von Willebrand disease R1374C: type 2A or 2M? A challenge to the revised classification. High frequency in the northwest of Spain (Galicia).

abstract：:We describe a 25-year-old black woman who presented with a long history of anemia requiring transfusions during childhood and adolescence. Molecular analysis revealed her to be a compound heterozygote for the sickle mutation and the approximately 22.7 kb deletion associated with hemoglobin Kenya. This patient's clinic...

journal_title：American journal of hematology

authors： Waye JS,Cai SP,Eng B,Chui DH,Francombe WH

更新日期：1992-12-01 00:00:00

abstract：:A risk score based on three biological features (CD38, ZAP-70, and IGHV mutational status) was previously developed to predict progression-free survival (PFS) in untreated Binet A CLL patients. Here we perform a score validation analysis in a prospective and independent cohort of patients. Biological markers (CD38, ZA...

journal_title：American journal of hematology

authors： Gentile M,Cutrona G,Mosca L,Matis S,Fabris S,Lionetti M,Ilariucci F,Zupo S,Musolino C,Levato L,Molica S,Di Raimondo F,Vincelli I,Di Rienzo N,Pesce EA,Angrilli F,Federico M,Neri A,Ferrarini M,Morabito F

更新日期：2014-07-01 00:00:00

abstract：:Splenic lymphoma with villous lymphocytes (SLVL) is a relatively new entity with only a few reports published. We report seven cases of SLVL with detailed clinicopathologic and comprehensive immunophenotypic studies to further characterize this lymphoma, which is frequently confused with hairy cell leukemia and other ...

journal_title：American journal of hematology

authors： Sun T,Susin M,Brody J,Dittmar K,Teichberg S,Weiner R,Lin JH,Felber N

更新日期：1994-01-01 00:00:00

abstract：:We analyzed 12 MLL/ENL positive ALL patients consecutively diagnosed between 1999 and 2009. The MLL/ENL fusion was identified in 4/150 (2.6%), 8/993 (0.8%), and 0/70 of pediatric, adult, and elderly patients, respectively. Eight patients had a WBC count >50 × 10(9) /L. Ten cases had an evaluable immunophenotyping. A B...

journal_title：American journal of hematology

authors： Elia L,Grammatico S,Paoloni F,Vignetti M,Rago A,Cenfra N,Mecarocci S,Mancini M,Luciani M,Di Raimondo F,Cazzaniga G,Matarazzo M,Moleti ML,Santoro L,Gaidano G,Foà R,Mandelli F,Cimino G

更新日期：2011-12-01 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：:Defective fibrinolysis due to decreased tissue-type plasminogen activator (t-PA) activity is a well-established finding in patients with systemic lupus erythematosus (SLE). The possibility that this decrease in t-PA activity may be related to the presence of autoantibodies directed against t-PA, and the possible role ...

journal_title：American journal of hematology

authors： Ruiz-Arguelles A,Angles-Cano E,Perez-Romano B,Ruiz-Arguelles GJ,Deleze M,Alarcon-Segovia D,Gaussem P

更新日期：1995-06-01 00:00:00

abstract：:Allogeneic hematopoietic stem cell transplantation (HSCT) represents the only curative option for primary hemophagocytic lymphohistiocytosis (HLH), a rare disease of infants and young children, characterized by recurrent fever, hepatosplenomegaly, and cytopenia. We report a case of successful engraftment and stable fu...

journal_title：American journal of hematology

authors： Cesaro S,Gazzola MV,Marson P,Calore E,Caenazzo L,Destro R,De Silvestro G,Varotto S,Pillon M,Zanesco L,Messina C

更新日期：2003-02-01 00:00:00

abstract：:We have studied 91 patients with SS genotype, 44 children and 47 adults. Excluding the Cameroon and atypical haplotypes, the distribution in the children's sample exhibited 43% Benin, 38% Bantu, and 3% Senegal. In adults, the sample exhibited 46% Benin, 30% Bantu, and 9% Senegal (chi 2: 13.511, 2 df, P = 0.001). When ...

journal_title：American journal of hematology

authors： Muniz A,Corral L,Alaez C,Svarch E,Espinosa E,Carbonell N,di Leo R,Felicetti L,Nagel RL,Martinez G

更新日期：1995-06-01 00:00:00

abstract：:Intravenous immunoglobulin (IVIG) therapy is associated with rare reports of thromboembolic events and severe hyponatremia. We hypothesized that IVIG therapy may result in hyperproteinemia, increased serum viscosity, and pseudohyponatremia. We conducted a prospective observational study to evaluate the incidence of hy...

journal_title：American journal of hematology

authors： Steinberger BA,Ford SM,Coleman TA

更新日期：2003-06-01 00:00:00

abstract：:A small number of thromboembolic events, including deep venous thrombosis and myocardial infarction, have been reported in patients receiving IVIG. These events have primarily occurred in patients receiving high-dose IVIG and have been attributed to an increase in blood viscosity. To test the hypothesis that a procoag...

journal_title：American journal of hematology

authors： Wolberg AS,Kon RH,Monroe DM,Hoffman M

更新日期：2000-09-01 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：:Graft failure is a life-threatening complication after allogeneic hematopoietic stem cell transplantation (HSCT). Graft failure is more prevalent after umbilical cord blood transplantation (UCBT) compared with conventional adult stem cell sources. We identified 21 consecutive patients who experienced graft failure aft...

journal_title：American journal of hematology

authors： Singh H,Nikiforow S,Li S,Ballen KK,Spitzer TR,Soiffer R,Antin JH,Cutler C,Chen YB

更新日期：2014-12-01 00:00:00

abstract：:Recombinant DNA technology and protein engineering are creating hope that we can address ongoing challenges in hemophilia care such as reducing the costs of therapy, increasing the availability to the developing world, and improving the functional properties of these proteins. Technological advances to improve the hal...

journal_title：American journal of hematology

authors： Pipe SW

更新日期：2012-05-01 00:00:00

abstract：:Endothelin-1 (Et-1) is a vasoconstrictor produced by endothelial and vascular smooth muscle cells in response to hypoxia, which induces hypertrophy in cultured cardiac myocytes. We measured plasma Et-1 levels and left ventricular dimensions in 13 patients with sickle cell anemia (SCD) and in 12 African-American contro...

journal_title：American journal of hematology

authors： Werdehoff SG,Moore RB,Hoff CJ,Fillingim E,Hackman AM

更新日期：1998-07-01 00:00:00

abstract：:Although tyrosine kinase inhibitors have redefined the care of chronic myeloid leukemia (CML), these agents have not proved curative, likely due to resistance of the leukemia stem cells (LSC). While a number of potential therapeutic targets have emerged in CML, their expression in the LSC remains largely unknown. We t...

journal_title：American journal of hematology

authors： Gerber JM,Qin L,Kowalski J,Smith BD,Griffin CA,Vala MS,Collector MI,Perkins B,Zahurak M,Matsui W,Gocke CD,Sharkis SJ,Levitsky HI,Jones RJ

更新日期：2011-01-01 00:00:00

abstract：:Polycythemia vera (PCV) and multiple myeloma are both clonal disorders of hematopoietic stem cells. The simultaneous occurrence of these diseases in an individual patient is rare. A case of synchronous PCV and smoldering myeloma is presented and the literature is reviewed. The issues of clinical importance in this unu...

journal_title：American journal of hematology

authors： Fink L,Bauer F,Perry JJ

更新日期：1993-11-01 00:00:00

abstract：:Thalassemia is one of the most prevalent monogenic diseases usually caused by quantitative defects in the production of β-globin leading to severe anemia. Technological advances in genome sequencing, stem cell selection, viral vector development, transduction and gene editing strategies now allow for efficient exvivo ...

journal_title：American journal of hematology

authors： Soni S

更新日期：2020-09-01 00:00:00

abstract：:Idiopathic thrombocytopenic purpura is an autoimmune disease which involves opsonization of platelets by autoantibodies directed against different surface glycoproteins, leading to their premature destruction by the reticuloendothelial system. Management of patients with refractory ITP is difficult. Recent studies hav...

journal_title：American journal of hematology

authors： Braendstrup P,Bjerrum OW,Nielsen OJ,Jensen BA,Clausen NT,Hansen PB,Andersen I,Schmidt K,Andersen TM,Peterslund NA,Birgens HS,Plesner T,Pedersen BB,Hasselbalch HC

更新日期：2005-04-01 00:00:00

abstract：:A common side effect of opioids is nausea and vomiting; however, the incidence in hospitalized patients receiving opioids for acute pain is unknown. We performed a retrospective study in adult patients with sickle cell disease admitted for an acute pain crisis during a six-month period to evaluate the incidence of nau...

journal_title：American journal of hematology

authors： Shord SS,Chew L,Villano J

更新日期：2008-03-01 00:00:00

abstract：:The occurrence of factor VIII inhibitors in non-hemophilic patients is a rare event with a potentially lethal outcome. Despite its infrequent occurrence, the association of this inhibitor with multiple autoimmune diseases is well recognized. We report the case of a patient with the recently described autoimmune lympho...

journal_title：American journal of hematology

authors： Fang BS,Sneller MC,Straus SE,Frenkel L,Dale JK,Rick ME

更新日期：2000-07-01 00:00:00

abstract：:Two patients underwent allogeneic hematopoietic stem cell transplantation (HSCT) for acute myeloid leukemia. Chronic graft-versus-host disease (GVHD) developed, with persistent symptomatic oral lesions. At 2 and 6 years post-HSCT, both patients developed squamous cell carcinoma (SCC) of the tongue in areas previously ...

journal_title：American journal of hematology

authors： Szeto CH,Shek TW,Lie AK,Au WY,Yuen AP,Kwong YL

更新日期：2004-10-01 00:00:00

abstract：:In patients with acute myelogenous leukemia (AML), 34 out of 35 patients (97%) had cytochemically abnormal polymorphonuclear neutrophils (PMN). Some interesting regularity was noted in the appearance of cytochemical abnormalities of the PMNs. When peroxidase (PO) activity was low, both alkaline phosphatase (AP) and ch...

journal_title：American journal of hematology

authors： Suda T,Onai T,Maekawa T

更新日期：1983-08-01 00:00:00

abstract：DISEASE OVERVIEW:The myelodysplastic syndromes (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk of transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older males and in individuals with prior exposure to cytotoxic th...

journal_title：American journal of hematology

authors： Garcia-Manero G

更新日期：2015-09-01 00:00:00

abstract：:Reduced intensity conditioning (RIC) regimens are widely used in allogeneic stem cell transplantation (SCT). In this study, we retrospectively investigated the clinical outcomes of RIC with fludarabine (Flu; 180 mg/m(2)), intravenous busulfan (BU; 6.4 mg/kg) or oral BU (8 mg/kg), and low-dose total body irradiation (T...

journal_title：American journal of hematology

authors： Takahata M,Hashino S,Okada K,Onozawa M,Kahata K,Sugita J,Shigematsu A,Kondo T,Yamamoto S,Endo T,Nishio M,Ito YM,Tanaka J,Koike T,Asaka M,Imamura M

更新日期：2010-04-01 00:00:00

abstract：:The FAB classification of myelodysplastic syndromes (MDS) has been useful in predicting prognosis; however, additional methods are required to detect patients at high risk for early conversion to acute nonlymphoblastic leukemia (ANLL). Using a panel of monoclonal antibodies to myelomonocytic surface antigens (MMSA) an...

journal_title：American journal of hematology

authors： Mittelman M,Karcher DS,Kammerman LA,Lessin LS

更新日期：1993-07-01 00:00:00

abstract：:Platelet-rich plasma were treated with increasing concentrations of vitamin E (alpha-tocopherol). Washed platelets were exposed to oxidized low density lipoprotein (LDL) and examined by aggregometry and electron microscopy. The treatment of washed platelets by oxidized LDL induced morphological signs of activation lik...

journal_title：American journal of hematology

authors： Szuwart T,Dierichs RB,Zhao B

更新日期：1999-03-01 00:00:00

abstract：:We report here the haplotypes of 10 MstII-defined SS patients and a S/beta o thalassemia from the Central African Republic, exhibiting 7 different atypical haplotypes that are different from the typical Bantu haplotype that characterize over 93% of the beta s bearing chromosomes in that region of Africa. Of the seven ...

journal_title：American journal of hematology

authors： Srinivas R,Dunda O,Krishnamoorthy R,Fabry ME,Georges A,Labie D,Nagel RL

更新日期：1988-09-01 00:00:00

abstract：:How often elevated serum ferritin in primary-care patients reflects increased iron stores (normally 0.8 g in men, 0.4 g in women) is not known. The Hereditary Hemochromatosis and Iron Overload Screening (HEIRS) study screened 101,168 primary-care participants (44% Caucasians, 27% African-Americans, 14% Asians/Pacific ...

journal_title：American journal of hematology

authors： Gordeuk VR,Reboussin DM,McLaren CE,Barton JC,Acton RT,McLaren GD,Harris EL,Reiss JA,Adams PC,Speechley M,Phatak PD,Sholinsky P,Eckfeldt JH,Chen WP,Passmore L,Dawkins FW

更新日期：2008-08-01 00:00:00

abstract：:Human T-cell leukemia virus (HTLV-I) is known to be associated with certain hematologic malignancies, and a related virus, HTLV-III/LAV, might be the cause of AIDS. Some persons with AIDS have had evidence of HTLV-I infection. Unrelated to these findings, it has been suggested that HTLV-I is transmitted via blood prod...

journal_title：American journal of hematology

authors： Jason JM,McDougal JS,Cabradilla C,Kalyanaraman VS,Evatt BL

更新日期：1985-10-01 00:00:00

abstract：:The increased adhesiveness of sickle erythrocytes (SS RBC) to endothelial cells has been confirmed in a static system utilizing fresh umbilical vein endothelium. Adherence of SS RBC to the endothelium was as great in the presence of calcium-containing buffer as when incubated in plasma. SS RBC suspended in autologous ...

journal_title：American journal of hematology

authors： Antonucci R,Walker R,Herion J,Orringer E

更新日期：1990-05-01 00:00:00