摘要:

SummaryThis is a study ofin vitroproduction of erythropoietic colonies from peripheral blood and bone marrow of normal subjects and patients with different polycythaemic conditions. Proliferative stimuli included: 1 Fetal calf serum (FCS) only. 2 FCS plus a source of erythropoietic‐stimulating activity (ESA). 3 FCS + ESA + erythropoietin (Ep). It was found that normal subjects and patients with secondary polycythaemia (SP) exhibited full colony growth only in the presence of both ESA and Ep, while patients with primary proliferative polycythaemia (PPP) showed colony production with FCS alone, further enhanced in the presence of ESA and Ep. A group of patients with idiopathic erythrocytosis (IE), namely with an increase of red cell mass not accompanied by other signs of myeloproliferative disorder, and without underlying cause, showed a heterogeneous response to ESA which in some patients was significantly greater than in normal subjects or in SP patients. It appears therefore that sensitivity of erythropoietic colony formation to Ep and ESA may be helpful in differentiating among various forms of polycythaemia; this study also establishes the heterogenicity of the IE grou

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01344.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

SummaryTwenty‐four patients have received bone marrow transplantation for severe aplastic anaemia at the Westminster Hospitals since 1974. Twelve patients are long term survivors. Infectious complications in association with graft rejection, graft versus host disease or prolonged neutropenia were the major cause of death. In the last 18 months the introduction of more effective conditioning regimes and Cyclosporin A as graft versus host disease prophylaxis has improved the survival rate to 85%. One patient has required regrafting for late graft failure without evidence of graft rejectio

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01345.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01346.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

SummaryIn von Willebrand's disease, chronic arthropathy is thought to be rare. We present four young patients, three girls and a boy, with severe von Willebrand's disease, who developed chronic progressive arthropathy due to joint haemorrhage, similar to that seen in haemophilia. Chronic joint disease is not as uncommon as is generally assumed.

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01347.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

SummaryOne hundred and thirteen haemarthroses involving the knees, ankles and elbows of 29 severe haemophiliacs presenting with at least two of the risk factors, pain, tenderness, loss of more than 50% of movement and a delay of more than 3 h in treatment were studied. Each was given either a 20% or 40% dose of factor VIII and progress was then reviewed by medical staff unaware of the initial dosage. There was no significant effect on the retransfusion rate nor on the time to complete resolution. However, the difference between the percentage of patients showing residual movement restriction was significantly in favour of the high dose at24, 36 and 48 h when all the bleeds were pooled and at 48 h for elbow bleeds.

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01348.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

SummaryPlasma was obtained from two patients with severe factor IX deficiency who had developed specific inhibitors of factor IX. Immunochemical characterization of the inhibitors by coagulation inhibitor neutralization assays and by immunoelectrophoretic methods demonstrated that both were IgG antibodies. One of the antibodies appeared to be monoclonal in origin with IgG subclass 4 heavy chains and lambda light chains. The other appeared to be oligoclonal and contained IgG subclass 1 and subclass 4 heavy chains and kappa and lambda light chains. One of the patients was treated with conventional, non‐activated factor IX concentrate and with activated factor IX concentrate (Feiba) for repeated bleeding episodes. Administration of Feiba resulted in a progressive shortening of the kaolin cephalin clotting time and was followed by a good clinical response. Infusion of non‐activated factor IX concentrate failed to induce clinical resolution of haemarthroses and had minimal effect on laboratory tests. The presence of circulating immune complexes could not be demonstrated in this pati

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01349.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

SummaryThis multicentre study compares in parallel nine commercial coagulometers and manual tilt‐tube methods for their capacity to distinguish two pairs of lyophilized plasmas with slightly different prothrombin times (PTs). The study was based on the rationale that the more precise a method is, the better it is able to discriminate between plasmas with similar PTs. Good or excellent discrimination, as judged by higher Studenttvalues, were achieved slightly more frequently by coagulometers than by manual methods, particularly when pairs of plasmas with smaller PT differences were teste

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01350.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

SummaryAntithrombin III (AT III) is the most potent physiologic inactivator of thrombin and other serine proteases in the blood clotting mechanism. Hereditary deficiency of this protein is associated with recurrent deep‐vein thrombosis that begins in late adolescence. Untreated, this disease may lead to early death from recurrent and massive pulmonary emboli. Attempts to identify groups of patients who are the most likely to develop thromboembolic disease because of an acquired deficiency of AT III have been frustrated by the lack of standardization of the assays and the inability to compare results of the different AT III assays. The functional assays and immunoelectrophoretic determinations do not measure the same component. In order to compare the ability of current AT III procedures to determine levels of AT III in various disease states, we used immunoelectrophoretic, chromogenic, and clottable assays to measure the AT III of patients with congenital AT III deficiency and of patients with possible acquired AT III deficienc

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01351.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

SummaryVIII procoagulant activity (VIII:C) was estimated by a two‐stage assay method, on 100 pre‐and post‐transfusion samples obtained from severe haemophiliacs receiving regular treatment with a variety of VIII concentrate preparations. Close and significant correlation (r= 0.97P<0.01) was obtained between freshly tested samples and samples stored for one month at −35°C. No significant difference between the two samples was demonstrated by Student's paired‐t‐test. Forty‐nine of the stored samples were also measured for VIII procoagulant antigen (VIII:C Ag) level. A similar close and significant correlation was found for this parameter (r= 0.9P<0.01), when compared with the VIII:C of the freshly tested samples. On average the VIII:C Ag value was 10% lower than the corresponding VIII:C value, although the difference was not statistical

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01352.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

SummaryThe Technicon H6000 haematology system combines haemoglobinometry with cell counting including differential counts of leucocytes. Evaluation confirmed it to be safe, precise and free from carry‐over of practical significance. Comparability with standard reference methods showed a difference with mean cell volume (MCV) and packed cell volume (PCV) but the haemoglobin, red cell count and mean haemoglobin concentration (MCH) were comparable. Total white cell counts and differential counts were comparable apart from eosinophils and basophils. Platelet counts were higher at both ends of the range. Throughput was confirmed at 90 samples per hour. The system was shown to be cost‐effective when taking into consideration staff costs, materials and maintenance but expensive with regard to materials al

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1983.tb01353.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY