摘要:

AbstractWe investigated the expression of a monoclonal antibody (HML‐1) defined antigen that appears on human intestinal T‐lymphocytes in HTLV‐I‐related disease. We studied 25 ATL, and 24 healthy HTLV‐I carriers. Patients with acute ATL showed a variety of the expression of the HML‐1 antigen (range 0.4–74.8%). HML‐1 expression on mononuclear cells (MNCs) in blood from patients with chronic ATL ranged from 1.7–43.6% (mean 13.5%). This level of expression was less than that of patients with acute ATL, but not significantly. In patients with smoldering ATL, the degree of patients with acute ATL, but not significantly. In patients with smoldering ATL, the degree of expression ranged from 1.6–13.3% (mean 8.0%). In contrast to patients wtih acute ATL, MNCs from patients with acute myelogenous leukemia (AML), acute lymphocytic leukemia (ALL), and B‐cell type chronic lymphocytic leukemia (B‐CLL) did not express the HML‐1 antigen, except for the 2 patients with ALL. Healthy HTLV‐I carriers and healthy controls also were negative for HML‐1 reactivity. In acute ATL, patients with gastrointestinal tract infiltration tended to have high expression of the HML‐1 epitope. After stimulation with phytohemagglutinin (PHA), healthy HTLV‐I carriers showed significantly increased expression of the HML‐1 epitope (P<0.05).Recently, the β7 integrin family has been found to play a specific role in mucosal localization or adhesion, and HML‐1 protein was found to match the deduced β7 N‐terminal sequence. We propose that the cellular gene responsible for HML‐1 epitope expression may, like IL‐2, IL‐2R, etc., be transactivated by infection with HTLV‐I, and that HML‐1 antigen gene expression by HTLV‐I infection may lead to infiltration of ATL cells wi

ISSN:0361-8609    

DOI:10.1002/ajh.2830500102

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

年代:1995

数据来源: WILEY

摘要:

AbstractBone marrow plasma cells and stromal cells in multiple myeloma (MM) have been shown to be capable of releasing cytokines with angiogenic properties. Plasma cells can also express adhesion molecules controlling their adhesive interactions with endothelial cells. In the present study, we have evaluated by immunohistochemistry the extent of angiogenesis in the bone marrow of: a) 51 patients with active and non‐active MM; b) 25 patients with monoclonal gammopathy of undetermined significance (MGUS). Plasma cells were investigated by flow cytometry for the expression of the adhesion molecules LFA‐1, VLA‐4, LAM‐1, and CD44. The results showed that, while angiogenesis was very low or absent in patients with MGUS and non‐active MM, it increased markedly in those with active MM. The highest detectability of plasma cell adhesion molecules, except LAM‐1, was also found in these patients. The functional significance of these findings is unknown. Their consistent occurrence in the bone marrow of active myeloma patients, however, strongly suggests that more frequent adhesive interactions between plasma cells and their microvasculature underlie tumor di

ISSN:0361-8609    

DOI:10.1002/ajh.2830500103

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

年代:1995

数据来源: WILEY

摘要:

AbstractAmong individuals of Mediterranean or Middle Eastern descent, the IVS‐I‐6 (T→C) mutation is one of the most common causes of β‐thalassemia. In this report, we describe the clinical phenotypes of a group of β‐thalassemia patients who are compound heterozygotes for the relatively mild IVS‐I‐6 (T→C) β‐thalassemia mutation and more severe β+‐ or β°‐thalassemia mutations. Although most of these patients are transfusion‐dependent, the requirement for regular transfusions generally occurred late in childhood. A correlation between concomitant α‐thalassemia and a mild transfusion‐independent phenotype is not apparent, indicating the involvement

ISSN:0361-8609    

DOI:10.1002/ajh.2830500104

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

年代:1995

数据来源: WILEY

摘要:

AbstractHeparin‐induced thrombocytopenia is a rare but severe complication of heparin therapy that can result in severe venous or arterial thromboembolic events and whose treatment remains partially unanswered. Recombinant hirudin is potentially effective as an antithrombotic treatment in the management of heparin‐induced thrombocytopenia, given its potent antithrombin effects without known interaction with platelets. We report the results obtained with intravenous recombinant hirudin (HBW 023) administered on a compassionate basis to patients suffering from heparin‐induced thrombocytopenia.Six patients suffering from heparin‐induced thrombocytopenia were submitted to intravenous recombinant hirudin (HBW 023) administered at a dose of 0.05 mg/kg/hr after an initial bolus injection of 0.07 mg/kg in the case of a venous thromboembolic event, and at a dose of 0.15 mg/kg/hr with the same initial bolus injection in the case of an arterial thromboembolic event. Whenever possible, oral anticoagulation with acenocoumarol was introduced at the same time as recombinant hirudin, which was interrupted as soon as the international normalized ratio reached 3. Clinical events, particularly thromboembolism and bleeding, were noted; activated partial thromboplastin time (aPTT), and platelet count were assessed throughout the administration of recombinant hirudin.Heparins responsible for heparin‐induced thrombocytopenia were porcine sodium or calcium heparinate in four cases, nadroparin in one case, and enoxaparin in one case. Thrombocytopenia was discovered on routine systematic platelet count in two patients and after the occurrence of arterial and venous thromboembolism in two patients, respectively. After discontinuation of heparin and the onset of recombinant hirudin, clinical evolution was uneventful in all patients, with no recurrence of thromboembolism, limb amputation, or hemorrhagic complication. The aPTT ratio varied from 1.8 to 3.5 (median 2.4) throughout administration of recombinant hirudin. Platelet count rose from nadir (median value 60 × 10915 to 90) to above 100 × 109/L in every patient within 3–6 days (median 5), after discontinuation of heparin.Intravenous administration of recombinant hirudin ensured safe anticoagulation in patients with heparin‐induced thrombocytopenia and made it possible to wait for oral anticoagulation to become efficient and platelet count to return to normal values without occurrence or recurrence of

ISSN:0361-8609    

DOI:10.1002/ajh.2830500105

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

年代:1995

数据来源: WILEY

摘要:

AbstractHb Lulu Island [β107(G9)Gly → Asp] was discovered in an East Indian female who carried a common β°‐thalassemia allele, i.e., codon 15, TGG → TAG (is a stop codon)in trans. Both abnormalities were detected through sequencing of the amplified β‐globin genes and were confirmed by hybridization with32P‐labeled probes. Hb Lulu Island is mildly unstable with a borderline decrease in oxygen affinity; its instability is less severe than that of Hb Burke or β107(G9)Gly → Arg. The compound heterozygosity expresses as a thalassemia intermedia with moderate anemia, a variable need for blood transfusions, Heinz body formation, and a red cell morphology which is typical for such a condition. The level of HbA2was greatly increased (6.5–7.0%) as was the δ chain level (12% of total non‐α) probably because of the instability of Hb Lulu Island and the decreased ability of the βxchain to form dimers

ISSN:0361-8609    

DOI:10.1002/ajh.2830500106

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

年代:1995

数据来源: WILEY

摘要:

AbstractIn 55 patients with Ph1+CML under interferon (IFN) monotherapy, an immunohistochemical and morphometric study on pretreatment bone marrow biopsies was performed to evaluate the prognostic impact of clinical as well as histological disease features. For identification of megakaryocytes we used the PAS stain and CD61 to calculate the subtraction of precursors (pro‐ and megakaryoblasts). Demonstration of macrophages and their different subsets was carried out by PG‐M1 (CD68) and the GSA‐I lectin. The erythroid precursors were stained by Ret40f (anti‐glycophorin C). Density of argyrophilic (reticulin plus collagen) fibers was determined by applying Gomori's silver impregnation method. Clinical variables like state of hematological response to IFN administration, age, spleen and liver size, myeloblasts plus promyelocytes, basophils as well as basophils and eosinophils exerted a predictive capacity by univariate statistical analysis. However, when entering these factors into previously published risk models, i.e., the so‐called Sokal score and its modifications, to assess subgroups with different survival patterns or relative risk groups, a clear‐cut discrimination was not feasible. Bone marrow features of prognostic value consisted of megakaryocytes and their precursors, fibers, and pro‐ and erythroblasts. Only when including histological variables into a formerly reported Cox model, could a significant separation of patients into the different categories or relative risk groups be computated. In conclusion, the present data emphasize the prognostic impact of histological parameters to be considered in all clinical

ISSN:0361-8609    

DOI:10.1002/ajh.2830500107

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

年代:1995

数据来源: WILEY

摘要:

AbstractWith platelet activation, there is modulation of platelet surface molecule expression. In flow cytometric analyses of in vivo platelet activation, results are often confounded by activation induced in vitro by the preparative procedures. It is particularly important therefore to prevent or retard platelet activation as soon as possible after withdrawal of the blood sample. Taking blood into paraformaldehyde, or fixing the cells with paraformaldehyde as soon as possible after withdrawal, has been employed to prevent platelet activation in vitro, but paraformaldehyde‐fixed platelets cannot be further used in functional studies. We investigated the efficacy of Diatube‐H, a commercially available combination of platelet antagonists (theophylline, adenosine, and dipyridamole), in preventing or retarding platelet activation in vitro, along with its effects on modulation of platelet membrane glycoproteins (GP) and adhesion molecules. In contrast to blood taken into EDTA, blood taken into Diatube‐H vacutainer tubes could be stored at room temperature for up to 4 hr prior to paraformaldehyde fixation without significant in vitro platelet activation, as measured by CD62P, CD63 and modulation of GPIb and GPIIbIIIa surface expression. Hence, paraformaldehyde fixation could be deferred for several hours, permitting transport of samples from distant sites. Studies of thrombin‐induced platelet activation indicated that platelets taken into Diatube‐H remained functional i.e. were able to be activated. Expression of the CD29, CD49b and CD31 adhesion molecules on the platelet surface was unaffected by storage in Diatube‐H. The results suggest that Diatube‐H may be a useful reagent for flow cytometric studies of platelets when the samples cannot be processe

ISSN:0361-8609    

DOI:10.1002/ajh.2830500108

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe report a patient with sickle cell anemia (homozygous Hb SS) and typical features of sickle chest syndrome but with no response to exchange transfusion. A right atrial thrombus was found, and antiphospholipid antibodies were detected in his blood. He responded to thrombolytic therapy. The relationship between right atrial thrombus and massive pulmonary embolus, and the implications of an additional thrombophilic state in sickle cell disease are discussed.

ISSN:0361-8609    

DOI:10.1002/ajh.2830500109

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

年代:1995

数据来源: WILEY

摘要:

AbstractWe report an unusual hyperdiploid karyotype characterized by the simultaneous occurrence of tetrasomy 21 and trisomy 8 detected during early blastic evolution of a BCR‐ABL‐negative chronic myeloproliferative disorder. Blast cells from this patient showed a striking response to all‐trans‐retinoic acid (ATRA)‐induced differentiation as evaluated by CD15 expression following in vitro exposure to this inducer. Our report represents the first description of such a composite karyotype in human hematologic mal

ISSN:0361-8609    

DOI:10.1002/ajh.2830500110

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

年代:1995

数据来源: WILEY

摘要:

AbstractPancreatitis is a rare (∼2.0%) complication of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). The opposite finding has rarely been reported. We present a case of an 18 years old obese male with alcohol associated pancreatitis (amylase 840 IU/L) who three days after onset, as the pancreatitis subsided (amylase 341 U/L), developed TTP/HUS. The TTP/HUS was marked by oliguria and severe renal failure (creatinine 1,326 μmol/L), was treated with daily plasma exchanges, obtained a complete response, and recovered renal function (creatinine 115 μmol/L). Similarly, in six of seven other cases from the medical literature the TTP/HUS occurred within 2–3 days of the onset of pancrea

ISSN:0361-8609    

DOI:10.1002/ajh.2830500111

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

年代:1995

数据来源: WILEY