摘要:

NADH‐methaemoglobin reductase deficiency has been found in 4 Cuban families; 3 subjects carried the mild form of the deficiency while in 2 sibs of the fourth family the deficiency was associated with neurological involvement. The parents in this family were consanguinous and the sibs were shown to be homozygous for a new fast electrophoretic variant. It was named Diaphorase Santiago de Cub

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02472.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

A metabolic study with radioactively labelled C3 has been performed in a patient with angioimmunoblastic lymphadenopathy (AILD). The labelled protein was metabolized at a faster rate in the patient than in normal individuals. This indicates that the humoral immune system may be of pathogenetic significance in the AILD syndrome.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02473.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Using ferritin as surface marker, the localization of the surface immunoglobulin (Ig) was studied on peripheral lymphocytes from normal human individuals and patients with macroglobulinaemia Waldenström by scanning immunoelectron microscopy. Normal IgG‐, IgM‐lymphocytes and pathological IgM‐lymphocytes were then compared with regard to their topographical differences.In all cells examined, IgG‐ and IgM‐conjugated ferritin particles were detected all over the cell surface, but the distribution of the former on the normal IgG‐lymphocytes was slightly more diffuse than that of the latter on the normal and pathological IgM‐lymphocytes. Furthermore, in the pathological IgM‐lymphocytes, the clustered IgM‐conjugated ferritin particles were found in great number on the microvilli.Normal IgG‐lymphocytes were almost always characterized by short rod‐like microvilli standing densely and vertically on the cell surface. Some of normal IgM‐lymphocytes had a similar appearance to those of normal IgG‐lymphocytes (type A) but others (type B) had tilted rod‐like microvilli or wide plate‐like processes on their surface.As for IgM‐lymphocytes of macroglobulinaemia, most lymphocytes had tilted rod‐like microvilli and wide plate‐like processes similar to type B, whereas a minor population of the pathological lymphocytes carried long, thin rod‐like microvi

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02474.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

A case of acute myelogenic leukaemia developing after 7 months treatment with alkylating agents for multiple myeloma is reported. The patient was also given irradiation for an expansive process in the thoracic column. By immunofluorescence staining of the bone marrow cells there was no evidence for production of pathological globulins in the blast cells, while the plasma cells contained IgA, kappa.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02475.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

3 children who fulfilled the criteria for the diagnosis of paroxysmal cold haemo‐globinuria of the classical Donath‐Landsteiner type are described. The benign clinical course of this illness is confirmed. A serological study of two of the Donath‐Landsteiner antibodies revealed the anti‐P specificity in one, but not in anothe

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02476.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

A case of preleukaemia with marked reticulocytosis and a slightly reduced red cell51Cr survival is presented. An in vitro ‘reticulocyte survival’ test suggested that the apparent reticulocytosis was due to delayed maturation of reticulocytes. The globin synthesis ratio suggested an abnormal ribonucleic acid.At autopsy, in addition to the findings of acute leukaemia, mesenteric lipodystrophy was discovered. This abnormality has been seen in patients with malignant diseases, especially lymphomas, but heretofore has not been reported in acute leukae

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02477.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

A clinical and laboratory investigation of a kindred in which haemophilia A and autosomal recessive von Willebrand's disease (VWD) were concomitantly present is described. 3 male patients were shown to be hemizygotes for moderate haemophilia A, one female appeared to be haemophilia A carrier and 3 males showed laboratory findings consistent with heterozygosity for autosomal recessive VWD. In one woman, the pedigree and laboratory findings suggest the possibility of double heterozygosity.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02478.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

The bone marrow cells of a patient with congenital dyserythropoietic anaemia, type II, were incubated with3H‐thymidine,3H‐uridine or3H‐leucine for 1 h and studied using the technique of electron microscope autoradiography. Several of the erythroblasts which either displayed the characteristic subsurface double membranes or showed various non‐specific abnormalities of the nuclear membrane were found to be actively engaged in DNA, RNA and protein synthesis. Both members of some pairs of erythroblasts which were joined together by a spindle bridge were found to be engaged in DNA synthesis, indicating that some spindle bridges persist for a period longer than the duration of the G1phase. A small proportion of mononucleate and binucleate late (non‐dividing) erythroblasts showed a marked depression or arrest of protein synthesis and some or all of such cells were presumably destined to be phago‐cytosed by the bone marrow

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02479.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Samples of peripheral blood from patients with β‐thalassaemia major which contained significant numbers of nucleated normoblasts were stained with acridine orange and analyzed with rapid‐flow cytofluorometry. The pyknotic normoblast‐nuclei gave less green ‘DNA’ fluorescence than the (diploid) leucocytes and constituted a separate, distinct subpopulation. Mean values of the fluorescence intensities and standard deviations as displayed by multichannel analyses gave a numerical value for normoblasts with regard to their maturation stages. These mean values correlated with the differential counts of ‘early and late’ normoblasts in the light microscope under rigidly standardized conditions. Rapid‐flow cytofluorometry thus provides an objective and quantitative way to monitor and define peripheral blood normoblast populations as a measure of the severity of ‘eryt

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02480.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Haemorrhagic tendency, including frequent epistaxis and easy bruising, has been observed in patients with β thalassaemia major. Disturbances in the coagulation system were also described in this condition, which probably resulted from liver damage associated with this disease. There was, however, no quantitative or qualitative correlation between the haemorrhagic manifestations on the one hand and the abnormalities in the clotting mechanism on the other.Platelet functions were studied in 15 patients with β thalassaemia major and in 5 with thalassaemia minor. In most of the thalassaemia major patients and in some with thalassaemia minor, diminished platelet aggregtion to ADP, collagen, ristocetin and cpinephrine was found. These anomalies could not be corrected by the resuspension of the thalassaemic platelets in normal plasm

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02481.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY