摘要:

In 5 siblings of a family comprising 7 siblings and 2 half sibs, chronic lymphocytic leukaemia was diagnosed. Since the occurrence of such families strongly suggest that a familial factor may play a role in the aetiology of chronic lymphocytic leukaemia, parameters of the immune system and several genetic markers were determined. In addition electron microscopy and chromosome studies of the lymphocytes were performed. However, so far no clue about the nature of such a familial factor could have been detected.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00102.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

A presentation is made of a patient with myelofibrosis and chronic haemolytic anaemia. Known causes of haemolysis in combination with myelofibrosis were excluded. A hereditary red cell glucose‐6‐phosphate dehydrogenase (G‐6‐PD) deficiency was found. The case history is reported, along with the results of haematological investigations in the affected members of the family. Emphasis is laid on the opinion that not only red cell sequestration in the spleen and a PNH‐like syndrome must be taken into account, but also G‐6‐PD deficiency, particularly when haemolytic episodes occur in combination with bacterial infections in patients with

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00103.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

PAS‐positive myeloblasts, which frequently occur in the blastic crisis of CML, may be due to disturbed myeloblast differentiation. As there is evidence suggesting that evolution of karyotypes containing excess chromosomes, in particular C members, is associated with delayed or inhibited CML granulocyte precursor differentiation, the present study concerns the relation between PAS‐positive CML myeloblasts and the cytogenetic characters of the granulocyte precursors. In 20 venous blood samples from 11 patients with Ph1‐positive CML the frequencies of PAS‐positive myeloblasts were determined. Karyotype analysis was performed after in vitro culture of the samples. It was shown that the frequencies of circulating PAS‐positive myeloblasts were positively correlated (1) to the relative size of the myeloblastic compartment, (2) to the frequencies of metaphases containing additional chromosomes, and (3) in particular to those showing excess C chromosomes. These observations may suggest that premature development of PAS positivity like the accumulation in blastic crisis of poorly differentiated cells reflects a defective granulocyte precursor differentiation which is due to evolution of certain types of aneuplo

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00104.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

In vitro labelling of lymphocytes by RNA precursors is a useful tool to study the characteristics of normal and leukaemic cell populations. Basic conditions have been studied for in vitro labelling and indicated a desired level of cell concentration (2 × 104to 2 × 105) and temperature (37° C).Normal leukocytes showed a fairly constant behaviour with respect to total uptake of radioactivity, incorporation into intracellular fractions and the ratio of3H‐cytidine to3H‐uridine incorporation (average ratio 10 ± 1). In contrast, the blood lymphocytes of patients with chronic lymphocytic leukaemia (CLL) exhibited a great variation in the parameters studied, ranging from below normal to several times normal values. This broad spectrum of in vitro characteristics has to be taken into consideration when populations of CLL lymphocytes are compared to normal lymphocytes and to those of other lymphatic di

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00105.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

A report is given on a case of scleroderma with a fulminant, autoimmune haemolytic anaemia with positive Ham's and Crosby's tests as the first symptom. The scleroderma was localized to the skin. The haemolytic anaemia was treated effectively with corticosteroids.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00106.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

The cytogenetic effect of melphalan was studied in bone marrow cells of Wistar rats in vivo. Melphalan produces structural aberrations of the chromatid and chromosome type, whereas no numerical aberrations were seen. The proportion of abnormal metaphases varied with the period that had elapsed since the administration of melphalan. Repeated large doses of melphalan were not able to produce permanent damage to the chromosome complement.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00107.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

The haematological findings in 113 cases of Down's syndrome have been compared with those in 106 age and sex matched control subjects from the same residential institution. The average value for MCV was higher and the serum vitamin B12was lower in the Down's syndrome patients than in the controls. These parameters were not significantly correlated in either group and the patients with low vitamin B12levels were shown to absorb the vitamin normally. Plasma and erythrocyte folate levels were normal. The band form counts and serum uric acid concentrations were higher in the Down's syndrome than in the control patients. The bone marrow was examined in 11 of the patients with Down's syndrome and MCV ≥ 99, only one showed megaloblastic changes. The results in the small number of patients with chromosome translocations and mosaicism did not appear to differ from those with the common trisomy‐21 anom

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00108.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

Several coagulation parameters were examined in 82 patients with hepatic cirrhosis. An increase of F VIII levels was found in 72 % of the cases. This raise was not correlated with the gravity, nor with the etiology of the hepatic cirrhosis. Increased serum fibrin‐flbrinogen degradation products were found in 11 cases (13 %). Only 3 of them also had a hypofibrinogenaemia. It is concluded that the frequency and the intensity of clinically important disseminated intravascular coagulation or of primary fibrinolysis, both described in hepatic cirrhosis, are quite lo

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00109.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

A case of selective vitamin B12malabsorption is described in an 8‐year‐old Negro boy. The importance of considering vitamin B12as a possible cause of megaloblastic anaemia amongst malnourished children is stres

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00110.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

Effects of phenytoin on folate metabolism were studied in 94 subjects. Even healthy volunteers on diphenylhydantoin showed decreasing serum folates, but neurological disease other then epilepsy did not cause folate deficiency. In conjunction with earlier studies, this indicates that folate deficiency in epilepsy is caused by the drugs, rather than by the disease. Diphenylhydantoin as well as carbamazepine (Tegretolr̀) appear to be involved.No antimetabolite effect of diphenylhydantoin could be demonstrated in a microbiologic system, nor could any correlation be found between the serum concentrations of diphenylhydantoin and folates. Such a correlation would be expected in case of an antimetabolite action. Therefore, intestinal absorption was studied. No effect could be shown in man on the intestinal absorption of medicinal folates. The absorption of food folates, on the other hand, seemed to be inhibited. A cerobrospinal folate pump exists, which keeps the cerebrospinal folate concentration almost five times higher than that in the serum. This ratio was numerically but not significantly lower in the phenytoin‐treated patien

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1973.tb00111.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY