Retrospective review of the management of elective surgery with desmopressin and clotting factor concentrates in patients with von Willebrand disease.

abstract：:The hereditary persistence of fetal hemoglobin (HPFH)-6 is sporadically found in Thailand whereas the deletion-inversion type (G)gamma((A)gamma delta beta)(0)-thalassemia is described among Indians. We report a hitherto un-described case in which these two defects co-segregate. He was a 3-year-old Thai boy who had a f...

journal_title：American journal of hematology

authors： Fucharoen S,Pengjam Y,Surapot S,Fucharoen G,Sanchaisuriya K

更新日期：2002-10-01 00:00:00

abstract：:A case of a leukemic transformation following a 27-year history of idiopathic myelofibrosis (IMF) is presented. The patient had two chromosomal abnormalities: a deletion of chromosome 13, del 13(q12q14), and a deletion of chromosome 11, del 11(q14q23). This patient's final diagnosis was acute micromegakaryocytic leuke...

journal_title：American journal of hematology

authors： Toubai T,Tanaka J,Higa T,Ota S,Ibata M,Shono Y,Mashiko S,Miura Y,Umehara S,Kahata K,Toyoshima N,Morioka M,Asaka M,Kasai M,Imamura M

更新日期：2005-01-01 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：:Leukemic cells of 43 patients with acute promyelocytic leukemia (M3) were investigated morphologically and cytochemically to determine the percentage of aberrant enzymes and whether or not the presence impacts on the clinical outcome. Twelve patients (27.9%) showed alpha-naphthyl acetate esterase (ANAE) activity in th...

journal_title：American journal of hematology

authors： Matsuo T,Jain NC,Bennett JM

更新日期：1988-11-01 00:00:00

abstract：:The 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 ...

journal_title：American journal of hematology

authors： Longo G,Messori A,Morfini M,Baudo F,Ciavarella N,Cinotti S,Filimberti E,Giustarini G,Molinari AC,Ferrini PR

更新日期：1989-03-01 00:00:00

abstract：:This report describes studies investigating the use of a collagen binding assay to improve the laboratory monitoring of desmopressin (DDAVP) therapy in patients with von Willebrand's disease (vWD). We evaluated the response of seven patients with vWD (four type I, three type IIA) to DDAVP, administered using a standar...

journal_title：American journal of hematology

authors： Favaloro EJ,Dean M,Grispo L,Exner T,Koutts J

更新日期：1994-03-01 00:00:00

abstract：:Precursor B-ALL blasts may be positive for CD20 in up to 50% of cases. There are few reports on the use of the anti CD20 monoclonal antibody, rituximab, in children with B-ALL. We report on two adult patients with precursor B-ALL who developed significant hepatic toxicity during induction chemotherapy. Single agent ri...

journal_title：American journal of hematology

authors： Koren-Michowitz M,Rahimi-Levene N,Volcheck Y,Hardan I,Kornberg A

更新日期：2006-12-01 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：: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：:In this study, we investigated the incidence and prognostic impact of monosomal karyotype (MK) in 405 higher-risk Myelodysplastic Syndromes (MDS) patients treated with 5-AZA. The MK was present in 66 out of 405 (16.3%) patients, most of whom had complex karyotype (CK). MK was strongly associated with CK and the cytoge...

journal_title：American journal of hematology

authors： Papageorgiou SG,Vasilatou D,Kontos CK,Kotsianidis I,Symeonidis A,Galanopoulos AG,Hatzimichael E,Megalakaki A,Poulakidas E,Diamantopoulos P,Vassilakopoulos TP,Zikos P,Papadaki H,Mparmparousi D,Bouronikou E,Panayiotidis P,V

更新日期：2018-07-01 00:00:00

abstract：:The intravascular pool of human polymorphonuclear leukocytes (PMN) is composed of one compartment which is circulating and another that is marginated to the vascular endothelium. Administration of B-adrenergic agonists leads to a rapid demargination with an increase in the circulating PMN pool. The marginating PMN has...

journal_title：American journal of hematology

authors： Berkow RL,Dodson RW

更新日期：1987-01-01 00:00:00

abstract：:Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimisti...

journal_title：American journal of hematology

authors： Chaturvedi S,DeBaun MR

更新日期：2016-01-01 00:00:00

abstract：:A rare beta-thalassemia mutation at the splicing junction [namely, G-->C in intervening sequence (IVS) I-1] was found in a Japanese family. The proband and his mother were heterozygous for the mutation. Analysis of mRNA extracted from the reticulocyte-rich fraction obtained from the proband's mother revealed that the ...

journal_title：American journal of hematology

authors： Fujihara N,Tozuka M,Ueno I,Yamauchi K,Nakagoshi R,Ishikawa S,Hirota M,Okumura N,Ishii E,Katsuyama T

更新日期：2003-01-01 00:00:00

abstract：:Three Indonesian patients with identical genotypes, each compound heterozygotes for Filipino beta(o)-thalassemia/HbE, expressed different clinical severities. One patient has mild disease and is transfusion independent, while the other two are severely affected and transfusion dependent. The size of the Filipino beta(...

journal_title：American journal of hematology

authors： Setianingsih I,Williamson R,Daud D,Harahap A,Marzuki S,Forrest S

更新日期：1999-09-01 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：:Patients initially diagnosed with type 1 von Willebrand disease (VWD) have been reclassified as type 2 after a more exhaustive analysis in several studies. Our study's objectives were (1) to reanalyze patients that were previously diagnosed as type 1 to achieve a more accurate diagnosis and (2) to compare the von Will...

journal_title：American journal of hematology

authors： Penas N,Pérez-Rodríguez A,Torea JH,Lourés E,Noya MS,López-Fernández MF,Batlle J

更新日期：2005-11-01 00:00:00

abstract：:Thirty-seven patients with chronic phase chronic myeloid leukaemia and fourteen healthy controls have been evaluated for lineage differentiation with immunological markers on purified bone marrow CD34 positive cells by multiparameter flow cytometry. The myeloid-associated antigen CD33 and the stem cell factor receptor...

journal_title：American journal of hematology

authors： Martín-Henao GA,Quiroga R,Sureda A,García J

更新日期：1999-07-01 00:00:00

abstract：:Several studies reported improved outcomes of adolescents and young adults (AYA) with acute lymphoblastic leukemia (ALL) treated with pediatric-based ALL regimens. This prompted the prospective investigation of a pediatric Augmented Berlin-Frankfurt-Münster (ABFM) regimen, and its comparison with hyper-fractionated cy...

journal_title：American journal of hematology

authors： Rytting ME,Jabbour EJ,Jorgensen JL,Ravandi F,Franklin AR,Kadia TM,Pemmaraju N,Daver NG,Ferrajoli A,Garcia-Manero G,Konopleva MY,Borthakur G,Garris R,Wang S,Pierce S,Schroeder K,Kornblau SM,Thomas DA,Cortes JE,O'Brie

更新日期：2016-08-01 00:00:00

abstract：:Comparison of the chromosome findings obtained on routine examination (10-50 cells) of the marrows from patients with Ph1-positive CML with those based on a large number (110-500 cells) of metaphases in six of these patients, in whom appropriate material was available, revealed the presence of small percentages of ane...

journal_title：American journal of hematology

authors： Sonta SI,Sandberg AA

更新日期：1977-01-01 00:00:00

abstract：:A rare association of congenital afibrinogenemia and hereditary protein C deficiency is described in a 37-year-old female who suffered from ischemic necrosis in the left first toe. The diagnosis of afibrinogenemia was assessed by the absence of fibrinogen in clotting and immunological assays. The diagnosis of heredita...

journal_title：American journal of hematology

authors： Hanano M,Takahashi H,Itoh M,Shibata A

更新日期：1992-09-01 00:00:00

abstract：:We describe 3 episodes of microangiopathic hemolytic anemia (MAHA) in 2 solid organ recipients under FK506 (tacrolimus) therapy. In both cases, discontinuation of FK506 and treatment with plasma exchange, fresh frozen plasma replacement, corticosteroids, aspirin, and dipyridamole led to resolution of MAHA. In one pati...

journal_title：American journal of hematology

authors： Mach-Pascual S,Samii K,Beris P

更新日期：1996-08-01 00:00:00

abstract：:We report a patient with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) who presented with rapid enlargement of a cervical lymph node due to localized herpes simplex lymphadenitis, which was clinically indistinguishable from large cell (Richter's) transformation. The diagnosis was made by excisional...

journal_title：American journal of hematology

authors： Joseph L,Scott MA,Schichman SA,Zent CS

更新日期：2001-12-01 00:00:00

abstract：:A modified protein-bound cobalamin absorption test was used to study dietary cobalamin absorption in healthy adults of different age groups and patients with isolated low serum concentrations of cobalamin. Dietary cobalamin absorption was significantly reduced in healthy adults aged 55-75 years compared with young adu...

journal_title：American journal of hematology

authors： Scarlett JD,Read H,O'Dea K

更新日期：1992-02-01 00:00:00

abstract：:Febrile neutropenia (FN) is a frequent complication of cancer chemotherapy, which causes death in 4-21% of patients and worsens the quality of life of patients. As a simple and accurate way of identifying patients who are at risk of FN, a lymphocyte count on post-chemotherapy day 5 was suggested. To confirm the feasib...

journal_title：American journal of hematology

authors： Choi CW,Sung HJ,Park KH,Yoon SY,Kim SJ,Oh SC,Seo JH,Kim BS,Shin SW,Kim YH,Kim JS

更新日期：2003-08-01 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：:We aimed to comprehensively study sex differences in disease and patients' characteristics, treatment and outcomes in patients with follicular lymphoma (FL) in the United States (USA) utilizing the National LymphoCare Study registry (2004-2014). Among evaluable males (n = 1277) and females (n = 1375) with FL, females ...

journal_title：American journal of hematology

authors： Nabhan C,Zhou X,Day BM,Dawson K,Zelenetz AD,Friedberg JW,Cerhan JR,Link BK,Flowers CR

更新日期：2016-08-01 00:00:00

abstract：:Iron refractory iron deficiency anemia (IRIDA) is a rare hereditary disease caused by mutations in TMPRSS6 gene encoding Matriptase-2, a negative regulator of hepcidin transcription. Up to now, 53 IRIDA patients from 35 families with different ethnic origins have been reported and 41 TMPRSS6 mutations have been identi...

journal_title：American journal of hematology

authors： Poggiali E,Andreozzi F,Nava I,Consonni D,Graziadei G,Cappellini MD

更新日期：2015-04-01 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：:The recent progress in sensitive KIT D816V mutation analysis suggests that mutation analysis of peripheral blood (PB) represents a promising diagnostic test in mastocytosis. However, there is a need for systematic assessment of the analytical sensitivity and specificity of the approach in order to establish its value ...

journal_title：American journal of hematology

authors： Kristensen T,Vestergaard H,Bindslev-Jensen C,Møller MB,Broesby-Olsen S,Mastocytosis Centre, Odense University Hospital (MastOUH).

更新日期：2014-05-01 00:00:00

abstract：:The temporal development of the erythrocyte sedimentation rate (ESR) was studied in wide, short vacuum tubes. It was found that in about 3% of the specimens arriving in the laboratory the ESR developed in three different phases during 60 min, whereas the other showed only two. The specimens with three phases behaved s...

journal_title：American journal of hematology

authors： Kallner A

更新日期：1991-07-01 00:00:00