摘要:

A child suffering from G6PD deficiency developed a severe haemolytic crisis without an apparent trigger. The possible pathogenetic role of the ingestion of unripe peaches was studied biochemically in this anaemia. We show that an extract from the unripe peach exerts an oxidative challenge on normal as well as on asymptomatic G6PD‐deficient erythrocytes. This effect is analogous to that of the favism‐inducing agents. The effect of the extract on the patient's red blood cells was more pronounced than on other asymptomatic G6PD‐deficient erythrocytes, particularly during his haemolytic crisis. The chemical nature of the deleterious component was not identified. It is suggested that unripe peaches be added to the list of hazards for G6PD‐deficient subjects in combination with other

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00704.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

4 patients are described with rheumatoid arthritis, splenomegaly, neutropenia and an unusual proliferation of T cells in the blood and marrow. These patients are clinically similar to patients with classical Felty's syndrome but can be distinguished from them by staining blood and marrow mononuclear cells with a panel of monclonal anti‐T cell antibodies. The T cells from patients with T cell proliferations stain with UCHT1 (OKT3 equivalent) and UCHT4 (OKT8 equivalent but do not stain with a panel of OKTl‐like antibodies. In 7 patients with classical Felty's syndrome there was no increase of UCHT4 cells in the blood and the large majority of T cells stained with OKTl‐like antibodies. The marrows from the patients with T cell proliferations contain plentiful haemopoietic progenitor cells and it is probable that the T lymphocytes suppress their normal maturation. There was a poor response to splenectomy in 2 patients with T cell proliferations, and single cytotoxic drug therapy may be more appropriate when therapy is required. The literature is reviewed and it is suggested that the T cell proliferations may be secondary to the rheumatoid pr

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00705.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

A 50‐year‐old female, heterozygous for beta‐thalassaemia was found to have a iytic lesion surrounded by osteosclerotic tissue in the 1st lumbar vertebra. Aspiration of the lesion showed 100% atypical plasma cells. The bone marrow contained 17% myeloma cells. Despite normal electrophoresis and immunoelectrophoresis of serum and urine, ‘rouleaux’ formation was pronounced. Treatment of the serum sample with 2‐mercaptoethanol and heat (56°C) disclosed an uncommon pyroglobulin. Analysis of the ammonium sulphate precipitate of the serum by sodium‐dodecyl‐sulphate polyacrylamide gel electrophoresis revealed a 43 kD component with higher anodic mobility than normal gamma chains. Ultrafiltration column chromatography of the serum revealed a narrow spike of approximately 4 S that contained gamma heavy chain antigenic determinants in addition to

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00706.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

16 young men in long‐term remission after standard treatment for the early stages of Hodgkin's disease were examined for testicular function 48 to 125 months after termination of therapy. The patients had received mantle field irradiation, plus either irradiation of infradiaphragmatic lymph nodes (7 patients) or 6 cycles of MOPP (9 patients). 5 patients had almost normal semen quality judged by seminal fluid analysis. 11 patients had reduced semen quality (8 severely reduced) and often elevated FSH values. Semen samples of poor quality were significantly more common in patients treated with MOPP (7/9) than in those treated with infradiaphragmatic irradiation (1/7). One patient showed signs of regeneration of spermatogenesis. None had experienced reduction in libido and sexual performance after therapy; correspondingly, none had below normal testosterone values or elevated LH values. Patients should be informed about the risk of infertility before treatment. If in a given case several treatment options with equal prospective antineoplastic effect are weighed against one another, the patient's possible desire to retain fertility should favour the use of radiotherapy as opposed to chemotherapy, especially including alkylating agents

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00707.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

We describe 2 cases of T cell leukaemia. Both patients react with OKT 4 monoclonal antibody, while their clinical course, surface markers and in vitro immunological reactivity present opposite behaviour. The cells of 1 patient react with all pan T cell markers (OKT 3, OKT 11, Leu 1), from E and Ea rosettes and respond well to mitogens. This patient does not present liver, spleen or lymph node enlargement; white cell count is stable and no other haematological alterations are present. The cells from the 2 patient are unreactive with OKT 3 and are unable to form Ea rosettes. The response to mitogens is almost abolished. The disease has an aggressive clinical course with progressively increasing blood leucocyte count and infiltration of liver and spleen. These data suggest that the study of antigenic and functional properties of T CLL may be useful to better define the biologic characteristics and the prognostic criteria of the disease.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00708.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

Lymphoprep®‐isolated mononuclear leucocytes (L‐MNs) from peripheral blood of healthy donors were mixed with yeast cells and filmed by time‐lapse microcinemato‐graphy in glass chambers at +37°C. The concentration of L‐MNs and yeast cells was lowered so as to make directional L‐MN movement (chemotaxis) a necessary conditon of phagocytosis. 54 cases of phagocytosis were filmed. Only yeast cells lying immobile on the glass surface were engulfed. In addition, 11 collisions between free‐floating yeast cells and glass‐adherent L‐MNs were noted. None of these collisions led to immediate engulfment. It is suggested that the process of phagocytosis requires prolonged leucocyte‐prey contact, in the order of magnitu

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00709.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

The basic ferritin content of red cells was measured in patients with untreated megaloblastic anaemia. The red cell ferritin of 10 patients with anaemia and vitamin B12deficiency (mean 579, range 68–2616 attogram (ag)/cell); and of 8 patients with folate deficiency (mean 792, range 141–2373 ag/cell) were significantly elevated (P<0.001) compared with normal subjects (mean 10.7, range 4–47 ag/cell) and showed a significant correlation with pre‐treatment levels of plasma ferritin and less so with percent transferrin saturation. Following vitamin replacement elevated red cell ferritin levels decreased during the period of reticulocytosis and was normal in 9 patients evaluated after 6 months.The magnitude of increase in red cell basic ferritin levels observed in untreated megaloblastic anaemia is comparable to that of subjects with idiopathic haemochro‐matosis and suggests that interpretation of this index for iron overload should take into consideration concomitant body folate or vitamin B

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00710.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

Spectrin is a major constituent of the erythrocyte membranoskeleton. The occurrence of spectrin during normal and malignant erythropoiesis was investigated by immuno‐fluorescence using a monospecific rabbit anti‐human spectrin antiserum. The expression of spectrin was correlated to the presence of glycophorin A, which is an early and specific marker for erythroid cells. The expression of spectrin during normal erythroid differentiation coincided with that of glycophorin A. Both markers were already present in the proerythroblasts. Spectrin was also found in leukaemic cells from patients with acute erythroleukaemia and erythroid blast crisis of chronic myelogenous leukaemia. In a large panel of human haematopoietic cell lines only those with erythroid phenotype (K 562 and HEL) stained positively for spectrin. It is concluded that spectrin appears early in the erythroid maturation. It is expressed both in normal and malignant erythroid precursors. Spectrin can be used as a marker for erythroid blasts in the diagnosis of erythroleukaemi

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00711.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

Natural killer (NK) cell activity of unseparated peripheral blood mononuclear cells from 30 untreated patients with Hodgkin's disease and 22 age‐ and sex‐matched normal controls was evaluated using the classical K 562 cells as targets. A significant defect was demonstrated in the patients with stage I‐II and seemed to be more profound in patients with advanced disease (stage III‐IV) and in those with B symptoms. The differences between subgroups of patients, however, were not statistically significant, mostly because of the wide dispersion of individual data. Pre‐incubation of effector cells with alpha A leucocyte recombinant interferon led to a clear increase in NK cell activity in 4 of 6 patients tested, showing that depressed NK activity in Hodgkin's disease is still susceptible to the enhancing effect of interferon, at least in some

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00712.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY

摘要:

A series of 57 patients with chronic lymphocytic leukaemia was typed for HLA‐A, B and DR antigens and compared with a control population of equal size. There was an increased frequency of HLA‐A1 and B8 in the patient group, although it was not statistically significant after allowance for the number of specificities tested. A subgroup of patients lacking both Al and B8 differed from the others in sex ratio, degree of lymphocytosis and incidence of hypogammaglobulinaemia at presentation. This subgroup also had less need for treatment within 6 months of presentation. While these findings must be considered preliminary, it is possible that tissue typing may help to identify patients with a relatively benign form of the disea

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1984.tb00713.x

出版商:Blackwell Publishing Ltd    

年代:1984

数据来源: WILEY