摘要:

AbstractThe levels of adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), lactic dehydrogenase (LDH), and LDH isoenzyme patterns (LD1 to LD5) have been measured in lymphocyte extract from 28 patients with B‐chronic lymphocytic leukemia (B‐CLL). The activities of ADA, PNP, and LDH have been correlated with two morphological groups of B‐CLL classified according to the percentage of large, nongranular, atypical lymphocytes (AL) in peripheral blood: “typical” B‐CLL (less than 10% of AL, 21 cases) and “atypical” B‐CLL (10–25% of AL, seven cases). Patients with atypical B‐CLL had significantly (P<0.001) higher activities of ADA (0.46 ± 0.17 U/109cells), PNP (1.74 ± 1.0 U/109cells), and LDH (48.3 ± 9.7 U/109cells) than patients with typical B‐CLL (ADA, 0.29 ± 0.1 U/109cells; PNP, 0.58 ± 0.23 U/109cells; and LDH, 29 ± 10 U/109cells). In addition, the “treatment‐free period” was also signficantly (P<0.025) shorter in the group of atypical B‐CLL compared with the typical B‐CLL group. No clear‐cut statistical differences in lymphocyte surface markers or in several other prognostic factors between the two subgroups of B‐CLL were found. The present study supports the idea that in B‐CLL the simultaneous determination of ADA, PNP, and LDH might be helpful in better understanding the pathophysiology,

ISSN:0361-8609    

DOI:10.1002/ajh.2830270302

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

年代:1988

数据来源: WILEY

摘要:

AbstractA retrospective multivariate analysis of 37 clinical, biochemical, and hematological data was performed in 107 cases of primary myelodisplastic syndromes (MDS) in order to recognize their prognostic significance. The most important individual variables, isolated in a previous univariate analysis, were placed in a multiple regression modeling procedure to identify major significant prognostic factors. Multivariate analysis tends to identify prognostic variables containing significant independent predictive information. Characteristics were examined on both continuous and binary bases. The FAB classification was the first parameter entered in regression equations on both models, followed by platelet count, hemoglobin level, and circulating erythroblasts in the binary model, and by hemoglobin level, systemic symptoms, platelet count, age, and dyserythropoiesis in the continuous model. Our analysis confirms FAB classification as the main prognostic factor in MDS, supports the previously noted predictive value of platelet count, hemoglobin level, and age, and recognises the importance of circulating erythroblasts, systemic symptoms, and dyserythropoiesis as prognostic characteristics in MDS.

ISSN:0361-8609    

DOI:10.1002/ajh.2830270303

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

年代:1988

数据来源: WILEY

摘要:

AbstractIn order to assess the functional property of neonatal polymorphonuclear leukocytes (PMNs), we studied their chemoattractant‐stimulated superoxide anion (O2−) production using a soluble chemotactic peptide N‐formyl‐methionyl‐leucyl‐phenylalanine (FMLP) as a PMN stimulus. O2−production by neonatal PMNs was increased: when stimulated with 10−7M FMLP, O2−values (nanomoles; mean ± SE) produced by 106PMNs were 16.5 ± 1.3 for neonatal PMNs and 12.6 ± 0.6 for adult PMNs (p<.02). When studied under various concentrations (10−9to 10−6M) of FMLP, neonatal PMNs produced more O2−than adult cells under the stimulation of a lower concentration (10−8M) as well as a higher concentration (10−7M) of FMLP. The increased O2−production by neonatal PMNs was also observed under their stimulation by phorbol myristate acetate. Neonatal and adult PMNs produced equal amounts of O2−when stimulated with a particulate stimulus of opsonized zymosan. To analyze further the mechanism for the increase in FMLP‐induced O2−production by neonatal PMNs, we next studied whether there was some abnormality in their FMLP receptors. Neonatal PMNs had normal numbers of FMLP receptors; the receptor numbers (mean) per cell were 54,000 in neonatal PMNs and 56,000 in adult cells. Additionally, the FMLP receptors had normal affinity for the peptide. These results demonstrate that the increased production of O2−by FMLP‐stimulated neonatal PMNs is not due to the abnormality of its binding to the receptors b

ISSN:0361-8609    

DOI:10.1002/ajh.2830270304

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

年代:1988

数据来源: WILEY

摘要:

AbstractWe have identified an unusual pediatric patient among twelve patients with myelodysplasia and an unbalanced translocation involving chromosomes 1 and 7: −7, + der(1)t(1;7)(p11; p11). This 16‐year‐old male patient developed myelodysplasia and evolving acute leukemia, which were preceded by a 7‐year history of marrow hypoplasia. The remaining patients were adults with clinical and hematologic findings similar to other reported cases with this chromosomal abnormality. The late appearance of this unbalanced clonal abnormality in this patient with marrow hypoplasia documents the importance of close cytogenetic follow‐up of all patients with suspected bone marr

ISSN:0361-8609    

DOI:10.1002/ajh.2830270305

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

年代:1988

数据来源: WILEY

摘要:

AbstractThrombotic thrombocytopenic purpura (TTP) is associated with abnormal platelet function and disturbances in coagulation; however, a specific causative factor is not defined. Plasma infusion or plasma exchange (PE) are thought to be of benefit in replacing a deficient plasma component or removing some toxic compound. In three patients with TTP, samples taken prior to initiation of PE showed high levels of vWF:Ag in the plasma (208, 264, and 321 U/dl), whereas the VIII:C levels were normal. The vWF:Ag multimer patterns of the plasma demonstrated a decrease in the amount of high molecular weight (HMW) forms. Analysis of the platelets from one patient also showed an increase in the HMW multimers. Platelets from all three patients showed a decreased ability to absorb vWF:Ag, with little or no absorption of the HMW forms. Following extensive PE and resolution of disease, the platelets regained their ability to absorb vWF:Ag in the one patient examined.

