be necessary. There were no any false‐positive or false‐negative results in β‐thalassemia major screening. We conclude that prenatal diagnosis is a reliable and accurate screening method for thalassemia and may be valuable in other areas of high prevalence for thalassemia in Southeast Asia and in Southern China. Am. J. Hemat.

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<65::AID-AJH3>3.0.CO;2-0

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

年代:1997

数据来源: WILEY

摘要:

AbstractIn this study, we have performed fluorescence in situ hybridization (FISH) with chromosome‐specific DNA painting probes 1, 2, 3, 4, 6, 8, and 12 and centromere‐specific DNA probes 7, 10, 12, 17, 18, and X after G‐banding on the same metaphase spreads from four patients with malignant hematological disorders to more precisely interpret their complex karyotypes. The findings demonstrated that the application of combined G‐banding and FISH can more accurately explain complex karyotypes of hematological malignancies. FISH can detect not only the origin of marker chromosomes, but also the complex rearrangements that cannot be identified by routine banding techniques. This approach is very important to complement the cytogenetic analysis of malignant disorders and to evaluate the role of chromosome change in the development, progression, and prognosis of tumors. Am. J. Hematol. 55:69‐76, 1997. © 1997 Wiley

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<69::AID-AJH4>3.0.CO;2-0

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

年代:1997

数据来源: WILEY

摘要:

AbstractStarting from the observation that a number of consecutive patients with non‐Hodgkin's lymphoma (NHL) resulted positive for hepatitis C virus (HCV) antibodies on routine testing, we set up a survey for HCV contact prevalence in all patients with lymphoproliferative disorders (LPD) followed in our institution. We searched for HCV antibodies by a third‐generation ELISA technique, followed by a confirmation test (RIBA III); serum viral RNA and HCV genotype were investigated by a RT‐PCR technique. We screened a total of 315 patients suffering from B‐NHL (91), multiple myeloma (56), MGUS (48), chronic lymphocytic leukemia (57), Waldentröm's macroglobulinemia (13), Hodgkin's disease (HD)(43), and T‐NHL (9). While only 1 of 52 patients with a non‐B‐LPD (HD or T‐NHL) had signs of HCV contact (i.e., 1.9%, which is in the range of the normal population in the South of Italy), 59 of 263 patients with a B‐LPD (22.4%) had HCV antibodies or RNA, or both, with no major differences among the various types of disorders, except for WM, in which the rate was higher (61.5%). The same prevalence was found for patients tested at diagnosis or during the follow‐up, and in transfused or never‐transfused patients. Only a few patients were aware of having a liver disease; one‐half of HCV‐positive patients never had transaminase increase. A review of data from Central and Northern Italy is included, showing similar findings; a report from Japan has confirmed such an association, while limited surveys in England have not revealed any correlation. These findings may have important biological and clinical implications. Am. J. Hematol. 55:77‐82,

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<77::AID-AJH5>3.0.CO;2-#

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

年代:1997

数据来源: WILEY

摘要:

AbstractThe pathophysiology and clinical severity of β‐thalassemia are related to the degree of α/non‐α‐chain imbalance. A triplicated α‐globin gene locus can exacerbate effects of excess α‐chains caused by a defective β‐globin gene, although this is not observed in all cases. Extensive studies on this condition are lacking. We report a group of 17 patients who are heterozygous for both the αααanti‐3.7allele and a mutation in the β‐globin gene cluster. Their clinical phenotypes varied: six had mild anemia with microcytosis and hypochromia, while 11 had more severe anemia with splenomegaly requiring splenectomy (three cases) and blood transfusions (four cases). Different phenotypes were also evident in the presence of the same β‐thalassemia mutation: in one family, two individuals had the same α‐ and β‐globin genotypes but presented with different hematologic phenotypes. In addition, the complex interaction involving a triplicated α‐globin gene, β39‐ and δ+27‐thalassemia mutations is studied in a family with two siblings presenting with hemolytic anemia, normal Hb A2and increased Hb F. Analysis of this series of patients suggests that additional genetic determinants play a role in modulating phenotypic expression in individuals with identical α‐ and β‐globin genotypes. Interaction with a triplicated α‐gene can play a role in the clinical presentation of patients with defective β‐globin gene expression and should be considered in the diagnosis of atypical cases.

