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：:Initially described in 1948 by Hertert thromboelastography (TEG) provides a real-time assessment of viscoelastic clot strength in whole blood. Rotational thromboelastometry (ROTEM) evolved from TEG technology and both devices generate output by transducing changes in the viscoelastic strength of a small sample of clot...

journal_title：American journal of hematology

authors： Whiting D,DiNardo JA

更新日期：2014-02-01 00:00:00

abstract：:Current information about clinical significance of IDH mutations in myelodysplastic syndromes (MDS), their association with other genetic alterations and the stability during disease progression is limited. In this study, IDH mutations were identified in 4.6% of 477 patients with MDS based on the FAB classification an...

journal_title：American journal of hematology

authors： Lin CC,Hou HA,Chou WC,Kuo YY,Liu CY,Chen CY,Lai YJ,Tseng MH,Huang CF,Chiang YC,Lee FY,Liu MC,Liu CW,Tang JL,Yao M,Huang SY,Ko BS,Wu SJ,Tsay W,Chen YC,Tien HF

更新日期：2014-02-01 00:00:00

abstract：:Iron deposition in combination with inflammatory and immunogenetic factors is involved in the pathophysiology of cardiac dysfunction in β-thalassemia major. We investigated the mechanical and endocrine function of the left atrium and ventricle to identify early signs of dysfunction. We studied 90 patients (mean age: 2...

journal_title：American journal of hematology

authors： Kostopoulou AG,Tsiapras DP,Chaidaroglou AS,De Giannis DE,Farmakis D,Kremastinos DT

更新日期：2014-01-01 00:00:00

abstract：:Over 300,000 infants are born annually with sickle cell anemia (SCA) in sub-Saharan Africa, and >50% die young from infection or anemia, usually without diagnosis of SCA. Early identification by newborn screening (NBS), followed by simple interventions dramatically reduced the mortality of SCA in the United States, bu...

journal_title：American journal of hematology

authors： McGann PT,Ferris MG,Ramamurthy U,Santos B,de Oliveira V,Bernardino L,Ware RE

更新日期：2013-12-01 00:00:00

abstract：:We examined inter- and intraobserver reproducibility and concordance between histological diagnosis and independently collected clinical findings in a large series of patients with the major subtypes of myeloproliferative neoplasms (MPNs) and controls. Seven hematopathologists reviewed 272 bone marrow biopsies includi...

journal_title：American journal of hematology

authors： Madelung AB,Bondo H,Stamp I,Loevgreen P,Nielsen SL,Falensteen A,Knudsen H,Ehinger M,Dahl-Sørensen R,Mortensen NB,Svendsen KD,Lange T,Ralfkiaer E,Nielsen K,Hasselbalch HC,Thiele J

更新日期：2013-12-01 00:00:00

abstract：:Eosinophilia may represent an early paraclinical sign of hematological malignant disease, but no reports exist on its predictive value for hematological malignancies. From the Copenhagen Primary Care Differential Count (CopDiff) Database, we identified 356,196 individuals with at least one differential cell count (DIF...

journal_title：American journal of hematology

authors： Andersen CL,Siersma VD,Hasselbalch HC,Lindegaard H,Vestergaard H,Felding P,de Fine Olivarius N,Bjerrum OW

更新日期：2013-10-01 00:00:00

abstract：:Protein S (ProS) is a physiological inhibitor of coagulation with an important function in the down-regulation of thrombin generation. ProS deficiency is a major risk factor for venous thrombosis. This study enrolled 40 ProS-deficient probands to investigate the molecular basis of hereditary ProS deficiency in Chinese...

journal_title：American journal of hematology

authors： Tang L,Jian XR,Hamasaki N,Guo T,Wang HF,Lu X,Wang QY,Hu Y

更新日期：2013-10-01 00:00:00

abstract：:The risk of graft-rejection after allogeneic hematopoietic cell transplantation using conventional cyclophosphamide-based conditioning is increased in patients with bone marrow failure syndromes (BMFS) who are heavily transfused and often HLA-alloimmunized. Fifty-six patients with BMFS underwent fludarabine-based redu...

journal_title：American journal of hematology

authors： Pantin J,Tian X,Shah AA,Kurlander R,Ramos C,Cook L,Khuu H,Stroncek D,Leitman S,Barrett J,Donohue T,Young NS,Geller N,Childs RW

更新日期：2013-10-01 00:00:00

abstract：:Thrombotic thrombocytopenic purpura (TTP) is a microangiopathy syndrome caused by a congenital or acquired deficiency of ADAMTS13, a plasma metalloprotease that cleaves von Willebrand factor (VWF) and thus prevents the formation of platelet-rich thrombi in the microcirculation. TTP can be fatal if not appropriately an...

journal_title：American journal of hematology

authors： Peyvandi F,Mannucci PM,Valsecchi C,Pontiggia S,Farina C,Retzios AD

更新日期：2013-10-01 00:00:00

abstract：:Plerixafor, a recently approved peripheral blood progenitor cell mobilizing agent, is often added to granulocyte-colony stimulating factor (G-CSF) to mobilize peripheral blood progenitor cells in patients with lymphoma or myeloma who cannot mobilize enough CD34+ cells with G-CSF alone to undergo autologous stem cell t...

journal_title：American journal of hematology

authors： Smith VR,Popat U,Ciurea S,Nieto Y,Anderlini P,Rondon G,Alousi A,Qazilbash M,Kebriaei P,Khouri I,de Lima M,Champlin R,Hosing C

更新日期：2013-09-01 00:00:00

abstract：:Aberrant promoter DNA methylation is a well-described mechanism of leukemogenesis within hematologic malignancies, including acute lymphoblastic leukemia (ALL). However, the importance of methylation patterns among the adolescent and young adult (AYA) ALL population has not been well established. DNA methylation of 18...

journal_title：American journal of hematology

authors： DiNardo CD,Gharibyan V,Yang H,Wei Y,Pierce S,Kantarjian HM,Garcia-Manero G,Rytting M

更新日期：2013-09-01 00:00:00

abstract：:The role of reduced-intensity conditioning (RIC) in adult acute lymphoblastic leukemia (ALL) remains unclear because of the small sample size, short follow-up duration, various regimens for conditioning and graft-versus-host disease (GVHD) prophylaxis, and the heterogeneity of selection criteria for transplantation. W...

journal_title：American journal of hematology

authors： Eom KS,Shin SH,Yoon JH,Yahng SA,Lee SE,Cho BS,Kim YJ,Kim HJ,Min CK,Kim DW,Lee JW,Min WS,Park CW,Lee S

更新日期：2013-08-01 00:00:00

abstract：:Most previous studies on telomere length (TL) in chronic lymphocytic leukemia (CLL) are based on referral cohorts including a high proportion of aggressive cases. Here, the impact of TL was analyzed in a population-based cohort of newly diagnosed CLL (n = 265) and in relation to other prognostic markers. Short telomer...

journal_title：American journal of hematology

authors： Mansouri L,Grabowski P,Degerman S,Svenson U,Gunnarsson R,Cahill N,Smedby KE,Geisler C,Juliusson G,Roos G,Rosenquist R

更新日期：2013-08-01 00:00:00

abstract：DISEASE OVERVIEW:Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extracutaneous organs. DIAGNOSIS:The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase...

journal_title：American journal of hematology

authors： Pardanani A

更新日期：2013-07-01 00:00:00

abstract：:Cobalamin (vitamin B12) deficiency is a common cause of megaloblastic anemia in Western populations. Laboratory evaluation of megaloblastic anemia frequently includes the assessment of patient cobalamin and folate status. Current total serum cobalamin measurements are performed in the clinical laboratory with competit...

