摘要:

AbstractThe purpose of this study was to test the value of the Ann Arbor staging procedures committee criteria in defining a group of newly diagnosed patients with Hodgkin disease who do not have involvement of the bone marrow and do not need this procedure performed. One hundred sixty‐six bilateral and 16 unilateral trephine bone marrow biopsies were performed in a consecutive series of 182 patients undergoing initial staging for Hodgkin disease. Bone marrow involvement was found in 13 patients. Advanced stage, defined as stage III or IV, occurring in 92%, anemia occurring in 100%, and “B” symptoms present in 100% were found to be the most sensitive indicators for bone marrow involvement. Osseous disease (99%), platelet count<150,000/mm3(98%), and WBC<4,800/mm3(94%) were the most specific parameters. The known association of bone marrow involvement with older patients, lymphocyte depleted histology, lower blood cell counts, anemia, advanced stage, and poorer survival is verified. The Ann Arbor staging procedures committee criteria for performing a bone marrow biopsy were shown to be 100% sensitive and 40% specific. Use of these criteria would have found all 13 patients with bone marrow involvement. Of the 73 patients who did not meet the criteria, no patient had bone marrow involvement. Use of the Ann Arbor staging procedures committee criteria is recomm

ISSN:0361-8609    

DOI:10.1002/ajh.2830300302

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

年代:1989

数据来源: WILEY

摘要:

AbstractFrom January 1980 to September 1986, 50 patients with Hodgkin disease, clinical stages (CS) IIIB (26 cases) and IVB (24 cases) were treated by three cycles of mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) chemotherapy, followed by high‐dose (40 Gy) (sub)total lymphoid irradiation, including the spleen. Ten patients (2 CS IIIB, 8 CS IVB) were in failure, and seven (4 CS IIIB, 3 CS IVB) died during their first complete remission (2 from treatment‐related complications, 1 from unknown cause, 4 from insufficient supportive care and/or a shortage of health supplies); three patients (CS IIIB) relapsed (2 alive in second complete remission, 1 deceased). After 7 years, actuarial survival and relapse‐free duration were, respectively, 64% for the 50 patients and 89% for the 40 patients in complete remission. Unfavourable outcome was observed in patients with pelvic nodal involvement. The low relapse rate (none in CS IVB) was the most striking result after brief chemotherapy followed by total lymphoid irradi

ISSN:0361-8609    

DOI:10.1002/ajh.2830300303

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

年代:1989

数据来源: WILEY

摘要:

AbstractHTLV‐I is associated with adult T‐cell leukemia/ lymphoma (ATL) characterized by monoclonal expansions of CD4+ T‐lymphocytes. In this report we describe a histologically benign, polyclonal HTLV‐I infection in a patient exhibiting both an absolute CD4+ and CD8+ lymphocytosis. Three T‐cell lines containing integrated HTLV‐I proviral copies established from this patient were initially polyclonal, but with time all grew out the same two clones as determined by analysis of their T‐cell antigen receptor beta chain gene rearrangements. The patient subsequently developed pulmonary and nasopharyngeal nodules containing HTLV‐I infected cells. Restriction analysis of the patient's HTLV‐I provirus revealed no differences from prototype HTLV‐I and the tax gene was normally expressed in vivo and in vitro. The patient's T‐lymphocytosis and HTLV‐I+ pulmonary tract nodules were put into a complete clinical remission by treatment with alkylating agents and steroids. Subsequently, the patient developed a severe immunodeficiency state and expired. Retrospective serologic and gene amplification assays for HIV‐1 demonstrated that he had been doubly infected from the time of presentation. Postmortem analysis by polymerase chain reaction revealed the presence of both HTLV‐I and HIV‐1 in lymphatic tissues and the testes; HIV‐1 wa

ISSN:0361-8609    

DOI:10.1002/ajh.2830300304

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

年代:1989

数据来源: WILEY

摘要:

AbstractThe pharmacokinetics of factor VIII were studied in a series of 20 hemophilia‐A patients undergoing surgery. Regardless of the type of operation, elimination of factor VIII was shown to be increased only in ten cases (50%) during the post‐operative period. In this subgroup of patients, factor VIII half‐life, measured immediately after surgery, was considerably shorter (mean = 9.6 hr, n = 10) than that determined in the same individual during the late operative period (mean = 17.8 hr, n = 10). These findings indicate that identification of patients with increased postoperative consumption of factor VIII can be of value in reducing the risk of hemorrhage in these subjects and in exposing other subjects with no postoperative increase in factor VIII clearance to less of the deficient factor. Data from 20 subjects were analyzed to construct a nomogram allowing individualized prediction of factor VIII dosing requirements. The nomogram, which is based on the “single point after a single dose” method, uses a value of factor VIII concentration measured at 10 hr after preoperative loading dose to predict the regimen producing the desired average steady‐state concentration of factor VIII (30, 60, or 9

ISSN:0361-8609    

DOI:10.1002/ajh.2830300305

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

年代:1989

数据来源: WILEY

摘要:

AbstractThe association of Type IIB von Willebrand disease (vWD) with chronic persistent thrombocytopenia and spontaneous platelet aggregation has recently been recognized. It has been shown that IIB von Willebrand factor (vWF) can initiate platelet aggregation by binding to the platelet glycoprotein (GP) lb receptor and inducing exposure of the Gpllb/llla fibrinogen receptor. In this study we demonstrate the increased binding of Type IIB Tampa vWF with normal platelets when compared with nonthrombocytopenic Type IIB vWF. Studies further demonstrate that spontaneous platelet aggregation initiated by IIB Tampa vWF can be blocked by a 52/48‐kDa fragment of normal vWF, which contains the binding domai

ISSN:0361-8609    

DOI:10.1002/ajh.2830300306

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

年代:1989

数据来源: WILEY

摘要:

AbstractThe effect of the synthetic vasopressin derivative 1‐desamino‐8D‐arginine vasopressin (DDAVP = desmopressin) on bleeding time was studied in three patients with Hermansky Pudlak syndrome. A good response was observed in this type of storage pool disease. DDAVP might be useful in managing the bleeding disorder found in patients with the Hermansky‐Pudlak s

ISSN:0361-8609    

DOI:10.1002/ajh.2830300307

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

年代:1989

数据来源: WILEY

摘要:

AbstractSix cases of microgranular variant acute promyelocytic leukemia (M3v) were studied by use of a multiparameter approach including morphology, cytochemistry, flow cytochemistry, flow cytometry, cytogenetics, and gene rearrangement. Three of six cases demonstrated both myeloid and monocytoid associated surface markers by flow cytometry. One of six cases had strong alpha‐naphthyl‐butyrate esterase (α‐NBE) activity in addition to myeloperoxidase activity. There was no correlation between percentage of positive monocytoid surface markers and intensity of cytoplasmic α‐NBE activity. Four of six cases also had a T‐cell‐associated surface antigen. Further studies indicated that the T‐cell markers appeared to be on the promyelocytes and that the T‐B receptor gene was not rearranged. Similarly, cytogenetics studies indicated only one clonal abnormality t(15q+; 17q‐). Whether these cases represent true “lineage infldelity” remains to be answered. Future important studies are needed on normal hematopoietic progenitor cells at early stages of development and childhood to study lineage‐specific characteristics and to determine whether co‐expression normally exis

ISSN:0361-8609    

DOI:10.1002/ajh.2830300308

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

年代:1989

数据来源: WILEY

摘要:

AbstractA cytochemical technique that uses silver nitrate staining has been used to study the nucleolus organizer activity in bone marrow cells from 13 patients with acute lymphocytic leukemia (ALL), 11 patients with chronic myelogenous leukemia (CML), 7 patients with acute myelogenous leukemia (AML), and 4 healthy persons. Our results indicate that the nucleolus organizer region (NOR) activity was significantly lower in the control group than in the ALL patients. The NOR activity level was significantly lower in both the CML patients in chronic phase and the AML patients than in the ALL group and similar to the control group. These disease‐related differences in NOR activity as detected by silver staining can be used as diagnostic procedure in evaluating human leukemia

ISSN:0361-8609    

DOI:10.1002/ajh.2830300309

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

年代:1989

数据来源: WILEY

摘要:

AbstractA 72‐year‐old man originally seen for anemia and thrombocytopenia was determined to have chronic lymphocytic leukemia (CLL). Bone marrow examination at the time of CLL diagnosis revealed a small but significant population of atypical blasts. Cytogenetic analysis of the bone marrow aspirate disclosed chromosomal abnormalities (‐7, +8) suggestive of a myelodysplastic syndrome. Shortly after treatment of the CLL, there was proliferation of the previously noted blast population, which cytochemical studies demonstrated to be of the myeloid series thus indicating acute myeloid leukemia superimposed on CLL. This report presents microscopic, cytogenetic, immunophenotypic, and cytochemical evidence to document the evolution of acute myeloid leukemia in the bone marrow of a patient with CLL after one course of chemoth

ISSN:0361-8609    

DOI:10.1002/ajh.2830300310

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

年代:1989

数据来源: WILEY

摘要:

AbstractA unique case of adult‐onset synchronous cyclic neutropenia and thrombocytopenia occurring at six‐week intervals is presented. Periods of cytopenia were associated with fever, myalgias, gastrointestinal symptoms, and mild mucocutaneous bleeding. Alternate‐day steroid treatment failed to correct the periodic fluctuations in peripheral blood counts but ameliorated symptoms during cytopenia. The treatment of cyclic hematopoiesis is rev

ISSN:0361-8609    

DOI:10.1002/ajh.2830300311

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

年代:1989

数据来源: WILEY