Fechtner syndrome: report of a third family and literature review.

abstract：:Screening of the skeleton by plain radiography was compared to magnetic resonance imaging (MRI) of the lumbar spine in 41 patients with multiple myeloma. In the lumbar spine, myeloma lesions were detected in 15 patients with radiography and in 28 patients with MRI. Radiography of the lumbar spine was not positive in a...

journal_title：British journal of haematology

authors： Tertti R,Alanen A,Remes K

更新日期：1995-11-01 00:00:00

abstract：:Familial haemophagocytic lymphohistiocytosis (FHLH) is a genetic disorder caused by defective lymphocyte cytotoxicity, resulting in impaired lymphocyte homeostasis and macrophage infiltration of solid tissues and bone marrow, with extensive haemophagocytosis. It is invariably fatal unless treated by allogeneic haemato...

journal_title：British journal of haematology

authors： Almousa H,Ouachée-Chardin M,Picard C,Radford-Weiss I,Caillat-Zucman S,Cavazzana-Calvo M,Blanche S,de Saint Basile G,Le Deist F,Fischer A

更新日期：2005-08-01 00:00:00

abstract：:We have investigated the kinetics of colony formation by progenitor cells in chronic myeloid leukaemia (CML) using erythroid burst-forming units (BFU-E) as a model system. For this, we scored the numbers of subcolonies produced by individual BFU-E in cultures of normal marrow and blood cells and in cultures of CML blo...

journal_title：British journal of haematology

authors： Marley SB,Lewis JL,Goldman JM,Gordon MY

更新日期：1996-06-01 00:00:00

abstract：:Sickle cell disease (SCD) affects approximately 100,000 people in the US, 12,500 in the UK, and millions worldwide. SCD is typified by painful vaso-occlusive episodes, haemolytic anaemia and organ damage. A secondary complication is infection, which can be bacterial, fungal or viral. Universal newborn screening, routi...

journal_title：British journal of haematology

authors： Sobota A,Sabharwal V,Fonebi G,Steinberg M

更新日期：2015-09-01 00:00:00

abstract：:We have determined the causative mutation in 12 cases of glucose-6-phosphate dehydrogenase deficiency associated with chronic non-spherocytic haemolytic anaemia. In 11 of them the mutation we found had been previously reported in unrelated individuals. These mutations comprise seven different missense mutations and a ...

journal_title：British journal of haematology

authors： Vulliamy TJ,Kaeda JS,Ait-Chafa D,Mangerini R,Roper D,Barbot J,Mehta AB,Athanassiou-Metaxa M,Luzzatto L,Mason PJ

更新日期：1998-06-01 00:00:00

abstract：:Platelet aggregation is commonly used to investigate patients with possible dense granule storage pool deficiency (delta SPD), but recent studies have shown that this investigation is not specific or sensitive for this disorder. We describe a simple one-step technique to detect mepacrine loaded platelets by flow cytom...

journal_title：British journal of haematology

authors： Gordon N,Thom J,Cole C,Baker R

更新日期：1995-01-01 00:00:00

abstract：:Mastocytosis is a rare disease with varied presentation, myriad symptomatology and variable prognosis. Most patients present with cutaneous disease and mediator-related symptomatology with a small subset having systemic disease (systemic mastocytosis, SM). A subset of the latter develops synchronous or metachronous ha...

journal_title：British journal of haematology

authors： Radia DH,Green A,Oni C,Moonim M

更新日期：2020-03-01 00:00:00

abstract：:The regulation of granulocyte-colony stimulating factor (G-CSF) and interleukin-6 (IL-6) mRNA was studied in human adherent monocytes in response to the protein kinase C activator, oleolyl-acetylglycerol (OAG), the calcium-ionophore A23187 and the cyclic AMP elevating agents, dibutyryl c-AMP (DBcAMP), cholera toxin an...

journal_title：British journal of haematology

authors： Vellenga E,van der Vinne B,De Wolf JT,Halie MR

更新日期：1991-05-01 00:00:00

abstract：:Sixty patients with haematological malignancies received a myeloablative regimen of total body irradiation, cyclophosphamide and fludarabine followed by a T-cell-depleted peripheral blood stem cell transplant from a human leucocyte antigen identical sibling. To improve donor immune function, 1 x 10(7) CD3+ cells/kg we...

journal_title：British journal of haematology

authors： Montero A,Savani BN,Kurlander R,Read EJ,Leitman SF,Childs R,Solomon SR,Barrett AJ

更新日期：2005-09-01 00:00:00

abstract：:Two cases of Ph1-positive chronic granulocytic leukaemia with hitherto undescribed translocations are presented. In case 1 the deleted part of chromosome number 22q- was translocated to the short arm of the X chromosome, t(X;22)(p22;q11). Pronounced basophilia, trisomy 19 in the majority of metaphases, and a partial c...

journal_title：British journal of haematology

authors： Hossfeld DK,Köhler S

更新日期：1979-02-01 00:00:00

abstract：:Myelofibrosis is characterized by splenomegaly and debilitating constitutional symptoms that negatively impact patients' quality of life. ROBUST, a UK, open-label, phase II study, evaluated the safety and efficacy of ruxolitinib in patients with myelofibrosis (N = 48), including intermediate-1 risk patients. The prima...

journal_title：British journal of haematology

authors： Mead AJ,Milojkovic D,Knapper S,Garg M,Chacko J,Farquharson M,Yin J,Ali S,Clark RE,Andrews C,Dawson MK,Harrison C

更新日期：2015-07-01 00:00:00

abstract：:The surface markers of immunoglobulin secreting cells (ISC) in bone marrow and peripheral blood were analysed. Circulating ISC bear surface Ig and Ia-like antigens. However, these markers were not detectable on ISC in bone marrow. Fc and complement receptors were not present on circulating ISC. The areas of plaques co...

journal_title：British journal of haematology

authors： Gyotoku Y,Mori M,Nonaka Y,Nagata Y,Saito Y

更新日期：1985-04-01 00:00:00

abstract：:Diamond-Blackfan anaemia (DBA) is an inherited bone marrow failure syndrome (IBMFS) characterized by red cell aplasia. Mutations in ribosomal genes are found in more than 50% of cases. Elevated erythrocyte adenosine deaminase (eADA) was first noted in DBA in 1983. In this study we determined the value of eADA for the ...

journal_title：British journal of haematology

authors： Fargo JH,Kratz CP,Giri N,Savage SA,Wong C,Backer K,Alter BP,Glader B

更新日期：2013-02-01 00:00:00

abstract：:SAn ageing population and the continuing expansion of clinical indications for coumadin therapy have increased pressure on hospital anticoagulant clinics. One solution is patient self-testing (PST) of the international normalized ratio (INR) using capillary blood samples on point-of-care coagulation monitors at home. ...

journal_title：British journal of haematology

authors： Gardiner C,Williams K,Mackie IJ,Machin SJ,Cohen H

更新日期：2005-01-01 00:00:00

abstract：:In a retrospective study, we examined the association between cytomegalovirus (CMV) infection and non-neutropenic fever immediately following autologous peripheral blood stem cell transplantation for a variety of haematological malignancies and solid tumours. Sixty non-neutropenic febrile episodes (41 in CMV-seroposit...

journal_title：British journal of haematology

authors： Fassas AB,Bolaños-Meade J,Buddharaju LN,Rapoport A,Cottler-Fox M,Chen T,Lovchik JC,Cross A,Tricot G

更新日期：2001-01-01 00:00:00

abstract：:Canine cyclic haematopoiesis (CH) appears to be a multipotential stem cell defect, possibly due to an intrinsic marrow defect. The in vitro adherent marrow cells of the cyclic haematopoietic (CH) dog were cultured as underlayers beneath normal dog nonadherent marrow cells. The marrow granulocyte-committed colony formi...

journal_title：British journal of haematology

authors： Jones JB,Jolly JD

更新日期：1982-04-01 00:00:00

abstract：:To determine whether human red cells contain a proteolytic system capable of rapidly degrading unstable proteins, the fate of pulse-labelled puromycyl polypeptides was investigated. In erythroid bone marrow cells these unstable polypeptides were degraded to TCA-soluble fragments with a mean half-life of 4 . 5 min. How...

journal_title：British journal of haematology

authors： Vissers MC,Winterbourn CC,Carrell RW

更新日期：1983-03-01 00:00:00

abstract：:Salivary gland mucosa-associated lymphoid tissue (MALT) lymphomas (SGML) are rare, as are data concerning their behaviour. We analysed clinical features at presentation, particularly the association with Sjoegren syndrome (SS) and hepatitis C virus (HCV) infection, and outcome in 33 cases of SGML diagnosed between Mar...

journal_title：British journal of haematology

authors： Ambrosetti A,Zanotti R,Pattaro C,Lenzi L,Chilosi M,Caramaschi P,Arcaini L,Pasini F,Biasi D,Orlandi E,D'Adda M,Lucioni M,Pizzolo G

更新日期：2004-07-01 00:00:00

abstract：:The continued improvement in the prognosis of childhood acute myeloid leukaemia (AML) has been paralleled by the use of increasingly intensive therapy. This has led to attempts to develop risk-directed strategies in which the most intensive treatment is reserved for those at highest risk of relapse. Unfortunately, cur...

journal_title：British journal of haematology

authors： Goulden N,Virgo P,Grimwade D

更新日期：2006-08-01 00:00:00

abstract：:Panobinostat in combination with bortezomib and dexamethasone demonstrated a significant and clinically meaningful progression-free survival benefit compared with placebo, bortezomib and dexamethasone in the phase 3 PANORAMA 1 (Panobinostat Oral in Multiple Myeloma 1) trial. Despite this benefit, patients in the panob...

journal_title：British journal of haematology

authors： San-Miguel JF,Hungria VTM,Yoon SS,Beksac M,Dimopoulos MA,Elghandour A,Jedrzejczak WW,Guenther A,Na Nakorn T,Siritanaratkul N,Schlossman RL,Hou J,Moreau P,Lonial S,Lee JH,Einsele H,Salwender H,Sopala M,Redhu S,Paul S

更新日期：2017-10-01 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：:This review will focus on the strengths and limitations associated with the current standard of care for primary prevention of ischaemic strokes in children with sickle cell anaemia (SCA) - transcranial Doppler ultrasound (TCD) screening followed by regular blood transfusion therapy when TCD measurement is above a thr...

journal_title：British journal of haematology

authors： Jordan LC,Casella JF,DeBaun MR

更新日期：2012-04-01 00:00:00

abstract：:18F-fluoro-deoxyglucose (FDG) positron emission tomography (PET) might be a better tool than computerized tomography (CT) in predicting long-term treatment outcome in patients with relapsed chemosensitive lymphoma who are candidates for autologous stem cell transplantation (ASCT). We studied patients with recurrent or...

journal_title：British journal of haematology

authors： Schot B,van Imhoff G,Pruim J,Sluiter W,Vaalburg W,Vellenga E

更新日期：2003-10-01 00:00:00

abstract：:The myelodysplastic syndromes (MDS) display both haematological and biological heterogeneity with variable leukaemia potential. MicroRNAs play an important role in tumour suppression and the regulation of self-renewal and differentiation of haematopoietic progenitors. Using a microarray platform, we evaluated microRNA...

journal_title：British journal of haematology

authors： Sokol L,Caceres G,Volinia S,Alder H,Nuovo GJ,Liu CG,McGraw K,Clark JA,Sigua CA,Chen DT,Moscinski L,Croce CM,List AF

更新日期：2011-04-01 00:00:00

abstract：:Leucocyte alkaline phosphatase (LAP) is a marker of post-mitotic granulocytes and its activity is reduced or absent in chronic myelogenous leukaemia (CML) granulocytes as a consequence of LAP messenger RNA (mRNA) deficiency. We provide evidence that along the granulocytic maturation in normal marrow, the acquisition o...

journal_title：British journal of haematology

authors： Dotti G,Garattini E,Borleri G,Masuhara K,Spinelli O,Barbui T,Rambaldi A

更新日期：1999-04-01 00:00:00

abstract：:Aspergillus terreus, a less common pathogen, appears to be an emerging cause of infection at our institution, the Medical University Hospital of Innsbruck. Thus the epidemiology and outcome of A. terreus infections over the past 10 years was assessed. We analysed 67 cases of proven invasive aspergillosis (IA) accordin...

journal_title：British journal of haematology

authors： Lass-Flörl C,Griff K,Mayr A,Petzer A,Gastl G,Bonatti H,Freund M,Kropshofer G,Dierich MP,Nachbaur D

更新日期：2005-10-01 00:00:00

abstract：:The efficiency of stored platelet transfusion was evaluated in terms of clinical status in 141 thrombocytopenic patients. In a paired prospective study in which fresh platelets were used as controls, clinical efficiency was assessed on the basis of the ability to increase platelet count (recovery) and the time to the ...

journal_title：British journal of haematology

authors： Norol F,Kuentz M,Cordonnier C,Beaujean F,Haioun C,Vernant JP,Duédari N

更新日期：1994-01-01 00:00:00

abstract：:Relapse of childhood acute lymphoblastic leukaemia (ALL) involving the eye is a rare but challenging problem. Twenty cases occurred in patients treated on the Medical Research Council United Kingdom Acute Lymphoblastic Leukaemia XI and ALL97 trials between 1991 and 2001, representing 2.2% of ALL relapses. Seventeen oc...

journal_title：British journal of haematology

authors： Somervaille TC,Hann IM,Harrison G,Eden TO,Gibson BE,Hill FG,Mitchell C,Kinsey SE,Vora AJ,Lilleyman JS,MRC Childhood Leukaemia Working Party.

更新日期：2003-04-01 00:00:00

abstract：:A total of 622 consecutive patients with acute promyelocytic leukaemia (APL) treated within the Gruppo Italiano per le Malattie Ematologiche dell'Adulto (GIMEMA) group during 1989-97 have been reviewed to assess the clinical effectiveness of all-trans retinoic acid (ATRA) on the incidence of early haemorrhagic deaths ...

journal_title：British journal of haematology

authors： Di Bona E,Avvisati G,Castaman G,Luce Vegna M,De Sanctis V,Rodeghiero F,Mandelli F

更新日期：2000-03-01 00:00:00

abstract：:We have previously shown that polymorphonuclear leucocytes (PMN) harvested from children with cancer and exposed to chemotherapy exhibit defective bactericidal activities against both Gram-positive and Gram-negative microorganisms as well as accelerated apoptosis. In this study, PMN from children with cancer were eval...

journal_title：British journal of haematology

authors： Lejeune M,Cantinieaux B,Harag S,Ferster A,Devalck C,Sariban E

更新日期：1999-09-01 00:00:00