ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02487.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

A 14‐month‐old girl suffering from a heavy bleeding tendency, caused by a severe isolated congenital factor V deficiency is described. In this study 56 family members were examined. 10 of them had a factor V level ranging 26–60% of the normal – these were classified as heterozygotes. The case histories of the heterozygotes did not reveal a bleeding tendency. The inheritance of this factor V deficiency is autosomal recessive, with varying expressivity in the hetero

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02488.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

In a patient with end‐stage acquired aplastic anaemia, unsuccessful treatment with splenectomy, prednisone, pyridoxine and anabolic steroid was followed by administration of antithymocyte globulin (ATG). This therapy soon led to a gradual improvement and after 12 months, complete remission was obtained. The possible mechanism of action of ATG and the general principles of treatment are discusse

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02489.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Medullary granulocyte progenitor (CFUC) cultures were grown in vitro from samples taken from 6 patients with toxic granulocytopenia caused by either thiamphenicol, cephalothin or amidopyrine and who are now apparently cured. A decrease in the medullary concentration of CFUChas been observed and a calculated estimate shows that there was a decrease in their absolute number. A decrease in the number of CFUCper 105metamyelocytes suggests a possible compensation by mitotic amplification between the stem cell and the differentiated cells. Two successive cultures have shown that the course of such medullary cultures is variable. The existence of medullary anomalies before drug toxicity as well as the practical consequences of the contrast between the apparent cure and the decrease in CFUCare discussed.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02490.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Marrow cells from an untreated man with preleukaemia were found to have trisomy for the long arm of chromosome 1 which was translocated to the end of the long arm of number 11, i.e., 46, XY, ‐11, +t (1:11) (q11 or q12; q25). The same abnormality was found in metaphases from 8 individual granulocytic colonies. With development of acute myelomonocytic leukaemia, in addition to the basic chromosome abnormality, trisomy of chromosome 8 and an extra chromosome number 19 with partial deletion were found. Fibroblasts grown from marrow biopsy material showed a normal diploid complement, 46, X

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02491.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Ferrokinetic studies were carried out in 4 patients with Fanconi's anaemia (FA). Experimental data were analysed by means of a mathematical model of iron kinetics in order to obtain a quantitative assessment of effective and ineffective erythropoietic activity, mean red cell lifespan, and non‐erythroid iron turnover. The major pathogenetic mechanism of the anaemia appeared to be relative marrow failure, i.e. a reduction in the proliferative capacity of the erythroid marrow. The role of ineffective erythropoiesis was of minor importance. On the basis of the results obtained both the pathogenesis and the natural course of the disease are discusse

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02492.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

The survival of patients with acute non‐lymphoid leukaemias (ANLL) receiving chemotherapy (32 patients) or chemotherapy plus BCG (31 patients) has been compared in a randomized trial which started in Nov 1974. Fresh BCG (Institut Pasteur, Paris) was administered by Heaf gun in the interval of chemotherapy cycles. The overall survival was better in the chemo‐immunotherapy group (median duration of survival of 25 months compared to 16 months in the chemotherapy group). However, the first remission duration did not differ (median duration of the 15 and 12 months, respectively). Plateau survival curves were not obtained in either group.A second complete remission was obtained in 7 out of 12 patients with bone marrow relapses in the chemo‐immunotherapy group, and only in 2 out of 15 in the chemotherapy group (P<0.05). BCG seemed more efficient in patients older than 40 years; no difference was observed according t

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02493.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

We have previously shown that non‐proliferating human T‐ but not B‐lymphocytes contain demonstrable amounts of acid α‐naphthyl acetate esterase (ANAE). The usefulness of this histochemical marker for the diagnosis and classification of malignant lymphoid tumors was investigated by use of a panel of established normal and malignant human haematopoietic cell lines and fresh biopsy cells from malignant lymphomas and myelomas. The results showed that not only the T‐cell derived acute leukaemia lines, but also histiocytic lymphoma and myeloma lines and some of the lymphoma (Burkitt and lymphocytic) and non‐neoplastic lymphoblastoid cell lines with B‐cell surface markers expressed strong ANAE reactivity. Some but not all of the immunoglobulin producing myeloma and lymphocytic lymphoma biopsies were ANAE‐positive. Inhibition experiments with sodium fluoride and E‐600 demonstrated that although the T‐lymphocyte specific esterase is predominantly of ‘A’‐type, the malignant lines contain also non‐specific ‘B’ esterase and pseudocholinesterase. As the presence of the various esterases did not demonstrate any specific distribution pattern among he haematopoietic cell lines of different origin, we concluded that the ANAE marker is no longer T‐specific when malignant lymphoid cells are considered, and that the usefulness of this marker in routine di

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02494.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Hairy‐cell leukaemia (leukaemic reticuloendotheliosis) is a well‐defined clinical entity. Most of the recent reports are almost entirely concerned with the pathological and functional aspects of the disease. In the present retrospective study the clinical features and laboratory data of 12 patients were analyzed together with a series of 123 adequately clinically documented cases from the literature. The Hb level and the sex of the patient proved to be the only parameters having some prognostic value for the survival time after diagnosis.The effect of splenectomy was assessed in two comparable groups of 24 splenectomized and 51 non‐splenectomized patients. The operation seemed to be beneficial, but after 2 years the difference was not significant (.05

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02495.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Type I dyserythropoietic anaemia was diagnosed in an infant, who presented with respiratory distress and hepatosplenomegaly soon after birth. Anaemia became manifest during the neonatal period. The case clearly proves the congenital nature of the disease. Abnormalities of the myelopoietic series indicate that it might be a stem cell disease and the presence of skeletal anomalies of the hands suggests a genetic relationship to some cases of Fanconi and Diamond anaemia. No serum lipid or vitamin E deficiency was present as in type II congenital dyserythropoietic anaemia. Serial serum ferritin determinations incidated that iron stores are increased early in type I congenital dyserythropoietic anaemia despite no transfusion load.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02496.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY