摘要:

Platelet serotonin (5‐HT) uptake and storage in the presence and absence of reserpine were studied simultaneously with platelet volume, density and dense bodies content (mecaprine test) in 33 patients affected with myeloproliferative disorders (MD): 12 chronic myeloid leukaemia (CML), 9 polycythaemia vera (PV), 6 essential thrombocythaemia (ET) and 9 agnogenic myeloid metaplasia (AMM). Observations were (1) a dramatic reduction of the initial velocity (Vi) uptake and of the granular pool of 5‐HT; (2) a slight reduction of the number of platelet dense bodies which, in many cases, were less fluorescent than in controls; (3) an increase of the percentage of light platelets while platelet volume was mostly normal; (4) a significant correlation between the number of dense bodies per platelet volume unit and either the percentage of light platelets (r = 0.76) or the size of the granular pool of 5‐HT (r = 0.81). These results support evidence of a quantitative and qualitative acquired storage pool syndrome in these patients. In addition, the Vi studies demonstrate that the serotonin uptake across the plasmatic membrane is abn

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01405.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

Changes in mean red cell count (RCC), mean cell volume (MCV) and plasma alkaline phosphatase activity (ALP) were observed in a group of 33 normal male university students at a 7‐week interval. Linear regression analysis shows a negative linear relationship between changes in RCC and MCV (r = ‐0.77, P<0.0005) while a positive correlation was found for changes both in ALP activity and in MCV (r = +0.37, P<0.025). Multiple regression analysis indicates that 70% of the variation in MCV could be accounted for by changes in RCC and plasma ALP activity (r = +0.84, P<0.00

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01406.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

In the early stages of blood collection for transfusion, whole blood is subjected to trauma from the anticoagulant. The blood – anticoagulant mixture does not attain a pH at which platelets can remain functional (approximately pH 6.0) until about 25% of the unit has been collected. We have examined platelet ultrastructure and in vitro aggregation responses to adenosine diphosphate (ADP) and epinephrine (EPN), to assess the platelet trauma of collection into citrate‐phosphate‐dextrose. For comparison blood was collected by 2 methods: one was conventional (CC), the other metered anticoagulant into the blood as it was collected (MC). Platelets from CC blood showed disruption of organelles and depletion of the dense bodies, whereas platelets from MC blood showed no ultrastructural damage. In addition, biphasic aggregation in response to ADP or EPN was seen in MC platelets but not in CC plat

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01407.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

An internationally standardized preparation and 10 commercial kits widely used to perform the activated partial thromboplastin time (APTT) were compared in 4 laboratories for the purpose of assessing their ability to detect mild deficiencies of factor VIII activity. The participating laboratories were asked to carry out with each APTT reagent quadruplicate readings of 3 coded lyophilized plasmas containing varying levels of factor VIII (109, 26 and 17 U/dl respectively). An analysis of variance of clotting times showed significant differences between reagents and laboratories. All the reagents detected the abnormality of the plasma containing 17 U/dl, whereas a number of failures were found when the plasma with 26 U/dl was tested. When analysis of variance was carried out on ratios of factor‐VIII deficient to normal plasma clotting times, the results showed less difference between laboratories and reagents. Clotting times of plasma with normal factor VIII level (109 U/dl) usually fell within the normal range indicated by manufacturers of the commercial reagent

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01408.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

A case of acquired dyserythropoiesis with inter‐erythroblastic connections is reported in a patient with chronic myeloid leukaemia, developing a terminal acute hepatic failure related to hepatocarcinoma. The erythroblastic series was abundant but only made of clusters grouping 10 to 20 closely adherent cells. The cellular membranes showed linear junctions or were interdigitated and the intercellular space was occupied with electron dense ferritin granules. This non specific aspect of dyserythropoiesis may be related to the hepatic carcinoma, which was probably induced by busulfan therap

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01409.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

The effect of ouabain on the osmotic resistance of red cells from 17 splenectomized patients with congenital or hereditary spherocytosis (HS), from 5 of their relatives suspected of having a subclinical form of the disease and from unsplenectomized and splenectomized normal controls was studied. In red cells from the HS patients and the splenectomized controls a small ouabain‐induced decrease in osmotic resistance was seen, whereas in the unsplenectomized controls no such change was observed. The osmotic resistance of red cells from the 5 relatives, on the other hand, was significantly decreased by ouabain. The usefulness of ouabain in unmasking HS in its subclinical form was further tested by studying monovalent cation influx into red cells. The active influx of86Rb+ into red cells from HS sufferers was significantly increased. A similar degree of ouabain‐induced inhibition of86Rb+ transport was seen in all groups studied. The passive influx of22Na+, which was also increased in HS red cells, was not affected by ouabain in any of the four gro

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01410.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

At the onset of disturbances of cardiac rhythm after a 7th injection of iron sorbitol to a patient with severe malabsorption syndrome an extremely low level of alpha‐tocopherol in serum – 0.04 mg per 100 ml serum – was observed. The clinical manifestations are interpreted as a possible consequence of the depletion of the vitamin, a natural scavenger of free radicals. The possible importance of alpha‐tocopherol in preventing the toxic effect of iron on the apparently very sensitive myocardium after parenteral iron to patients with severe malabsorption syndrome is di

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01411.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

The red cells of a patient heterozygous for β‐thalassaemia contained 19% fetal Hb. Study of his family suggested that the proband had inherited the Swiss type of hereditary persistence of fetal Hb (HPFH) from his mother who is not thalassaemic and possessed 1.37% of Hb and 11% F‐cells. Studies of globin synthesis showed a similar imbalance in the heterocellular HPFH‐β‐thalassaemia compound heterozygotes and in the heterozygous β‐thalassaemic members of the family. Age stratification of the red cells showed a slight enrichment in Hb F and a decreased Hb A2level in the older cell populations. Hb F production in the BFU‐E colonies of the proband was higher than that found in vivo and in other β‐thalassaemic heterozygotes in culture. Study of single erythroid burst colonies showed a marked heterogeneity in Hb F synthesis from one colony to another, while the pool of free α‐chains remained of similar magnitude. It is suggested that in the proband, the HPFH gene, which is in trans with respect to the β‐thal‐gene, increases the size of the F‐cell population and its activity is carried on at the e

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01412.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

A 6‐year‐old boy suffered from a severe lymphadenopathy, characterized histologically by a fulminant polyclonal immunoblast proliferation simulating malignant lymphoma with many immunoblasts resembling Reed‐Sternberg cells. He had no history of infectious mononucleosis but Epstein‐Barr virus (EBV) infection was evident from serological findings, and EBV‐associated nuclear antigen (EBNA) was demonstrated in a high percentage of lymphocytes of blood and lymph nodes. An adequate humoral response to EBV ruled out the possibility of an X‐linked recessive lymphoproliferative syndrome as the underlying cause of chronic EBV infection. A chromosomal defect in a subpopulation of lymphocytes was induced by interferon and might somehow be associated with a subtle immunodeficiency of our patient. After exacerbation of the disease chemotherapy was included in the treatment but the patient died 9 months after the onset of the disease. At autopsy the lymphoblastic cell proliferation had changed from that of immunoblasts to cells resembling the Burkitt's lymphoma cells. A change of the proliferating cell type was supported by means of cytochemical and immunological cell markers. The presence of EBV in these cells was demonstrated with the EBNA technique. It is concluded that our case may well support the hypothesis, that EBV may induce lymphoma‐like polyclonal immunoblast proliferation in immunodeficient individuals, and that occasionally a monoclonal proliferation of the Burkitt's lymphoma type

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01413.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

In a patient with refractory idiopathic sideroblastic anaemia without signs of leukaemia a clone with the karyotype 46, XX, 5q– showed markedly different frequencies in mitotic preparations obtained from bone marrow by different methods. This clone was absent or rare (0–15 %) in preparations made by a direct method and prevalent (55–95%) after culture for 22 h or 46 h in the presence or absence of methotrexate. In cells without the 5q– chromosome there were minor clones with trisomies 8, 14 or 19. These were somewhat more frequent in preparations made by the methotrexate than by the direct method.If these findings are confirmed in larger series of experiments, it will follow that results obtained for different abnormalities and in different disorders by different methods may not be comparable and that modifications of the present cytogenetic methodology for bone marrow may lead to new findings of practical and theoretical signi

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1981.tb01414.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY