摘要:

AbstractIn this study, we analyzed the phenotypic manifestations resulting from the interaction of heterozygous βo‐thalassemia(βo‐39 nonsense mutation) with the functional loss of three α‐globin structural genes in six subjects, of whom four had the [‐αl–] α‐globin genotype and two the [–/αThα]α‐globin genotype. The β‐thalassemia defect was in all cases the nonsense mutation at codon 39. The nondeletion α‐thalassemia αthwas the initiation codon mutation (AUG→GUG) of the α‐2 gene. In all these subjects hypochromia and microcytosis were more marked than in βα ‐thalassemia heterozygotes with a full complement of four α‐globin genes. All but one had moderate anemia. The α:β globin chain synthesis ratios were consistently decreased. No cases had Hb H on electrophoresis. Subjects with [–/αThα] α‐globin genotype had more severe thalassemia‐like m

ISSN:0361-8609    

DOI:10.1002/ajh.2830290202

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

年代:1988

数据来源: WILEY

摘要:

AbstractWe examined the zinc status of 80 children with sickle cell disease (SCD) and 44 disease‐free sibling controls aged 3 to 18 years. For both patients and controls, variations in serum zinc by age, type of hemoglobinopathy, and growth status were measured. The mean serum zinc concentration of patients was significantly lower than for controls (77.8 ± 9.9 vs. 82.2 ± 9.8 μg/dl, mean ± 1SD,P<.05). Serum levels of alkaline phosphatase (AP) and retinol‐binding protein (RBP), two zinc‐dependent proteins, were also lower among patients (AP: 171 ± 66 vs. 243 ± 97 IU/L,P<.001; RBP: 1.92 ± .9 vs. 2.77 ± .9 mg/dl,P<.001). Patients ≥ 12 years of age (n = 34) had significantly lower zinc levels than those<12 years (74.5 ± 8.4 vs. 80.3 ± 10.3 μg/dl,P<.01), and children with homozygous SCD (Hb SS, n = 55) had a more pronounced deficiency than those with a variant hemoglobinopathy (76.3 ± 8.9 vs. 81.5 ± 11.5, μg/dl,P<.05). Patients classified as having “poor” growth (height‐for‐age<5th percentile, n = 24) had a lower serum zinc level than those with “normal” growth (72.8 ± 8.0 vs. 79.8 ± 10.0 μg/dl,P<.01). Dietary intake data, body mass index, and serum total protein and albumin levels were similar for patients and controls, suggesting that zinc deficiency in SCD does not relate to inadequate dietary intake. The origin of low serum zinc levels in children with SCD is more likely to relate to factors such as increased urinary zinc excretion, chronic intravascular

ISSN:0361-8609    

DOI:10.1002/ajh.2830290203

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

年代:1988

数据来源: WILEY

摘要:

AbstractAccording to previous clinical studies, the neurological manifestations observed in patients with thrombotic thrombocytopenic purpura (TTP) are considered to be transient. The introduction of plasma therapy in 1977 as major treatment modality for TTP has changed the prognosis of the disease. In a clinical survey of 38 patients with TTP who received plasma therapy, we have observed five patients who developed permanent neurological deficits despite their prompt recovery from TTP. In this study, we describe the new complication of TTP and summarize the neurological manifestations observed in these patients during their first episode of TTP and during the relapses, which occurred in 12 of them.

ISSN:0361-8609    

DOI:10.1002/ajh.2830290204

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

年代:1988

数据来源: WILEY

摘要:

AbstractThere is currently little experience using a continuous intravenous infusion of low‐dose cytosine arabinoside (LDARA‐C) in the treatment of acute nonlymphocytic leukemia (ANLL). We report the results in 12 patients with ANLL described as either relapsed ANLL, ANLL with a preceding myelodysplastic phase, or ANLL in the elderly treated with 14 days of continuous intravenous LDARA‐C (20 mg/m2/day). Complete responses (CR) were seen in five patients (42%) and partial responses (PR) in three patients (25%). Treatment resulted in overall and clonal cytoreduction, which was evident by serial bone marrow exams and bone marrow cytogenetic analysis. The ability to obtain a CR correlated with the finding of a low initial marrow cellularity (P<.05). This study finds that continuous intravenous infusion of LDARA‐C for ANLL can achieve response rates comparable to standard induction programs in a subset of patients traditionally defined as having a poor pr

ISSN:0361-8609    

DOI:10.1002/ajh.2830290205

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

年代:1988

数据来源: WILEY

摘要:

AbstractWe describe the first three patients with a large granular lymphocytosis/lymphocytic leukemia and another blood malignancy. In two, a myeloproliferative disorder developed soon after the diagnosis of abnormal proliferation of large granular lymphocytes‐natural killer (LGL‐NK) cells, a myelodysplastic syndrome evolving to acute leukemia and a Philadelphia‐positive chronic myelogenous leukemia. In these cases, LGLs expressed the phenotype of CD2 + NK and CD3 ‐ NK cells, respectively, and were clonal in the first patient as demonstrated by T‐cell receptor gene rearrangement study. In the third case, a similarly clonal excess of LGLs, phenotypically CD3 + NK cells, was detected following a diagnosis of B‐cell hairy‐cell leukemia. Clinically, the concurrence of LGL proliferation and other leukemia did not seem to confer a worse prognosis on the patients. Although an association by chance remains a possible explanation, a common origin from an altered precursor cell for both myeloid and LGL proliferations in the first two cases is discussed, whereas in the third it might be related to the severe immune derangement frequently observed in hairy

ISSN:0361-8609    

DOI:10.1002/ajh.2830290206

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

年代:1988

数据来源: WILEY

摘要:

AbstractHematologic abnormalities are common in patients with HIV infection. This review will focus on HIV‐associated cytopenias and coagulation abnormalities. Their occurrence, laboratory evaluation, and clinical significance and the mechanisms underlying their development are discussed. Therapeutic modalities are presented, with an emphasis on treatment strategies for HIV‐associated thrombocytope

ISSN:0361-8609    

DOI:10.1002/ajh.2830290207

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

年代:1988

数据来源: WILEY

摘要:

AbstractThe driving force behind development of in vitro methods for platelet antibodies is identification of plasma factors causing platelet destruction. Early methods relied on measurement of platelet activation. Current methods are more specific and use a purified antibody against immunoglobulin or complement, which is usually labeled with125I or tagged with an enzyme or fluorescein. Comparisons of guantitation of platelet‐associated IgG show wide variability between different methods. The disparate results can be related both to differences in binding of secondary antibodies to immunoglobulin in solution compared to immunoglobulins attached to platelets and to the improper assumption that the binding ratio between the secondary detecting and primary antiplatelet antibody is one. Most assays can 1) identify neonatal isoimmune thrombocytopenia and posttransfusion purpura, 2) help to differentiate between immune and nonimmune thrombocytopenias, 3) help to sort out the offending drug when drug‐induced thrombocytopenia is suspected, and 4) identify platelet alloantibodies and potential platelet donors via a cross match assay for refractory patients. However, the advantages of quantitative assays over qualitative methods with respect to predictions of patients clinical course and response to different treatments remain to be investiga

ISSN:0361-8609    

DOI:10.1002/ajh.2830290208

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

年代:1988

数据来源: WILEY

摘要:

AbstractAcute tumor lysis syndrome developed in a 30‐year‐old man with non‐Hodgkin lymphoma after dexamethasone administration. To the best of our knowledge this case is the first one reported following single agent corticosteroid trea

ISSN:0361-8609    

DOI:10.1002/ajh.2830290209

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

年代:1988

数据来源: WILEY

摘要:

AbstractA 27‐year‐old man with chronic myelocytic leukaemia sustained two episodes of extramedullary blast transformation. The first episode was a lymphoblastic transformation in his cervical lymph nodes, which was treated and in remission for 15 months when a second blastic transformation occurred in the meninges. All the while, the bone marrow was free from blastic cri

ISSN:0361-8609    

DOI:10.1002/ajh.2830290210

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

年代:1988

数据来源: WILEY

摘要:

AbstractA patient with congenital protein‐C deficiency was treated with stanozolol for 8 weeks to increase circulating levels of protein C. A rise in protein C was achieved, accompanied by an increase in factor II, factor X, antithrombin III, and protein S; but at the 8th week the patient suffered a transient ischemia attac

ISSN:0361-8609    

DOI:10.1002/ajh.2830290211

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

年代:1988

数据来源: WILEY