摘要:

To evaluate the extent of denaturation of factor VIII‐coagulant activity (VIII:C) during production of factor VIII concentrates, the factor VIII‐coagulant antigen (VIII:CAg)/VIII:C ratio was measured in plasma, cryoprecipitate and cryosupernatant from fresh and stored blood. This ratio was close to unity for both cryoprecipitate and other concentrates, suggesting that VIII:CAg is lost concurrently with VIII:C during cryoprecipitation and further fractionation.Storage of blood (18 h, 22°C) before processing resulted in a 30% loss of VIII:C from the separated plasma; however, VIII:CAg was not affected. In cryoprecipitate prepared from this plasma, VIII:C and VIII:CAg both were 30% lower than when prepared from fresh plasma. In the corresponding cryosupernatant, however, more VIII:CAg but less VIII:C was present compared with fresh material.Gel chromatography revealed that the rise of VIII:CAg in cryosupernatant prepared from stored blood, was due to an increased amount of VIII:CAg of low molecular weight, not being associated with factor VIII‐related antigen. Such an increase in dissociated VIII:CAg was not detected in the plasma prior to cryoprecipitation.It is concluded that during storage of blood, molecular changes are induced in the factor VIII‐VWF complex, possibly by limited proteolysis, which make the complex more liable to dissociation during subsequent cryopreci

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00608.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

A platelet function study in a patient with megakaryoblastic leukaemia is reported. The abnormalities of the platelet function suggest a probable platelet membrane injury and a platelet release defect. The reduced platelet half‐life and the non changing splenohepatic ratio confirm the clinical and histological features of the systemic diseas

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00609.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

The in vitro release of B‐thromboglobulin (BTG) from platelets after stirring the platelet rich plasma (PRP) 5 min (37°C/1000rpm) was studied in a group of 13 healthy subjects before and after ‘in vivo’ aspirin ingestion, 1 g/d for 3 d. A significant reduction in the platelet BTG release (31%) was observed after aspirin ingestion in comparison to the pre‐aspirin values, suggesting that the BTG release by stirring is related to a platelet activation process.Small doses of collagen (0.625 μg/ml) and thrombin (0.1 U/ml) always released platelet BTG, whether or not there was a visible platelet aggregation, suggesting that platelet activation is not necessarily followed by visible platelet aggregation.A significant correlation was found between the BTG released by stimulation with collagen (2.5 μg/ml) and thrombin (0.2 U/ml) and the maximum velocity and intensity of the aggregation curve

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00610.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

The iron uptake in bone marrow and spleen was measured in 29 patients with myelofibrosis using52Fe and quantitative scanning. In 10 patients, no iron uptake in the marrow could be observed and active erythropoiesis was extramedullary only. In the bone marrow of patients with myelofibrosis, the iron uptake per nucleated red cell was less than that observed in conditions without myelofibrosis or extramedullary erythropoiesis. Increasing splenic iron uptake was likely to be associated with a decreasing bone marrow iron uptake and was related to the size of the spleen. The data suggest that in myelofibrosis, the spleen dominates iron uptake through intense erythropoiesis and a high splenic blood flow, thus restraining iron supply to the bone marrow.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00611.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

There was no difference in monocyte‐mediated cytotoxicity between monocytes from patients with multiple myeloma or malignant lymphomas and monocytes from control persons after in vitro culture for 5 d. Cytostatic and cytolytic ability of lymphokine‐activated monocytes cultured in medium with patient serum was significantly depressed compared to the ability of monocytes cultured with normal serum. A similar depression of cytostasis was found with non‐activated monocytes of both patient and control origin. Sera from myeloma and lymphoma patients impaired the monocyte‐mediated cytotoxicity

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00612.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

12 cases of hereditary myeloperoxidase (MPO) deficiency are reported. Histochemical stainings, lysosomal enzyme determinations, electron microscopic study of MPO and granulocytic function were performed. Family studies on 2 generations were carried out in 5 patients and histochemical stainings and biochemical lysosomal enzyme determinations were done. MPO deficiency was found to follow autosomal recessive inheritance and only rarely to have clinical effects.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00613.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

Cells from 13 cases of T‐prolymphocytic leukaemia (T‐PLL) were studied with a battery of immunological techniques in order to define their membrane phenotype. All cases were E‐rosette positive and were negative with OKT6, anti‐HLA‐DR, anti‐Ig and M‐rosettes; in 3, 20–30% of the cells had receptors for C3b. 7 cases had predominantly a ‘helper/inducer’ T‐subset phenotype, (OKT4+, OKT8‐) and 4 had a ‘suppressor/cytotoxic’ phenotype (OKT8+, OKT4‐). Cells in 2 cases coexpressed OKT4 and OKT8 in 48% and 95% of prolymphocytes and in another, both OKT4 and OKT8 were negative. Terminal transferase (TdT) was negative by IF in all the cases, but a low positive level was detected biochemically in one. Although T‐PLL appears to be heterogenous in respect of membrane phenotype, the observation of unexpected features in 8 of the cases raises the possibility that it may originate in a cell of intermediate maturation between late thymocytes and mature T‐lymphocytes. These features plus the clinical manifestation of the disease — typical morphology, splenomegaly, lymphadenopathy, skin lesions, high WBC and aggressive clinical course — help define T‐PLL a

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00614.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

2 patients with coexistent polycythaemia vera and chronic lymphocytic leukaemia are described. A 61‐year‐old man presented with an increased packed cell volume (PCV) and a leucoerythroblastic blood picture, which was reversible upon treatment, neutrophil leucocytosis, bone marrow lymphocyte infiltration and splenomegaly, and subsequently developed lymphomas, blood lymphocytosis and thrombocytosis. The second case was a 58‐year‐old female presenting with increased PCV and leucocyte alkaline phosphatase score. She later had neutrophil leucocytosis, thrombocytosis, lymphocytosis, lymphomas and splenomegaly. These cases, together with 6 cases published by others, suggest that an association exists between the two d

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00615.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

The relation of thrombokinetics to quantitative determinations of megakaryocytes (mgkc) in bone marrow sections was studied in 11 consecutive cases of untreated Ph1‐positive chronic granulocytic leukaemia (CGL). The results were compared with controls and with previously obtained data in polycythaemia vera (PV), primary thrombocythaemia (PT) and in idiopathic thrombocytopenic purpura (ITP).Platelet survival was significantly reduced in CGL. Platelet production was 5.8 x normal and the mgkc number and volume/μl bone marrow were significantly increased as compared to controls. The increase in mgkc volume was not in proportion to that of number due to a significant decrease of mgkc size.Platelet production was strongly related to mgkc number/mm2and to the mgkc volume/μl bone marrow. The platelet production rate in relation to a unit of mgkc volume/μl bone marrow was, however, greater in CGL than in controls, PV, PT and ITP. The chief reason for this is most probably the greater expansion of the total bone marrow mass in

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00616.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

The activity of 5‐aminolaevulinate (ALA) synthase, the first and rate‐limiting of haem synthesis, was markedly reduced (13 % of controls) in erythroblasts of a patient with acquired, primary sideroblastic anaemia (PASA). The reduced activity of ALA synthase could not be restored in vitro with 1 mmol/1 pyridoxal‐5‐phosphate (PLP). Treatment of the patient with pyridoxine for several months increased the ALA synthase activity from 13 % to 50% of controls in the absence and to 100% in the presence of PLP in the incubation medium. These studies suggest that both increased degradation of apo‐ALA synthase and decreased affinity of ALA synthase for PLP may be involved in pyridoxine‐resp

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1982.tb00617.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY