ADAMTS13 cleavage efficiency is altered by mutagenic and, to a lesser extent, polymorphic sequence changes in the A1 and A2 domains of von Willebrand factor.

abstract：:Immuno-alkaline phosphatase staining (by the APAAP technique) has been used to identify promegakaryoblasts in cell smears from 10 cases of leukaemia (three acute leukaemia, seven blast transformations). In all cases promegakaryoblasts were labelled by at least two anti-platelet glycoprotein (gp) antibodies, the highes...

journal_title：British journal of haematology

authors： Erber WN,Breton-Gorius J,Villeval JL,Oscier DG,Bai Y,Mason DY

更新日期：1987-01-01 00:00:00

abstract：:The effect of urea on the oxygen affinity of sickle cells and normal cells was studied up to a concentration of about 1.0 M. Besides the increase in oxygen affinity found in both normal and sickle cells there was a further increase found only in the sickle cells. This specific increase was caused by the direct inhibit...

journal_title：British journal of haematology

authors： May A,Huehns ER

更新日期：1975-05-01 00:00:00

abstract：:The significance of paroxysmal nocturnal haemoglobinuria (PNH(pos) ) cells and leucocyte subset telomere lengths in paediatric aplastic anaemia (AA) is unknown. Among 22 children receiving immunosuppressive therapy (IST) for AA, 73% (16/22) were PNH(pos) , of whom 94% achieved at least a partial response (PR) to IST; ...

journal_title：British journal of haematology

authors： Tutelman PR,Aubert G,Milner RA,Dalal BI,Schultz KR,Deyell RJ

更新日期：2014-03-01 00:00:00

abstract：:Mature (peripheral) T-cell lymphoma (PTCL) other than anaplastic large cell lymphoma is a heterogeneous group of diseases and exceedingly rare in children and adolescents. Survival rates range between 46% and 85%. This study reports the disease characteristics, treatment and outcome of all patients with the diagnosis ...

journal_title：British journal of haematology

authors： Kontny U,Oschlies I,Woessmann W,Burkhardt B,Lisfeld J,Salzburg J,Janda A,Attarbaschi A,Niggli F,Zimmermann M,Reiter A,Klapper W

更新日期：2015-03-01 00:00:00

abstract：:Therapeutic options for treatment of recurrence of leukaemia after allogeneic bone marrow transplantation (BMT) are limited. A beneficial effect of donor lymphocyte infusions (DLI) has not previously been described in acute myeloid leukaemia (AML) relapse. We report a case of AML with t(8;21), relapsing 3 months after...

journal_title：British journal of haematology

authors： Buzyn-Veil A,Belanger C,Audat F,Hermine O,Bodemer C,Ribrag V,Radford I,Poirel H,Chane C,Valensi F,Macintyre E,Varet B

更新日期：1996-02-01 00:00:00

abstract：:We describe two British families with similar, dominantly-inherited, temperature-related variants of hereditary stomatocytosis, consistent with the original description of 'cryohydrocytosis'. The cells show a 5-6-fold increase in passive permeability at 37 degrees C with abnormal intracellular Na and K levels at 15-20...

journal_title：British journal of haematology

authors： Coles SE,Chetty MC,Ho MM,Nicolaou A,Kearney JW,Wright SD,Stewart GW

更新日期：1999-06-01 00:00:00

abstract：:Glucose-6-phosphate dehydrogenase (G6PD) deficient individuals are at increased risk of developing haemolysis following treatment with various antimalarial drugs. Reliable field tests for G6PD deficiency are thus needed in chemotherapy studies and their validity has to be assessed. In two phase II clinical trials on m...

journal_title：British journal of haematology

authors： Meissner PE,Coulibaly B,Mandi G,Mansmann U,Witte S,Schiek W,Müller O,Schirmer RH,Mockenhaupt FP,Bienzle U

更新日期：2005-11-01 00:00:00

abstract：:Detection of a 17p13.1 deletion (loss of TP53) or 11q22.3 deletion (loss of ATM), by fluorescence in situ hybridization (FISH), in chronic lymphocytic leukaemia (CLL) patients is associated with a poorer prognosis. Because TP53 and ATM are integral to the TP53 pathway, we hypothesized that 17p13.1- (17p-) and 11q22.3-...

journal_title：British journal of haematology

authors： Greipp PT,Smoley SA,Viswanatha DS,Frederick LS,Rabe KG,Sharma RG,Slager SL,Van Dyke DL,Shanafelt TD,Tschumper RC,Zent CS

更新日期：2013-11-01 00:00:00

abstract：:Five monoclonal antibodies have been tested for their ability to bind to myeloid precursor cells in normal human bone marrow. Indirect immunofluorescence and the fluorescence activated cell sorter was used to separate cells according to their reactivity for trial culture in vitro in order to grow granulocyte-macrophag...

journal_title：British journal of haematology

authors： Crawford DH,Francis GE,Wing MA,Edwards AJ,Janossy G,Hoffbrand AV,Prentice HG,Secher D,McConnell I,Kung PC,Goldstein G

更新日期：1981-10-01 00:00:00

abstract：:The PFA-100(R) (PFA) diagnostic system for the detection of platelet dysfunction was evaluated to determine reference ranges in a normal population. The PFA determines the primary haemostasis capacity (PHC) of anticoagulated whole blood, expressed by the system's closure time (CT). In this study the CT reference range...

journal_title：British journal of haematology

authors： Böck M,De Haan J,Beck KH,Gutensohn K,Hertfelder HJ,Karger R,Heim MU,Beeser H,Weber D,Kretschmer V

更新日期：1999-09-01 00:00:00

abstract：:The use of microfabrication technology in the study of biological systems continues to grow rapidly in both prevalence and ascendancy. Customised microdevices that provide superior results than traditional macroscopic methods can be designed in order to investigate specific cell types and cellular processes. This stud...

journal_title：British journal of haematology

authors： Gifford SC,Derganc J,Shevkoplyas SS,Yoshida T,Bitensky MW

更新日期：2006-11-01 00:00:00

abstract：:Disease progression occurs in over 1% of monoclonal gammopathy of undetermined significance, monoclonal B-cell lymphocytosis and early stage chronic lymphocytic leukaemia patients every year therefore regular monitoring is indicated. We assessed the efficacy of an outreach service to replace clinic monitoring using lo...

journal_title：British journal of haematology

authors： Rawstron AC,Jones RA,Ferguson C,Hughes G,Selby P,Reid C,Dalal S,Howard M,Smith G,Hillmen P,Owen RG,Jack AS

更新日期：2007-12-01 00:00:00

abstract：:Plasmas having no detectable factor VIII-related antigen but moderate factor VIII coagulant were obtained from two unrelated dogs homozygous for von Willebrand's disease and with the severe clinical expression of the disease. when these plasmas were gel-filtered in a buffer at physiologic ionic strength, the factor VI...

journal_title：British journal of haematology

authors： Benson RE,Johnson GS,Dodds WJ

更新日期：1981-12-01 00:00:00

abstract：:Sickle cell disease (SCD) in Saudi patients from the Eastern Province is associated with the Arab-Indian (AI) HBB (β-globin gene) haplotype. The phenotype of AI SCD in children was described as benign and was attributed to their high fetal haemoglobin (HbF). We conducted a hospital-based study to assess the pattern of...

journal_title：British journal of haematology

authors： Alsultan A,Alabdulaali MK,Griffin PJ,Alsuliman AM,Ghabbour HA,Sebastiani P,Albuali WH,Al-Ali AK,Chui DH,Steinberg MH

更新日期：2014-02-01 00:00:00

abstract：:A new gravity filtration technique for the study of sickle cell deformability has shown a significant loss of filterability of oxygenated erythrocytes on day 2 of painful crisis in eight patients with homozygous sickle cell anaemia; there was no increase in irreversibly sickled cell (ISC) count. Serial study (mean of ...

journal_title：British journal of haematology

authors： Lucas GS,Caldwell NM,Stuart J

更新日期：1985-02-01 00:00:00

abstract：:Nine patients with Fanconi anaemia (FA) were conditioned for HLA-identical sibling bone marrow transplant (BMT) with reduced dose of cyclophosphamide (Cy) without radiation or antithymocyte globulin (ATG). The total dose of Cy was 140 mg/kg (n = 2) or 120 mg/kg (n = 7). The median patient age was 8 years (range 4-19)....

journal_title：British journal of haematology

authors： Flowers ME,Zanis J,Pasquini R,Deeg HJ,Ribeiro R,Longton G,Medeiros CR,Doney K,Sanders J,Bryant J

更新日期：1996-03-01 00:00:00

abstract：:Total knee replacement (TKR) is a safe treatment for alleviating pain and restoring physical function in end-stage arthropathy of the knee. First reports of TKR in haemophiliacs date back to the mid-1970s, however detailed information on long-term outcome is scarce. This study evaluated factors influencing the outcome...

journal_title：British journal of haematology

authors： Solimeno LP,Mancuso ME,Pasta G,Santagostino E,Perfetto S,Mannucci PM

更新日期：2009-04-01 00:00:00

abstract：:Dendritic cells (DC) are attractive candidates for use in vaccine-based immunotherapy. We have analysed the functional capability of DC generated in vitro from blood CD14(+) cells of chronic lymphocytic leukaemia (CLL) patients and healthy donors by culturing for 10 d with granulocyte-macrophage colony-stimulating fac...

journal_title：British journal of haematology

authors： Rezvany MR,Jeddi-Tehrani M,Biberfeld P,Söderlund J,Mellstedt H,Osterborg A,Rabbani H

更新日期：2001-11-01 00:00:00

abstract：:It has been proposed that when ABO unmatched platelets are transfused circulating immune complexes (CIC) may be formed between the patient's soluble ABH antigens and the transfused antibodies. Platelets might then be destroyed by bystander mechanisms or by the binding of CIC to the Fc receptor or to C3 binding membran...

journal_title：British journal of haematology

authors： Heal JM,Masel D,Rowe JM,Blumberg N

更新日期：1993-11-01 00:00:00

abstract：:In a prospective phase II trial, pentostatin combined with cyclophosphamide and rituximab (PCR) induced strong responses and was well-tolerated in previously untreated patients with advanced-stage, indolent non-Hodgkin lymphoma (iNHL). After a median patient follow-up of more than 108 months, we performed an intent-to...

journal_title：British journal of haematology

authors： Khashab T,Hagemeister F,Romaguera JE,Fanale MA,Pro B,McLaughlin P,Rodriguez MA,Neelapu SS,Fayad L,Younes A,Feng L,Vega F,Kwak LW,Samaniego F

更新日期：2019-05-01 00:00:00

abstract：:Cooley's original description of beta-thalassaemia major included marked bone deformities as a characteristic feature. These were thought to be due to expansion of haemopoiesis attempting to compensate for the congenital anaemia. Regular blood transfusions from infancy prevents these skeletal problems. Nevertheless, s...

journal_title：British journal of haematology

authors： Jensen CE,Tuck SM,Agnew JE,Koneru S,Morris RW,Yardumian A,Prescott E,Hoffbrand AV,Wonke B

更新日期：1998-12-01 00:00:00

abstract：:Allogeneic haematopoietic stem cell transplantation (HSCT) after a reduced-intensity conditioning (RIC) regimen with fludarabine, melphalan and alemtuzmab is an effective therapy for haematological malignancies. Alemtuzumab, a monoclonal antibody against CD52, a glycosylphosphatidylinositol-anchor-bound surface protei...

journal_title：British journal of haematology

authors： Woelfinger P,Epp K,Schaefer L,Kriege D,Theobald M,Bopp T,Wagner-Drouet EM

更新日期：2020-10-01 00:00:00

abstract：:Four children with acute lymphoblastic leukaemia (ALL) who relapsed after allogeneic bone marrow transplantation (BMT) were treated with donor lymphocyte infusion (DLI) without prior conditioning. Three patients had previously received a non-T-cell-depleted matched sibling BMT and the fourth had a T-cell-depleted matc...

journal_title：British journal of haematology

authors： Atra A,Millar B,Shepherd V,Shankar A,Wilson K,Treleaven J,Pritchard-Jones K,Meller ST,Pinkerton CR

更新日期：1997-04-01 00:00:00

abstract：:Mantle cell lymphoma (MCL) is an incurable mature B cell proliferation, combining the unfavourable clinical features of aggressive and indolent lymphomas. The blastic variant of MCL has an even worse prognosis and new treatment options are clearly needed. We analysed the effects of BL22, an immunotoxin composed of the...

journal_title：British journal of haematology

authors： Bogner C,Dechow T,Ringshausen I,Wagner M,Oelsner M,Lutzny G,Licht T,Peschel C,Pastan I,Kreitman RJ,Decker T

更新日期：2010-01-01 00:00:00

abstract：:Peripheral blood mononuclear cells (PBMNC) from 23 healthy subjects and 39 patients with B-cell chronic leukaemia (B-CLL) were assayed for ribonuclease H activity using as substrate the filter-immobilized synthetic homopolymer hybrid 3H-poly(rA):poly(dT). In 69% of the leukaemia patients examined enzyme activities wer...

journal_title：British journal of haematology

authors： Papaphilis AD,Kamper EF,Kattamis C,Pangalis GA

更新日期：1988-11-01 00:00:00

abstract：:To examine any role of macrophage colony-stimulating factor (M-CSF), in the immune responses in Kawasaki disease (KD), we serially assayed M-CSF and several related cytokines using ELISA. In 10 paediatric patients with KD the level of M-CSF was significantly higher in the acute phase than in the convalescent phase (14...

journal_title：British journal of haematology

authors： Igarashi H,Hatake K,Tomizuka H,Yamada M,Gunji Y,Momoi MY

更新日期：1999-06-01 00:00:00

abstract：:In order to evaluate the feasibility of first trimester prenatal diagnosis of beta-thalassaemia by restriction fragment length polymorphism (RFLP) in Campania, one of the most affected regions in Southern Italy, DNA polymorphism analysis was performed on 40 unrelated patients, affected with homozygous beta-thalassaemi...

journal_title：British journal of haematology

authors： Carestia C,Pagano L,Fioretti G,Mastrobuoni A

更新日期：1987-10-01 00:00:00

abstract：:We describe and compare five assay systems for Protein C (PC) in human plasma; a functional assay for PC activity, Laurell electroimmunoassay with EDTA or calcium (EDTA-Laurell or Ca-Laurell), radioimmunoassay (RIA) and immunoradiometric assay (IRMA). The lower limit of sensitivity of PC in normal reference plasma was...

journal_title：British journal of haematology

authors： Mikami S,Tuddenham EG

更新日期：1986-01-01 00:00:00

abstract：:Primary systemic amyloidosis (AL) is a plasma cell disorder characterized by deposition of monoclonal light chains in different organ systems. Although multiple and complex numerical chromosomal abnormalities have been described in patients with multiple myeloma, it is currently unknown whether such changes occur in s...

journal_title：British journal of haematology

authors： Fonseca R,Ahmann GJ,Jalal SM,Dewald GW,Larson DR,Therneau TM,Gertz MA,Kyle RA,Greipp PR

更新日期：1998-12-01 00:00:00

abstract：:The in vitro haemostatic functions of fresh whole blood (FWB) are well preserved after cold storage. This study aimed to determine whether platelets derived from FWB and stored whole blood (SWB) contribute to clot formation in tissue injury after transfusion into coagulopathic rats with polytrauma/haemorrhage (T/H). T...

journal_title：British journal of haematology

authors： Wu X,Darlington DN,Montgomery RK,Liu B,Keesee JD,Scherer MR,Benov A,Chen J,Cap AP

更新日期：2017-12-01 00:00:00