Systemic amyloidosis associated with chronic lymphocytic leukemia/small lymphocytic lymphoma.

abstract：:Sitosterolemia (phytosterolemia) is a rare inherited sterol storage disorder, characterized by significantly elevated plasma levels of plant sterols. The clinical features of sitosterolemia are xanthomas, premature atherosclerosis, arthritis, and, occasionally, liver function impair and hematologic abnormalities. This...

journal_title：American journal of hematology

authors： Wang Z,Cao L,Su Y,Wang G,Wang R,Yu Z,Bai X,Ruan C

更新日期：2014-03-01 00:00:00

abstract：:Thrombospondin (TSP), a large protein found in platelet alpha-granules (as TSP-1), mediates adhesion of sickle reticulocytes to cultured vascular endothelium. To further explore the physiologic relevance of this observation, we have measured plasma TSP levels and platelet TSP-1 content in subjects with sickle cell dis...

journal_title：American journal of hematology

authors： Browne PV,Mosher DF,Steinberg MH,Hebbel RP

更新日期：1996-04-01 00:00:00

abstract：:Using an in vitro system to evaluate the simultaneous use of two drugs, we previously have confirmed the synergism of vincristine and prednisolone cytotoxicity against lymphoid cells. Experiments were now carried out to determine whether other steroid hormones can be substituted for prednisolone. Partial or complete p...

journal_title：American journal of hematology

authors： Rosner F,Hirshaut Y,Grünwald HW,Deutsch S

更新日期：1978-01-01 00:00:00

abstract：:Genetic modifiers contribute to phenotypic variability in patients with sickle cell anemia (SCA). The influence of the bilirubin UDP-glucuronosyltransferase (UGT) 1A1 (TA)(n)TAA promoter polymorphism on bilirubin levels and gallbladder disease in SCA was examined using prospectively collected data from the Cooperative...

journal_title：American journal of hematology

authors： Carpenter SL,Lieff S,Howard TA,Eggleston B,Ware RE

更新日期：2008-10-01 00:00:00

abstract：:A 17-year-old male with bilineal hybrid acute leukemia is described. Two-color flow cytometric analysis of blast surface phenotype revealed that there were two groups of blasts which showed either CD 10+ CD 19+ CD 13- CD 33- or CD 10- CD 19- CD 13+ CD 33+, but not both. He developed a complete remission by treatment w...

journal_title：American journal of hematology

authors： Jinnai I,Kusumoto S,Shiomi Z,Bessho M,Saitoh M,Hirashima K,Minamihisamatsu M,Kamiyama T,Takase K

更新日期：1990-10-01 00:00:00

abstract：:Nucleotide sequence analysis of the 5' beta-globin gene flanking region has been carried out for numerous homozygous beta-thalassemia patients with different mutations and of various ethnic backgrounds. Four different rearrangements were found associated with numerous beta-thalassemia mutations. The (AT)X(T)Y repeat m...

journal_title：American journal of hematology

authors： Dimovski AJ,Adekile AD,Divoky V,Baysal E,Huisman TH

更新日期：1994-01-01 00:00:00

abstract：:BCR-ABL1-negative myeloproliferative neoplasms (MPNs) are clonal stem cell disorders defined by proliferation of one or more myeloid lineages, and carry an increased risk of vascular events and progression to myelofibrosis and leukemia. Portal hypertension (pHTN) occurs in 7-18% of MPN patients via both thrombotic and...

journal_title：American journal of hematology

authors： Reilly CR,Babushok DV,Martin K,Spivak JL,Streiff M,Bahirwani R,Mondschein J,Stein B,Moliterno A,Hexner EO

更新日期：2017-09-01 00:00:00

abstract：:The introduction of Imatinib (IM) has significantly altered the treatment for CML, although only limited follow-up results are available. As failure of Interferon-alpha had been associated with poor prognosis and results of IM-treatment in this patient group may allow earlier estimation of long-term benefits for early...

journal_title：American journal of hematology

authors： Hess G,Meyer RG,Schuch B,Bechthold K,El-Kholy I,Huber C

更新日期：2008-03-01 00:00:00

abstract：:The lupus anticoagulant may be accompanied by an acquired factor II deficiency and bleeding. We report on a patient with a lupus anticoagulant and factor II (Fll) deficiency responsive to Danazol. Acquired hypoprothrombinemia (FII) with the lupus anticoagulant (LA) may be accompanied by a hemorrhagic diathesis. A 64-y...

journal_title：American journal of hematology

authors： Williams S,Linardic C,Wilson O,Comp P,Gralnick HR

更新日期：1996-12-01 00:00:00

abstract：:We report on 3 female patients with immunologic thrombocytopenic purpura (ITP) for whom diagnostic procedures evidenced a chronic Hepatitis C virus (HCV) infection. In 2 cases, a transfusion performed more than 10 years ago represented the probable way of contamination. One patient received a course of steroids, which...

journal_title：American journal of hematology

authors： Bauduer F,Marty F,Larrouy M,Ducout L

更新日期：1998-04-01 00:00:00

abstract：:The activation pathways for the generation of enzymes involved in blood clotting, clot lysis, complement activation, and kinin generation are briefly reviewed. The interrelationship of the four systems is illustrated by the multiple functions of four key enzymes: Factor XIIa, kallikrein, plasmin, and C1 esterase. The ...

journal_title：American journal of hematology

authors： Murano G

更新日期：1978-01-01 00:00:00

abstract：:To elucidate the precise mechanisms of molecular and cellular regulation of hemopoiesis, it is necessary to develop a chemically defined culture assay for purified hemopoietic progenitors. To approach this long-term goal, we attempted to develop a serum-free culture system for enriched human progenitors that permits e...

journal_title：American journal of hematology

authors： Sonoda Y,Ogawa M

更新日期：1988-08-01 00:00:00

abstract：:This report describes the bone marrow findings in four patients whose marrow was involved by anaplastic large-cell Ki-1 lymphoma, an uncommon event in this type of lymphoma. In the marrow aspirate smears, the involvement was subtle, and was in the form of isolated large cells with irregular nuclear configuration, coar...

journal_title：American journal of hematology

authors： Wong KF,Chan JK,Ng CS,Chu YC,Lam PW,Yuen HL

更新日期：1991-06-01 00:00:00

abstract：:Seventy-three patients with hereditary spherocytosis (HS) (58 nonsplenectomized, 15 splenectomized) were studied to evaluate iron status and the adequacy of iron availability for erythropoiesis. Splenectomized patients, who had hemoglobin levels in the normal or upper normal range, had higher levels of serum iron, tra...

journal_title：American journal of hematology

authors： Zanella A,Barosi G,Berzuini A,Colombo MB,Quaglini S,Volpes D,Sirchia G

更新日期：1989-06-01 00:00:00

abstract：:Primary iron overload encompasses a variety of genetic iron overload syndromes, dominated in frequency by HFE-related, or Type 1 hemochromatosis, for which French diagnostic and therapeutic guidelines have been recently proposed. Differential diagnosis of Type 1 hemochromatosis can be made from both clinical data and ...

journal_title：American journal of hematology

authors： Brissot P

更新日期：2007-12-01 00:00:00

abstract：:The 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 mo...

journal_title：American journal of hematology

authors： Vives Corrons JL,Rozman C,Pujades MA,Colomer D,Perez Vila E,Anegon I,Gallart T,Vives Puiggrós J,Viñolas N,Montserrat E

更新日期：1988-03-01 00:00:00

abstract：:Flow cytometry (FCM) is an adjunct study to routine analysis of cerebrospinal fluid (CSF) to investigate for involvement by a hematologic malignancy. However, in our experience, FCM only infrequently detects abnormalities in CSF. To help optimize resources without forfeiting clinically important data, we sought to det...

journal_title：American journal of hematology

authors： Kovach AE,DeLelys ME,Kelliher AS,Dillon LJ,Hasserjian RP,Ferry JA,Preffer FI,Sohani AR

更新日期：2014-10-01 00:00:00

abstract：:Hb Saint Nazaire [beta 103 (G5) Phe-->Ile] was found in four apparently unrelated French families. The five patients carrying this hemoglobin have been detected because of a moderate erythrocytosis. The structural abnormality of Hb Saint Nazaire concerns the same residue as in Hb Heathrow [beta 103 (G5) Phe-->Leu). A ...

journal_title：American journal of hematology

authors： Wajcman H,Kister J,M'Rad A,Promé D,Milpied N,Rapp MJ,Harousseau JL,Riou J,Bardakdjian J,Galacteros F

更新日期：1993-09-01 00:00:00

abstract：:In some cases, bone marrow aplasia has been thought to result from immunologic abnormalities. Our patient had severe transfusion-dependent aplastic anemia, which responded to treatment with prednisone on two occasions. The exacerbations of aplastic anemia were associated with lymphocytic proliferation which on one occ...

journal_title：American journal of hematology

authors： Bodner S,Cohen HJ

更新日期：1981-11-01 00:00:00

abstract：BACKGROUND:T-cell lymphomas make up approximately 10%-15% of lymphoid malignancies. The frequency of these lymphomas varies geographically, with the highest incidence in parts of Asia. DIAGNOSIS:The diagnosis of aggressive peripheral T-cell lymphoma (PTCL) is usually made using the World Health Organization classifica...

journal_title：American journal of hematology

authors： Armitage JO

更新日期：2015-07-01 00:00:00

abstract：:In patients with light-chain myeloma or primary AL-amyloidosis, 24-hr light-chain excretion in the urine is considered an essential marker of the tumor mass. However, 24-hr urine collection and analysis may be cumbersome and prone to inaccuracy. Recently, a sensitive immunonephelometric assay for immunoglobulin free l...

journal_title：American journal of hematology

authors： Alyanakian MA,Abbas A,Delarue R,Arnulf B,Aucouturier P

更新日期：2004-04-01 00:00:00

abstract：:A patient with Felty's syndrome (FS) and persistent profound neutropenia developed recurrent infections and sepsis syndrome. No impairment of granulocyte-macrophage colony development was observed in vitro. Marrow morphology revealed an absence of mature neutrophil forms despite administration of granulocyte-colony st...

journal_title：American journal of hematology

authors： Pixley JS,Yoneda KY,Manalo PB

更新日期：1993-08-01 00:00:00

abstract：:We report the clinical, molecular, and immunohistological findings of 20 Chinese patients with mantle cell lymphoma diagnosed over a 10-year period. The disease affected mainly elderly patients (median age, 65.5 years) with a male predominance (M/F, 3/1). Eighty percent presented with advanced stage III/IV disease but...

journal_title：American journal of hematology

authors： Chim CS,Chan AC,Choo CK,Kwong YL,Lie AK,Liang R

更新日期：1998-12-01 00:00:00

abstract：:The goal of the present study was to search for criteria that allow one to distinguish between normal individuals and heterozygotes as well as compound heterozygotes for pyruvate kinase (PK) deficiency. As the residual activity of PK with heterozygotes was between 35% and 110% of the normal activity, it was necessary ...

journal_title：American journal of hematology

authors： Lakomek M,Winkler H,Linne S,Schröter W

更新日期：1989-08-01 00:00:00

abstract：:In polycythemia vera (PV), treatment with chlorambucil and radioactive phosphorus (p32) increases the risk of leukemic transformation from 1% to 13-14%. This risk has been estimated to be 1-5.9% with hydroxyurea (HU) therapy. When compared with historical controls, the risk with use of HU does not appear to be statist...

journal_title：American journal of hematology

authors： Nand S,Stock W,Godwin J,Fisher SG

更新日期：1996-05-01 00:00:00

abstract：:We present a case of acquired von Willebrand syndrome (AVWS) due to a monoclonal gammopathy of undetermined significance. Initially this case was diagnosed as congenital von Willebrand disease (VWD); however, re-examination of the medical history rendered a congenital bleeding disorder unlikely. A normal plasma von Wi...

journal_title：American journal of hematology

authors： Eikenboom JC,Tjernberg P,Van Marion V,Heering KJ

更新日期：2007-01-01 00:00:00

abstract：:A 63-year-old white man with myasthenia gravis and red cell aplasia was found to have hepatic iron overload. The differential diagnosis included hereditary hemochromatosis and secondary iron overload related to red cell aplasia. Family studies of siblings, including HLA typing, initially suggested a diagnosis of secon...

journal_title：American journal of hematology

authors： Adams PC

更新日期：1994-03-01 00:00:00

abstract：:A 67-year-old woman was admitted to our hospital because of lymphadenopathy and lymphocytosis. Monoclonal integration of HTLV-I provirus DNA was detected, and a diagnosis of adult T-cell leukemia (ATL) was made. Flow cytometry revealed that the ATL cells expressed CD20 as well as T-cell-associated antigens, and expres...

journal_title：American journal of hematology

authors： Yasukawa M,Arai J,Kakimoto M,Sakai I,Kohno H,Fujita S

更新日期：2001-01-01 00:00:00

abstract：:Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/...

journal_title：American journal of hematology

authors： Orfao A,Gonzalez M,San Miguel JF,Rios A,Canizo MC,Hernandez J,Maricato ML,Lopez Borrasca A

更新日期：1989-05-01 00:00:00

abstract：:Autoantibody production following allogeneic stem-cell transplantation is common and is often ascribed to the immune dysregulation associated with graft-versus-host disease. Recent data suggests that donor-memory B cells can be reactivated on exposure to antigen and result in antibody production in the recipient ident...

journal_title：American journal of hematology

authors： Ritchie DS,Sainani A,D'Souza A,Grigg AP

更新日期：2005-08-01 00:00:00