Clinical course and molecular characterization of a compound heterozygote for sickle hemoglobin and hemoglobin Kenya.

abstract：:Anemia is a major cause of morbidity and mortality among patients with sickle cell anemia. In this study, 108 episodes of severe anemia were prospectively evaluated in 108 patients with hemoglobin SS disease attending the pediatric sickle cell clinic of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Youn...

journal_title：American journal of hematology

authors： Juwah AI,Nlemadim A,Kaine W

更新日期：2003-03-01 00:00:00

abstract：:A single point mutation of the factor V (FV) gene, leading to the substitution Arg506Gln in the FV molecule (FV-Leiden) and hence resistance to its breakdown by activated protein C (APC), is the most prevalent risk factor for venous thrombosis in the Caucasians. A ratio determined by activated partial thromboplastin t...

journal_title：American journal of hematology

authors： Ts'ao C,Neofotistos D,Oropeza M,Vahabinejad S,Finn WG

更新日期：1997-03-01 00:00:00

abstract：:Recent literature has demonstrated concern over the risk of Pneumocystis jirovecii pneumonia (PJP) when administering rituximab with combination chemotherapy such as in R-CHOP; however, the exact risk and potential need for prophylaxis is unknown. We sought to determine the incidence of PJP infection following R-CHOP ...

journal_title：American journal of hematology

authors： Barreto JN,Ice LL,Thompson CA,Tosh PK,Osmon DR,Dierkhising RA,Plevak MF,Limper AH

更新日期：2016-11-01 00:00:00

abstract：:Evaluation of pharmacologic agents that stimulate fetal hemoglobin production has been done mainly in baboons and macaques. We investigated whether results in transgenic mice can predict the stimulation of fetal hemoglobin in primates, by testing gamma globin induction in response to a new ribonucleotide reductase inh...

journal_title：American journal of hematology

authors： Pace BS,Elford HL,Stamatoyannopoulos G

更新日期：1994-02-01 00:00:00

abstract：:The acute sickle cell painful episode is the most common cause of hospitalization of patients with sickle cell anemia. Its detailed clinical features and peri-discharge features are not well known. In order to determine the actual pattern of hospital admissions of patients with SS and the causes of frequent hospital r...

journal_title：American journal of hematology

authors： Ballas SK,Lusardi M

更新日期：2005-05-01 00:00:00

abstract：:In a phase II trial, we evaluated chlorambucil and rituximab (CLB-R) as first-line induction treatment with or without R as maintenance for elderly chronic lymphocytic leukemia (CLL) patients. Treatment consisted of eight 28-day cycles of CLB (8 mg/m(2) /day, days 1-7) and R (day 1 of cycle 3, 375 mg/m(2) ; cycles 4-8...

journal_title：American journal of hematology

authors： Foà R,Del Giudice I,Cuneo A,Del Poeta G,Ciolli S,Di Raimondo F,Lauria F,Cencini E,Rigolin GM,Cortelezzi A,Nobile F,Callea V,Brugiatelli M,Massaia M,Molica S,Trentin L,Rizzi R,Specchia G,Di Serio F,Orsucci L,Ambros

更新日期：2014-05-01 00:00:00

abstract：:This study demonstrates the quantitative characteristics of the first patient-reported outcome (PRO) tool developed for patients with nontransfusion-dependent β-thalassemia (NTDT), the NTDT-PRO© . A multicenter validation study was performed over 24 weeks, involving 48 patients from Italy, Lebanon, Greece, and Thailan...

journal_title：American journal of hematology

authors： Taher A,Cappellini MD,Viprakasit V,Sutcharitchan P,Mahmoud D,Laadem A,Khan A,Gwaltney C,Harding G,Attie K,Zhang X,Zou J,Pariseau J,Henry Hu X,Kattamis A

更新日期：2019-02-01 00:00:00

abstract：:Community practice experience allows a nonselective care of patient using information derived from a more controlled clinical trial environment. We present our community experience with multiple myeloma patients with advanced age, long disease duration since diagnosis, advanced stage, multiple prior therapies includin...

journal_title：American journal of hematology

authors： Onitilo AA,Engel J,Olatosi B,Fagbemi S

更新日期：2007-07-01 00:00:00

abstract：:Several syndromes characterized by striking eosinophilia may be complicated by thrombosis. The experiments described indicate that, paradoxically, eosinophils and certain of their constituents inhibit the activation of Hageman factor (HF, factor XII). In earlier studies, suspensions of mixed types of granulocytes, oth...

journal_title：American journal of hematology

authors： Ratnoff OD,Gleich GJ,Shurin SB,Kazura J,Everson B,Embury P

更新日期：1993-01-01 00:00:00

abstract：:A 68-year-old previously well woman developed sudden onset of limb gangrene in association with liver dysfunction. An immediately acting inhibitor to factor V with some of the features of lupus anticoagulant was demonstrated. The patient required limb amputation within 2 weeks and activity of the anticoagulant seemed ...

journal_title：American journal of hematology

authors： Kapur A,Kelsey PR,Isaacs PE

更新日期：1993-04-01 00:00:00

abstract：:Decreased von Willebrand factor (VWF)-cleaving protease activity (<5%) has been implicated in patients with congenital thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (Upshaw-Schulman syndrome) and associated with mutations within the ADAMTS13 gene. In this report, we describe longitudinal studies in a p...

journal_title：American journal of hematology

authors： Snider CE,Moore JC,Warkentin TE,Finch CN,Hayward CP,Kelton JG

更新日期：2004-12-01 00:00:00

abstract：:Quantification of sickle hemoglobin (HbS) in patients with sickle cell disease (SCD) undergoing hydroxyurea or chronic transfusion therapy is essential to monitoring the effectiveness of these therapies. The clinical monitoring of %HbS using conventional laboratory methods is limited by high per-test costs and long tu...

journal_title：American journal of hematology

authors： Piety NZ,Yang X,Lezzar D,George A,Shevkoplyas SS

更新日期：2015-06-01 00:00:00

abstract：:Pentosan polysulfate is a low-molecular-weight sulfated polysaccharide used as an antithrombotic drug. We present two patients who developed thrombocytopenia and venous thrombosis during treatment with pentosan polysulfate. The relationship between pentosan polysulfate and thrombocytopenia is supported by platelet agg...

journal_title：American journal of hematology

authors： Tardy-Poncet B,Tardy B,Grelac F,Reynaud J,Mismetti P,Bertrand JC,Guyotat D

更新日期：1994-03-01 00:00:00

abstract：:Cobalamin derivatives serve as coenzymes for the body's two cobalamin-dependent enzymes--adenosylcobalamin-dependent methylmalonyl CoA mutase, and methylcobalamin-dependent methionine methyltransferase. This essay reviews, in brief form and in personal terms, the history, beginning in the mid-1950s, of how these enzym...

journal_title：American journal of hematology

authors： Beck WS

更新日期：1990-06-01 00:00:00

abstract：:Transplant-associated thrombotic microangiopathy (TA-TMA) has a wide range of presentations after hematopoietic stem-cell transplantation (HSCT). We retrospectively studied the risk factors and outcomes of patients with early (≤day 100) and late (>day 100) TA-TMA. Among the 1451 HSCT recipients, early TA-TMA occurred ...

journal_title：American journal of hematology

authors： Heybeli C,Sridharan M,Alkhateeb HB,Villasboas Bisneto JC,Buadi FK,Chen D,Dingli D,Dispenzieri A,Gertz MA,Go RS,Hashmi SK,Hayman SR,Hogan WJ,Inwards DJ,Kenderian SS,Kumar SK,Litzow MR,Porrata LF,Lacy MQ,Micallef IN,

更新日期：2020-07-02 00:00:00

abstract：:Hemoglobin Lyon-Bron was found in two members of a family of German ascent presenting with a moderate normocytic anemia. In this alpha 2 globin variant, the N-terminal valine of the chain was replaced by an alanine. Electrospray mass spectrometry of the alpha chain showed that, as normally, the initiator methionine wa...

journal_title：American journal of hematology

authors： Lacan P,Souillet G,Aubry M,Promé D,Richelme-David S,Kister J,Wajcman H,Francina A

更新日期：2002-03-01 00:00:00

abstract：:Anthracyclines have contributed to a marked increase in survival in different types of cancer [1,2]. Unfortunately, they are associated with dose-dependent cardiotoxicity and heart failure (HF) [3–8]. Change to a weekly dosage schedule with slow infusions has been tested, a strategy that requires more frequent hospita...

journal_title：American journal of hematology

authors： Georgakopoulos P,Roussou P,Matsakas E,Karavidas A,Anagnostopoulos N,Marinakis T,Galanopoulos A,Georgiakodis F,Zimeras S,Kyriakidis M,Ahimastos A

更新日期：2010-11-01 00:00:00

abstract：:Drug metabolizing genes are involved in the detoxification of chemical carcinogens. Polymorphisms in drug-metabolizing genes affect the risk of some forms of cancer. We analyzed six polymorphisms to evaluate their association with risk for non-Hodgkin lymphoma (NHL), and to examine whether smoking modifies these assoc...

journal_title：American journal of hematology

authors： Kim HN,Kim NY,Yu L,Kim YK,Lee IK,Yang DH,Lee JJ,Shin MH,Park KS,Choi JS,Kim HJ

更新日期：2009-12-01 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：:Extensive extralymphatic Hodgkin disease developed in a young man with common variable immunodeficiency manifested by hypogammaglobulinemia, recurrent sinopulmonary infections, and multiple autoimmune phenomena. Both humoral and cell-mediated immune dysfunction were present prior to treatment. After two cycles of chem...

journal_title：American journal of hematology

authors： Fesus SM,Hagemeister FB,Manning J

更新日期：1989-10-01 00:00:00

abstract：:Central nervous system (CNS) thrombotic events are a well-known complication of acute lymphoblastic leukemia (ALL) induction therapy, especially with treatments including l-asparaginase (l-ASP). Data on risk factors and clinical evolution is still lacking in adult patients. We report on the clinical evolution of 22 CN...

journal_title：American journal of hematology

authors： Couturier MA,Huguet F,Chevallier P,Suarez F,Thomas X,Escoffre-Barbe M,Cacheux V,Pignon JM,Bonmati C,Sanhes L,Bories P,Daguindau E,Dorvaux V,Reman O,Frayfer J,Orvain C,Lhéritier V,Ifrah N,Dombret H,Hunault-Berger M,

更新日期：2015-11-01 00:00:00

abstract：:Twenty-two normal volunteers had approximately eight, 2-hr-long leukapheresis procedures over a 2-year period and their natural killer (NK) cell function was prospectively measured. The NK activity of the preprocedure peripheral blood (pre-PB) was found to correlate well with the NK activity of the inital leukocytes r...

journal_title：American journal of hematology

authors： Stevenson HC,Beman JS,Crisp B,Coggin D,Kanapa DJ,Miller P,Herberman RB,Maluish AE

更新日期：1986-06-01 00:00:00

abstract：:The clinical features of patients with splenic marginal zone cell lymphoma (SMZCL) have rarely been reported. In the present study, immunological abnormalities, particularly hematological abnormalities, observed in SMZCL were described. Autoimmune hemolytic anemia, immune thrombocytopenia, and appearance of lupus anti...

journal_title：American journal of hematology

authors： Murakami H,Irisawa H,Saitoh T,Matsushima T,Tamura J,Sawamura M,Karasawa M,Hosomura Y,Kojima M

更新日期：1997-11-01 00:00:00

abstract：:One of the more intriguing aspects of the spleen is the protection against certain bacterial infections afforded by its unique vascular and immune function. There have been extensive clinical surveys which indicate an incidence of overwhelming postsplenectomy infection (OPSI) above that of the disease for which the sp...

journal_title：American journal of hematology

authors： Krivit W

更新日期：1977-01-01 00:00:00

abstract：:The molecular basis of triosephosphate isomerase (TPI) deficiency was studied in 3 patients from three separate families. In all 3 patients, genomic DNA directly sequenced after amplification by the polymerase chain reaction exhibited the point mutation TPI315C amino acid 104 Glu-->Asp. Although other mutations known ...

journal_title：American journal of hematology

authors： Schneider A,Westwood B,Yim C,Prchal J,Berkow R,Labotka R,Warrier R,Beutler E

更新日期：1995-12-01 00:00:00

abstract：:Factor V G1691A (FV-Leiden) and prothrombin G20210A mutations are major inherited risk factors for venous thrombosis. Recently, it was suggested that both mutations, through stimulation of venous and placental thrombosis events, were strongly associated with recurrent idiopathic miscarriages, although other studies di...

journal_title：American journal of hematology

authors： Finan RR,Tamim H,Ameen G,Sharida HE,Rashid M,Almawi WY

更新日期：2002-12-01 00:00:00

abstract：:Despite advances in chemotherapeutic agents, the prognosis for some cancers remains extremely poor, suggesting the need for other treatment modalities. Immunotherapy appears an ideal approach because the mechanisms of tumor cell killing induced by tumor vaccines are different from those from chemotherapy. Various inve...

journal_title：American journal of hematology

authors： Dadabayev AR,Wang Z,Zhang Y,Zhang J,Robinson WR,Lim SH

更新日期：2005-09-01 00:00:00

abstract：:Although karyotypic abnormalities are well documented in B-cell chronic lymphocytic leukemia (B-CLL), few sequential cytogenetic studies have been done. In this study, peripheral blood lymphocytes from fifty-one patients with B-CLL were sequentially karyotyped over a mean interval of 13.8 months (range, one to 51 mont...

journal_title：American journal of hematology

authors： Finn WG,Kay NE,Kroft SH,Church S,Peterson LC

更新日期：1998-11-01 00:00:00

abstract：:The "platelet function analyzer" (PFA)-100 was first introduced to us in 1995. Since then, the instrument has appeared in over 50 reviews and almost 1000 publications. Recently, the PFA-100 has been "upgraded" to the PFA-200, which has transformed the user interface and electronic management, but retained the fundamen...

journal_title：American journal of hematology

authors： Favaloro EJ

更新日期：2017-04-01 00:00:00

abstract：:Red blood cells from patients with sickle cell disease (SS RBC) adhere to laminin and over-express the high-affinity laminin receptor basal cell adhesion molecule/Lutheran protein (B-CAM/LU). This receptor has recently been shown to undergo activation in vitro through a protein kinase A-dependent mechanism. Low-densit...

journal_title：American journal of hematology

authors： Zen Q,Batchvarova M,Twyman CA,Eyler CE,Qiu H,De Castro LM,Telen MJ

更新日期：2004-02-01 00:00:00