摘要:

The clinical, haematological and biochemical findings in a person with δβ‐thalassaemia and Hb‐Lepore are described. The patient was a 24‐year‐old student who suffered from anaemia of intermediate seventy with late onset of the clinical manifestations, had minor bone and facial deformities, but had no necessity for regular transfusions. Haemoglobins A and A2were absent in this individual, and the Hb‐Lepore has been identified as Lepore‐Baltimore. Heterogeneity of γ chain of the Hb‐F follows the expected pattern. The study provides further evidence that neither β nor δ chains are synthesized incisto δβ‐tha

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01122.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

were treated with a standardized high dosage regime of an anabolic steroid (oxymetholone, Anasteron®). 53 patients were included and divided into two groups according to bone marrow cellularity. Furthermore the hypocellular group was subdivided in order to make comparison with earlier studies possible.In the hypocellular group, the frequency of remission was 56% and the 2‐year‐survival from the onset of symptoms was 75%. This is longer than in some earlier studies, perhaps because of possible differences in etiology andor because of the effect of systematic high dosage, long term androgen therapy. Patient selection was minimized and was not considered to be of major importance.Patients with hypercellular marrows, on the other hand, responded poorly to androgens. In this group 63% died of acute leukaemia, which confirms earlier suggestions that this form of aregenerative anaemia, frequently is of a prelejukaemic na

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01123.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Normal human non‐separated bone marrow cells were cultured in fibrin clots in diffusion chambers implanted intraperitoneally in mice, and harvested at different intervals by a previously described chamber centrifugation technique. This method demonstrates the presence of cell aggregates in the diffusion chambers. When the chambers are implanted in irradiated mice (450 R) and retransplantated into newly irradiated mice every seventh day, a continous increase in number of cells per granulocytopoietic aggregate is observed from day 8 to day 21. This is compatible with the view that the aggregates are colonies. The term ‘colony forming unit diffusion chamber’ (CFUD) is suggested to denote the ancestor(s) of the colonies. However, formal proof that one colony is derived from one cell is lacking.Preirradiation of mice with 450 R significantly increases the number of neutrophilic granulocytopoietic colonies at day 14, provided the chambers are retransplantated to newly irradiated mice at day 7, indicating that the neutrophilic colony forming unit or its progeny is involved as at least one of the targets of the stimulating effect of host irradiation. In contrast, no effect of host irradiation on the numbem of eosinophilic colonies was observed.Aggregates of megakaryocytic cells were present during the entire culture p

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01124.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Antisera were produced in rabbits against idiotypic determinants of the myeloma protein from 3 patients with IgG‐kmyeloma. The antisera did neither cross‐react nor react with normal immunoglobulin (Ig). Idiotypic Ig‐structures were demonstrated by indirect immunofluorescence (IFL) on 70–94 % of peripheral B‐lymphocytes in untreated patients. The total numbers of circulating B‐lymphocytes were increased with a simultaneous decrease of T‐lymphocytes. Only one of the patients had blood lymphocytes which stained for cytoplasmic Ig with idiotypic antiserum.The monoclonal lymphocyte population decreased gradually during treatment. Plasma cell counts in bone marrow, IgG concentration in serum and erythrocyte sedimentation rate decreased simultaneously, while the haemoglobin concentration increased. Very few monoclonal lymphocytes were present during remission. Later, the monoclonal lymphocyte population began to increase in 2 of the patients in parallel with clinical signs indicating relapse.It is concluded that presence of monoclonal B‐lymphocytes indicates dissemination of the disease and that they may belong to the malig

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01125.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

The correlations between the deoxyuridine‐suppressed value and some conventional haematological parameters (Hb concentration, red cell count, MCV, total white cell count, red cell folate level and serum vitamin B12level) have been investigated in 40 patients with megaloblastic haemopoiesis due to folate or vitamin B12deficiency. The only statistically significant correlations found were (a) an inverse correlation between the deoxyuridine‐suppressed value and the Hb level or red cell count in both the folate‐ and vitamin B12‐deficient groups and (b) a direct correlation between the deoxyuridine‐suppressed value and MCV in the vitamin B12‐deficient group. The results indicated that the deoxyuridine‐suppressed value was a better index of the magnitude of the disturbance in red cell production induced by folate or vitamin B12deficiency than either the red cell folate or serum vita

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01126.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

This paper reports the biological data found during an acquired bleeding disorder occurring in a 65‐year‐old woman affected with chronic lymphocytic leukaemia. They consists of haemostatic abnormalities which resemble an presently undescribed association of an acquired von Willebrand's syndrome with a thrombopathy.von Willebrand abnormalities were temporarily corrected by infusion of normal cryoprecipitate and reproduced in vitro by the incubation of normal platelets with the patient's IgA. The platelet defect was partially corrected after corticotherapy. The possible relationship of the associated defects is discus

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01127.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

Four patients are described in whom seven episodes of haemolytic anaemia associated with pregnancy were observed during a follow‐up period of 2 to 16 years. Normal haemoglobin levels were found both before and after pregnancy. Despite consistently negative antiglobulin tests, an autoimmune haemolytic mechanism is suggested by the rapid destruction of transfused blood, favourable response to corticosteroids and the development of transient haemolytic anaemia in three of the infants. In view of its unique clinical and laboratory features and relatively mild course, recognition of this condition as a distinct clinical entitity seems to be justifie

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01128.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

The streptokinase resistance test (SRT) has been studied in a group of patients with recent streptococcal infection and/or titres of antistreptolysin higher than 625 units. These patients generally had a higher SRT.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01129.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

The granulocytes of a patient with generalized pustular psoriasis (GPP) were found to have impaired ability to fix iodine after ingestion of yeast particles. Since hexose monophosphate shunt (HMS) activity was increased and the contents of 3 other lysmomal enzymes, β‐glucuronidase, N‐acetyl‐β‐glucosaminidase and lysoeyme were within normal range, the impaired iodination appeared to be due to a selective defect of myeloperoxidase (MPO) activity within the phagocytic cells. The deficient iodination was accompanied by a decreased intracellular killing of E. coli and C. albicans. Since hexose monophosphate shunt activity was enhanced and azide and cyanide inhibited the intracellular killing of E. coli only moderately, the patient's granulocytes may possess azide‐ and cyanide‐resistant, MPO‐independant microbicidal systems coupled to the oxidative metabolism. Assessment of granulocyte iodination and enzyme contents of the relatives of the patient revealed no hereditary transmission. Since GPF is characterized by the development of subcorneal pustules containing granulocytes, the MPO‐deficiency may be the cause of or enhance the developme

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01130.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY

摘要:

10 patients with hypothyroidism were examined before and 3 months after initiation of thyroxin treatment. All patients had high levels of plasma total cholesterol and total phospholipids, and 5 had an additional hypertriglyceridaemia. 1 patient had a prolonged primary bleeding time and all had a prolonged activated partial thromboplastin time and recalcification time in plasma. Platelet factor 3 activity in platelet rich plasma was reduced and total phospholipid and protein concentration in platelets were low. No significant correlation could be established between the observed lipid, protein and haemostatic parameters, and it is suggested that they represent independent phenomena. All abnormalities were corrected by thyroxin replacement.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1976.tb01131.x

出版商:Blackwell Publishing Ltd    

年代:1976

数据来源: WILEY