journal_title：American journal of hematology

authors： Oberley MJ,Yang DT

更新日期：2013-06-01 00:00:00

abstract：:Lentiviral modification combined with ex vivo erythroid differentiation was used to stably inhibit RhAG expression, a critical component of the Rh(rhesus) membrane complex defective in the Rh(null) syndrome. The cultured red cells generated recapitulate the major alterations of native Rh(null) cells regarding antigen ...

journal_title：American journal of hematology

authors： Cambot M,Mazurier C,Canoui-Poitrine F,Hebert N,Picot J,Clay D,Picard V,Ripoche P,Douay L,Dubart-Kupperschmitt A,Cartron JP

更新日期：2013-05-01 00:00:00

abstract：:To clarify the presentation and course of patients with chronic lymphocytic leukemia (CLL) and amyloidosis. Mayo databases were interrogated for patients who carried a diagnosis of amyloidosis and CLL evaluated at Mayo Clinic, Rochester from January 1974 to October 2012. Charts were abstracted and data analyzed. Of th...

journal_title：American journal of hematology

authors： Kourelis TV,Gertz M,Zent C,Lacy M,Kyle R,Kapoor P,Zeldenrust S,Buadi F,Witzig T,Hayman S,Lust J,Russell S,Lin Y,Rajkumar VS,Kumar S,Leung N,Dingli D,Dispenzieri A

更新日期：2013-05-01 00:00:00

abstract：:Glomerular hyperfiltration and microalbuminuria/proteinuria are early manifestations of sickle nephropathy. The effects of hydroxyurea therapy on these renal manifestations of sickle cell anemia (SCA) are not well defined. Our objective was to investigate the effects of hydroxyurea on glomerular filtration rate (GFR) ...

journal_title：American journal of hematology

authors： Aygun B,Mortier NA,Smeltzer MP,Shulkin BL,Hankins JS,Ware RE

更新日期：2013-02-01 00:00:00

abstract：:Since multiple myeloma (MM) is still not-curable, the management of relapse remains challenging. Given the known efficacy of alkylating agents in MM, we conducted a phase I/II study to test a new three drug combination in which Fotemustine (Muphoran), an alkylating agent of nitrosurea family, was added to bortezomib +...

journal_title：American journal of hematology

authors： Mangiacavalli S,Pochintesta L,Pascutto C,Cocito F,Pompa A,Cazzola M,Corso A

更新日期：2013-02-01 00:00:00

abstract：:Familial Pseudohyperkalemia (FP) is a dominant red cell trait characterized by increased serum [K(+)] in whole blood stored at or below room temperature, without additional hematological abnormalities. Functional gene mapping and sequencing analysis of the candidate genes within the 2q35-q36 critical interval identifi...

journal_title：American journal of hematology

authors： Andolfo I,Alper SL,Delaunay J,Auriemma C,Russo R,Asci R,Esposito MR,Sharma AK,Shmukler BE,Brugnara C,De Franceschi L,Iolascon A

更新日期：2013-01-01 00:00:00

abstract：:Primary systemic light-chain (AL) amyloidosis a disorder characterized by accumulation of monoclonal light chains as aggregated amyloid fibrils in tissues of multiple organs to cause organ dysfunction and death (Kyle and Gertz, Semin Hematol 1995;32:45-59; Merlini and Bellotti, N Engl J Med 2003;349:583-596). Although...

journal_title：American journal of hematology

authors： Jun HJ,Kim K,Kim SJ,Mun YC,Bang SM,Won JH,Kim CS,Lee JH,Korean Multiple Myeloma Working Party (KMMWP).

更新日期：2013-01-01 00:00:00

abstract：:Clinical management of lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinemia (WM) has changed considerably over recent years, reflected in the use of new therapeutic agents (purine analogs, monoclonal antibodies, thalidomide- and bortezomib-based therapies). No population-based studies and few randomized tria...

journal_title：American journal of hematology

authors： Kristinsson SY,Eloranta S,Dickman PW,Andersson TM,Turesson I,Landgren O,Björkholm M

更新日期：2013-01-01 00:00:00

abstract：:The clinical development of lenalidomide (Revlimid™), then pomalidomide (Actimid™) as members of immunomodulatory drugs (IMiDs) for the treatment of multiple myeloma (MM), exemplifies how insight into disease biology can lead to design of effective therapeutic agents. Increased experience and understanding of IMiD's d...

journal_title：American journal of hematology

authors： Quach H,Kalff A,Spencer A

更新日期：2012-12-01 00:00:00

abstract：DISEASE OVERVIEW:Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 9,000 new patients annually and representing approximately 11% of all lymphomas in the United States. DIAGNOSIS:HL is composed of two distinct disease entities; the more commonly diagnosed classical HL and the rare nodular lymph...

journal_title：American journal of hematology

authors： Ansell SM

更新日期：2012-12-01 00:00:00

abstract：:Acute lymphoblastic leukemia (ALL) with distinct fusion transcripts has unique clinical features. In this study, the incidence, clinical characteristics, early treatment response, and outcomes of 1,004 Chinese pediatric ALLs were analyzed. Patients with TEL-AML1 and E2A-PBX1 fusion genes or other B cell precursor ALLs...

journal_title：American journal of hematology

authors： Gao C,Zhao XX,Li WJ,Cui L,Zhao W,Liu SG,Yue ZX,Jiao Y,Wu MY,Li ZG

更新日期：2012-11-01 00:00:00

abstract：:Measurements of the iron regulatory hormone hepcidin by various methodologies and laboratories are not harmonized. As a result different numeric results are obtained for the same clinical sample. We investigated whether better agreement between plasma hepcidin methods can be achieved by harmonization. Native plasma po...

journal_title：American journal of hematology

authors： Kroot JJ,van Herwaarden AE,Tjalsma H,Jansen RT,Hendriks JC,Swinkels DW

更新日期：2012-10-01 00:00:00

abstract：:The objective of this study is to establish a method to identify patients with primary immune thrombocytopenia (ITP) utilizing administrative data from diverse data sources that would be appropriate for epidemiologic studies of ITP, regardless of patients' age and source of health care. Medical records of the Oklahoma...

journal_title：American journal of hematology

authors： Terrell DR,Beebe LA,Vesely SK,Neas BR,Segal JB,George JN

更新日期：2012-09-01 00:00:00

abstract：:Although standard clinical prognostic factors predict outcome in diffuse large B-cell lymphoma (DLBCL), predicting the outcome of patients might be further refined using biological factors. We tested whether serum cytokines could provide prognostic information in DLBCL patients. Thirty cytokines were measured in pretr...

journal_title：American journal of hematology

authors： Ansell SM,Maurer MJ,Ziesmer SC,Slager SL,Habermann TM,Link BK,Witzig TE,Macon WR,Dogan A,Cerhan JR,Novak AJ

更新日期：2012-09-01 00:00:00

abstract：:Reverse transcription quantitative polymerase chain reaction (RTqPCR)is currently the most sensitive tool available for the routine monitoring of disease level in patients undergoing treatment for BCRABL1 associated malignancies. Considerable effort has been invested at both the local and international levels to stand...

journal_title：American journal of hematology

authors： Gerrard G,Mudge K,Foskett P,Stevens D,Alikian M,White HE,Cross NC,Apperley J,Foroni L

更新日期：2012-07-01 00:00:00

abstract：:Venous thromboembolism (VTE) recurs frequently. Greater height is associated with increased risk of incident VTE, but it is unclear whether height is related to risk of VTE recurrence. Recurrent VTE is associated with substantial morbidity and mortality, thus identifying individuals at greatest risk of experiencing a ...

journal_title：American journal of hematology

authors： Lutsey PL,Folsom AR

更新日期：2012-07-01 00:00:00

abstract：:Persistent thrombocytopenia after stem cell transplantation can lead to increased morbidity and mortality [1,2]. The underlying causes are often multifactorial in this patient population [3,4]. In autologous transplantation, thrombocytopenia is usually a result of poor engraftment or a sign of impending disease relaps...

journal_title：American journal of hematology

authors： Reid R,Bennett JM,Becker M,Chen Y,Milner L,Phillips GL 2nd,Liesveld J

更新日期：2012-07-01 00:00:00

abstract：:Monoclonal B-cell lymphocytosis (MBL) with normal lymphocyte counts is associated with decreased numbers of normal circulating B-cell subsets.Little is known about the distribution of normal lymphoid cells and their subsets in the peripheral blood (PB) of subjects with monoclonal B-cell lymphocytosis (MBL). In our stu...

journal_title：American journal of hematology

authors： Hauswirth AW,Almeida J,Nieto WG,Teodosio C,Rodriguez-Caballero A,Romero A,López A,Fernandez-Navarro P,Vega T,Perez-Andres M,Valent P,Jäger U,Orfao A,Primary Health Care Group of Salamanca for Study of MBL.

更新日期：2012-07-01 00:00:00

abstract：:A growing body of evidence suggests that iron overload is associated with inferior outcomes after myeloablative allogeneic hematopoietic stem cell transplantation (HSCT). However, all of those studies used surrogate markers of iron overload, especially serum ferritin, and most had a retrospective design. We conducted ...

journal_title：American journal of hematology

authors： Armand P,Sainvil MM,Kim HT,Rhodes J,Cutler C,Ho VT,Koreth J,Alyea EP,Neufeld EJ,Kwong RY,Soiffer RJ,Antin JH

更新日期：2012-06-01 00:00:00

abstract：:Regulatory T-cells (Tregs) are increased in chronic lymphocytic leukemia(CLL) and correlates with clinical and biological features of active/progressive disease. However, little is known about their ability to predict the time to first treatment (TFT). We evaluated 75 patients with Rai stage 0 CLL, in whom the absolut...

journal_title：American journal of hematology

authors： D'Arena G,D'Auria F,Simeon V,Laurenti L,Deaglio S,Mansueto G,Del Principe MI,Statuto T,Pietrantuono G,Guariglia R,Innocenti I,Martorelli MC,Villani O,De Feo V,Del Poeta G,Musto P

更新日期：2012-06-01 00:00:00

abstract：:Although treatment with plasma exchange increased the survival of patients with thrombotic thrombocytopenia purpura to 80% in the 1980s, no further increase of survival occurred over the next 20 years. However, more consistent use of adjuvant treatment with corticosteroids and rituximab in recent years has begun to fu...

journal_title：American journal of hematology

authors： George JN

更新日期：2012-05-01 00:00:00

abstract：:Imatinib is currently the standard treatment for chronic myeloid leukemia(CML). Previous studies have shown that imatinib affects bone metabolism in CML patients. However, these effects are not well-studied prospectively. The authors studied bone-mineral density (BMD) and bone metabolism in 17 CML patients and matched...

journal_title：American journal of hematology

authors： Jönsson S,Standal T,Olsson B,Mellström D,Wadenvik H

更新日期：2012-05-01 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：:Zoledronic acid (ZOL), an intravenous bisphosphonate, has been shown to reduce and delay the incidence of skeletal-related events (SREs) in multiple myeloma (MM) patients with bone disease. A retrospective claims-based analysis was conducted that used two distinct US managed care databases to examine the relationship ...

journal_title：American journal of hematology

authors： Henk HJ,Teitelbaum A,Perez JR,Kaura S

更新日期：2012-05-01 00:00:00

abstract：:The prevention of Rhesus D alloimmunization through Rh immune globulin (RhIg) administration is the major indication for the accurate detection and quantification of fetomaternal hemorrhage (FMH). In the setting of D incompatibility, D-positive fetal cells can sensitize the D-negative mother, resulting in maternal ant...

journal_title：American journal of hematology

authors： Kim YA,Makar RS

更新日期：2012-04-01 00:00:00