摘要:

AbstractThe standard lymphocytotoxicity assay (LCT), a biotin‐avidin enzyme immunoassay (ELISA), platelet suspension immunofluorescence test (PSIFT), and platelet radioactive antiglobulin test (PRAT) were examined in prospective crossmatching for selection of compatible random donor platelets for refractory patients. One hundred seven episodes of pooled random donor platelet transfusions were evaluated in 26 patients. There was good reproducibility of results by individual techniques. Concordance of results by the different methods was 40–60%. One‐hour and 24 hr posttransfusion corrected count increments (CCI) were compared as parameters for assessing success or failure of the transfusion. Using a rank scoring system, the relative efficiency of predictiveness for all transfusions was PRAT>LCT>PSIFT>ELISA. Combination of PRAT and LCT afforded the best predictability and sensitivity was higher than for either PRAT or LCT alone (93 vs. 79 and 62%, respectively). Mean posttransfusion CCI (x 108/L) following PRAT‐compatible platelets was 13.9 ± 12.7 at 1 hr and 7.3 ± 6.9 at 24 hr; following PRAT‐incompatible 5.7 ± 7.8 (1 hr) and 2.1 ± 4.1 (24 hr). Results were similar for LCT‐tested platelets. A radioimmunofiltration modification of the PRAT developed and used in selected cases was simple, fast, efficient, and inexpensive. The study indicated that the techniques evaluated are practical and feasible for routine use in the provision of compatible random donor platelets to the refractory patient who has no other cause for increased plate

ISSN:0361-8609    

DOI:10.1002/ajh.2830280102

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

年代:1988

数据来源: WILEY

摘要:

AbstractA four‐generation 25‐member kindred with Factor XI:C deficiency is reported. Factor XI:C levels in heterorygotes varied from 15 to 58%, suggesting that Factor XI:C values for homozygote determination should be less than 15%. The frequency of bleeding was not correlated with Factor XI:C levels in this range. Individuals with joint pain had significantly lower Factor XI:C levels than members without joint pain and pain occurred more frequently in frequent bleeders. Lod scores showed no close genetic linkage of Factor XI:C deficiency with blood group MNSs (chromosome 4), complement components Bf and C4B (chromosome 6), or blood grou

ISSN:0361-8609    

DOI:10.1002/ajh.2830280103

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

年代:1988

数据来源: WILEY

摘要:

AbstractChromosome analyses were carried out on bone marrow cells from 43 consecutive patients with primary myelodysplastic syndromes (MDS), classified according to the French‐American‐British (FAB) cooperative group criteria. The objective was to evaluate the prognostic value of clonal chromosomal abnormalities and of an excess of blasts for early death from acute nonlymphocytic leukemia (ANLL) and/or bone marrow failure (BMF). Patients were subdivided into two main groups: (1) refractory anemia without an excess of blasts (RAWEB), grouping patients with refractory anemia (RA) and refractory anemia with ringed sideroblasts (RARS), and (2) refractory anemia with an excess of blasts (RAEB), grouping patients with refractory anemia with an excess of blasts (RAEB) and refractory anemia with an excess of blasts in transformation (RAEBt). There were 29 patients with RAWEB and 14 with RAEB. The median time of observation was 26 months for RAWEB and 12 months for RAEB. Ten RAWEB patients (34%) and 11 RAEB patients (78%) had clonai chromosomal abnormalities. Among the ten RAWEB patients with clonal abnormalities, one (10%) died from ANLL, while of 19 RAWEB patients with a normal karyotype, two (10%) died from ANLL or BMF. The median survival for patients with RAWEB and an abnormal karyotype was not reached. In contrast, eight of the 11 RAEB patients with clonal chromosomal abnormalities (74%) died from ANLL or BMF. The median survival in this sub‐group was 7 months. By using a Cox proportional hazard regression analysis, it was determined that a karyotype abnormality was not a significant predictory of survival once the contribution of the RAWEB/RAEB variable was taken into account. Being in the RAEB group was associated with a relative risk of 10.6 of dying from ANLL or BMF (β = 2.36, standard error (SE) = 0.68,P= .0001). We conclude that classifying patients according to an excess of blasts will lead to a better prediction of survival than determining karyotype abnor