ISSN:0361-8609    

DOI:10.1002/ajh.2830270306

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

年代:1988

数据来源: WILEY

摘要:

AbstractOur studies of an acute leukemia with a clonal t(1;11) marker have demonstrated a conversion from pre‐B cell to myelomonocytic phenotype associated with the acquisition of trisomy 8. This finding suggests that trisomy 8, a frequent clonal abnormality in acute myeloid leukemias, may be associated with the myeloid phenotype rather than the neoplastic transformation. A permanent myelomonocytic cell line, designated UF‐SK1, has been established from leukemic cells with a 47,XX, + 8,t(1;11) (p31;q25) karyotype and shown to have myelomonocytic characteristics, including phorbol ester‐induced differentiation to macrophages. This new cell line will be a valuable tool in the study of leukemogenesis and lineage commi

ISSN:0361-8609    

DOI:10.1002/ajh.2830270307

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

年代:1988

数据来源: WILEY

摘要:

AbstractIn 28 unselected patients with hyperthyroidlsm, platelet count, platelet hematocrft, mean platelet volume, and platelet distribution width were measured at the time of presentation and again when the patients were euthyroid. On return to the euthyroid state, there were highly significant falls in the mean values of the mean platelet volume (16% decline,P<0.001) and the platelet hematocrit (16% decline,P<0.001) and a slight but highly significant increase in the mean value of the platelet distribution width (2% increase,P<0.01). A decline in mean platelet volume was observed in 24 of 28 patients (86%); three patients showed no change. There was no significant change in the mean value of the platelet count. The observed effects of thyrotoxicosis on platets appeared to be largely independent of the well‐known effects on erythrocytes and white blood cells. An increase in mean platelet volume is a regular feature of hyperthyroidism that has not previously been describe

ISSN:0361-8609    

DOI:10.1002/ajh.2830270308

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

年代:1988

数据来源: WILEY

摘要:

AbstractThis study consists of 25 patients with chronic granulocytic leukemia in blast crisis (BC) or with acute leukemia who had a Ph1chromosome and one or more other chromosome abnormalities and who were investigated by cytochemistry and immunocytochemistry techniques to determine whether the predominant blasts were myeloid or lymphoid. The disorder was myeloid in 15 patients, lymphoid in 8, and mixed in 2. Among the 15 patients with myeloid disorders, 13 (86.6%) had an additional Ph1chromosome, i(17q), +8 +19, or some combination of these abnormalities. None of the eight patients with a lymphoid disorder had +8, +19, or i(17q), but one had an additional Ph1chromosome. Among the eight patients with lymphoid disorders, two had structural abnormalities of chromosome 7 and two were monosomy 7. None of the patients with myeloid disease had a structurally abnormal chromosome 7, but one was monosomy 7. Our findings suggest that the number of chromosomes in an abnormal clone may be unreliable for distinguishing between lymphoid and myeloid BC. Most patients with myeloid disease had only abnormal metaphases, whereas many patients with lymphoid disorders had both normal and abnormal metaphases. This finding may partially explain why many patients with lymphoid BC respond better to treatment than do those with myeloid BC.

ISSN:0361-8609    

DOI:10.1002/ajh.2830270309

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

年代:1988

数据来源: WILEY

摘要:

AbstractHemoglobin Pasadena [β75(E19)Leu→Arg] was found in a boy who had an acute episode of anemia and rapid splenic enlargement. His father was the only other member of a large family with this hemoglobinopathy. We have used gene mapping techniques for direct identification of the β‐globin gene mutation. To correlate the DNA findings with the structural identification of this variant, we have also performed globin chain separation and analysis of the tryptic peptides using high performance liquid chromatography and secondary ion mass spectral ana

ISSN:0361-8609    

DOI:10.1002/ajh.2830270310

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

年代:1988

数据来源: WILEY

摘要:

AbstractAn Israeli Arab family with type I antithrombin III (AT‐III) deficiency with several affected symptomatic members in three generations is reported. The propositus presented with deep vein thrombosis and pulmonary emboli associated with gestation.The propositus infant presented at the age of 2 weeks with superior sagittal and rectus sinus thrombosis.Hereditary AT‐III deficiency should be considered in infants with cerebral thrombosis, especially if they have a family history of thromboembolism. The role of prophylactic therapy by AT‐III concentrates in these infants should be further ass

ISSN:0361-8609    

DOI:10.1002/ajh.2830270311

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

年代:1988

数据来源: WILEY