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<83::AID-AJH6>3.0.CO;2-Z

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

年代:1997

数据来源: WILEY

摘要:

AbstractWe evaluated the changes in reticulocyte maturity fractions and indices, as measured by flow cytometry, during preoperative treatment with recombinant human erythropoietin (epoetin beta) in cardiac surgery patients. A total of 72 patients was enrolled in this double‐blind, randomized, placebo‐controlled clinical trial and assigned to the two treatment groups (5 × 500 U/kg bodyweight epoetin beta or placebo intravenously over 14 days preoperatively).Therapy with epoetin beta produced continuous increases in hematocrit/hemoglobin, in the most mature fraction of reticulocytes (LR), and in reticulocyte count. In the first treatment week there were parallel increases in the fraction of most immature reticulocytes (HR) and in the reticulocyte mean cell volume. During the second week of treatment the reticulocyte mean cell hemoglobin content (CHr) decreased, but CHr was independent of all iron parameters, affecting neither the reticulocyte fractions nor the hematocrit/hemoglobin increase. The total preoperative rise in hematocrit correlated with the rises in LR fraction (P= 0.0270) and reticulocyte count (P= 0.0486) during the first week of treatment. Whereas in the epoetin beta patients the preoperative change in HR fraction showed negative correlations with transferrin saturation at baseline (P= 0.0058) and with the preoperative change in iron (P= 0.0113), the preoperative change in the LR fraction correlated positively with transferrin at baseline (P= 0.0115). Postoperatively, the reticulocyte parameters revealed that the onset of increased stimulation of erythropoiesis did not occur in the placebo patients until the second postoperative day, whereas erythropoietic activity in the epoetin beta patients was much higher during the postoperative period as well, as a result of the preoperative stimulation of erythropoiesis.The reticulocyte parameters measured by flow cytometry permitted an objective analysis of erythropoietic activity during treatment with epoetin beta and in all patients postoperatively. Further studies in various types of epoetin beta therapy are needed in order to clarify the value of these reticulocyte parameters for identification of iron deficiency and optimization of epoetin beta treatment regimen. Am. J. Hematol. 55:89‐96, 1997. © 1997 Wiley‐

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<89::AID-AJH7>3.0.CO;2-Z

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

年代:1997

数据来源: WILEY

摘要:

AbstractIt has been demonstrated that cysteine modification in irreversibly sickled cell β‐actin slows down the remodeling of membrane skeletons [Shartava et al.: J Cell Biol 128:805‐812, 1995]. This slow remodeling can be due to alterations in spectrin‐actin binding and/or actin‐actin interactions in irreversibly sickled cell (ISC) membrane skeletons. In these studies we demonstrate that ISC actin binds spectrin normally. However, ISC β‐actin polymerizes and depolymerizes more slowly than control β‐actin, and forms unusual aggregates when placed under polymerizing conditions. Electron microscopic analysis of actin polymers indicated that ISC actin generates a large amount of aggregates which we conclude are due to the structural modification caused by the disulfide bridge between cysteine264and cysteine373in β‐actin. Am. J. Hematol. 55:97‐103, 1997. ©

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<97::AID-AJH8>3.0.CO;2-Y

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

年代:1997

数据来源: WILEY

摘要:

AbstractTwo population groups from Western India with a high prevalence of the βsgene, one tribal (Valsad) and the other nontribal (Nagpur), were studied. The βsgene frequency in both populations was similar (0.22 vs. 0.23), but not the clinical expression of sickle‐cell anemia (SS): the sickle homozygotes in the tribal group appeared to have a mild clinical course, whereas the majority in the nontribal group exhibited a more severe clinical phenotype. Both tribal and nontribal SS patients had a similarly high mean hemoglobin (Hb)F expression (18.5% vs. 15.5%) and a high number of F cells (72.3% vs. 66.6%). DNA analysis of the β‐globin gene cluster region revealed that in these two populations, this portion of DNA was identical with and corresponded to the typical Arab‐Indian haplotype. Nevertheless, in heterozygotes, the mean βsexpression was lower (27.9%) in the tribal as compared to the nontribal group (35.5%). The major epistatic factor distinguishing the milder presentation in tribals vs. a more severe manifestation in nontribals was the very high frequency (0.97) of the α‐thalassemia gene in the former as compared to the latter (0.24). We conclude that the phenotypic expression of sickle‐cell anemia, linked to the Arab‐India haplotype and expressing similar levels of HbF and F cells, is not uniformly mild in India and that α‐thalassemia is a powerful and additional epistatic factor in the Indian subcontinent. Am. J. Hematol. 55:104‐109, 1997.

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<104::AID-AJH9>3.0.CO;2-X

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

年代:1997

数据来源: WILEY

摘要:

AbstractA patient with paroxysmal nocturnal hemoglobinuria developed lactic acidosis associated with severe anemia. The lactic acidosis corrected after blood transfusion. In the absence of shock, sepsis, or other identifiable causes of lactic acidosis, the severe anemia (hemoglobin 1.2 g/dl) appeared to be the primary etiologic factor. Am. J. Hematol. 55:110‐111, 1997. © 1997 Wiley‐Liss,

ISSN:0361-8609    

DOI:10.1002/(SICI)1096-8652(199706)55:2<110::AID-AJH10>3.0.CO;2-I

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

年代:1997

数据来源: WILEY