ISSN:0361-8609    

DOI:10.1002/ajh.2830280104

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

年代:1988

数据来源: WILEY

摘要:

AbstractA patient with Philadelphia chromosome (Ph) chronic myelogenous leukemia (CML), in chronic phase, was treated with recombinant γ‐interferon (rγ‐IFN) in a phase I clinical trial. Prior to treatment, analysis of in vitro agar culture parameters indicated hyporesponsiveness of granulocyte‐macrophage colony‐forming cells (CFU‐GM) to inhibition by prostaglandin E and acidic isoferritins and diminished expression of class II major histocompatibility complex (MHC) antigens (HLA‐DR). Treatment was associated with no change in bone marrow cellularity or in the percentage of Ph cells. However, in vitro cultures of bone marrow cells showed a return to normal levels of both expression of CFU‐GM class II antigen and of sensitivity to inhibition by prostaglandin E and acidic isoferritins which predicted and/or confirmed clinical response. Throughout the course of interferon therapy, white blood cell counts (WBC) and the percentage of bone marrow blast cells were maintained at normal levels. Onset of aggressive‐phase disease was associated with increased WBC, an increase in bone marrow blast cells, a secondary chromosomal abnormality, loss of CFU‐GM sensitivity to inhibition by putative negative growth regulators, and markedly diminished MHC class II antigen expression. Following a bone marrow transplant from a matched sibling, all hematologic parameters studied were found to be normal. These findings indicate that treatment with rγ‐IFN can modulate some of the abnormal growth characteristics of

ISSN:0361-8609    

DOI:10.1002/ajh.2830280105

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

年代:1988

数据来源: WILEY

摘要:

AbstractTo examine the variables associated with heterosexual transmission of human immuno‐deficiency virus (HIV), we studied 32 couples in our hemophilia center who had steady sexual relationships for periods more than 1 year. Of the 32 sexual partners of the hemophiliacs, five (15.6%) were HIV seropositive. All five hemophiliacs with HIV transmission to their sexual partners had measurable immunologic deficiencies, as shown by their lower median T‐helper (CD‐4 +) lymphocyte count of 172 cells/mm3. The hemophiliacs without transmission had a slightly higher median CD‐4 + count of 297 cells/mm3(P= 26). To determine if factors other than the degree of immunologic deficiency in the hemophiliac might contribute to HIV transmission, 18 of the 32 couples were studied more intensively by confidential, coded questionnaires. Regular condom use was reported by nine couples (50%). Two of nine women (22%) without condom usage acquired HIV. One of nine women (11%) using condoms was seropositive; she also reported eight needlestick injuries while assisting her spouse with clotting factor treatments. Intravenous drug abuse was reported in two of the five couples with HIV transmission. Thus, hemophiliacs are at risk for transmitting HIV parenterally as well as venereally. Despite various risk behaviors associated with HIV transmission, the prevalence of infection in our cohort of hemophiliacs' sexual partners is low and within the range (6.8–22%) reported by others. This study underscores the need for comprehensive education and counseling in what previously appeared to be a homogeneous clinic population at risk for transmitting HIV

ISSN:0361-8609    

DOI:10.1002/ajh.2830280106

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

年代:1988

数据来源: WILEY

摘要:

AbstractMost patients with chronic myelogenous leukemia (CML) have Philadelphia (Ph) chromosome. Breakpoints on chromosome 22 in CML occur in a small region designated as the breakpoint cluster region (bcr). More than 90 percent of CML patients have breakpoints in thebcr; the remaining patients had no detectable rearrangement. In our study, a commercially available 1.2 kbHindlll‐Bglll(1.2 HBg)bcrprobe was used to locate breakpoints in thebcr, which were found in 22 of 24 patients. Furthermore, using a probe upstream from the 1.2 HBg probe, rearranged bands were clearly detected in the two patients in whom no extra bands had been found with the 1.2 HBg probe. These results strongly susggest that these two patients carry a deletion at theacr‐ablrecombination point encompassing the area of the 1.2 HBg probe. Therefore, in our series, all CML patients eventually had breakpoints in thebcr, and the involvement of rearrangement was demonstrated to be highly specific for CML. Our data indicate that hybridization of CML cellular DNA with severalbcrprobes is important in examining accurately the frequency ofbcr‐ablrearrangements in CML, as some cases contain a deletion within the r

ISSN:0361-8609    

DOI:10.1002/ajh.2830280107

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

年代:1988

数据来源: WILEY

摘要:

AbstractWe have used the new Technicon H.1 Hematology Analyzer to determine the indices of erythrocytes obtained from 18 nonthalassemic (αα/αα genotype) adult patients with Hb SC disease. Controls were 14 normal black adults and 29 white adults. The data showed that SC erythrocytes are significantly smaller than normal RBC (P<.001) with a significantly higher MCHC value (P<.001) than controls. These features of SC RBC could not be reproduced by an Ortho ELT‐8 electonic counter. Hb SC erythrocytes have unique indices which are best demonstrated either by semimanual methods or by the H.1 s

ISSN:0361-8609    

DOI:10.1002/ajh.2830280108

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

年代:1988

数据来源: WILEY

摘要:

AbstractFour chloramphenicol (CAP) metabolites known to be produced by intestinal bacteria were examined with respect to their capacity to induce DNA damage in intact cells. The induction of DNA single‐strand breaks in Raji cells, activated human lymphocytes, and human marrow cells was assayed by the alkaline elution technique. One of the four compounds tested, dehydro‐CAP, was capable of inducing DNA single‐strand breaks in all three cell systems at concentrations of 10−4M. This effect is comparable to that observed previously with nitroso‐CAP, the nitroreduction intermediate of CAP. The nitroreduction of dehydro‐CAP by human bone marrow cell homogenate was detected by the production of the corresponding amino derivative amounting to 5.6 × 10−5M from 2 × 10−3M substrate under aerobic conditions. In sharp contrast, nitroreduction of CAP by bone marrow could not be demonstrated. The genotoxicity of dehydro‐CAP, its relative stability compared to the nitroso‐CAP, and its nitroreducibility by bone marrow suggest that this bacterial metabolite of CAP may play a key role as a mediator of aplastic anemia in

ISSN:0361-8609    

DOI:10.1002/ajh.2830280109

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

年代:1988

数据来源: WILEY

摘要:

AbstractTwo related infants with indistinct slow‐moving minor hemoglobin bands were detected by electrophoretic cord blood screening. The variant separated into a major and a minor band on isoelectric focusing (IEF), anion exchange chromatography (AEC), and high‐performance liquid chromatography (HPLC). Analysis of the products of tryptic hydrolysis of the abnormal chain revealed a truncated AγT‐l peptide containing a glu→lys substitution at position 5, identifying the variant as Hb F Texas 1. Microsequencing of the major early peak from AEC confirmed the substitution. However, γ chains from the minor peak resisted Edman degradation and were shown to be acetylated by fast atom bombardment mass spectrometry (FABMS). Acetylation of HbF Texas I was nearly three times that of normal HbF measured in the same hemolysate, and this ratio remained constant for over 1 year in the proband. This represents the first demonstration of increased Nα‐acetylation of a variant feta

ISSN:0361-8609    

DOI:10.1002/ajh.2830280110

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

年代:1988

数据来源: WILEY

摘要:

AbstractInfection with Ehrlichia canis should be suspected in patients with fever, headache, malaise, leukopenia, thrombocytopenia, and a history of recent exposure to ticks. The cytopenia is caused by bone marrow hypoplasia which may be severe. The disease may be confused with spotless Rocky Mountain spotted fever but can be differentiated from this infection serologically with acute and convalescent sera. In humans, recovery has occurred with and without antibiotic therapy. However, prompt antibiotic therapy is advised prior to serologic studies, especially in immunocompromised individuals, splenectomized persons, and patients with AIDS—who may develop a more overwhelming rickettsial infectio

ISSN:0361-8609    

DOI:10.1002/ajh.2830280111

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

年代:1988

数据来源: WILEY