摘要:

Clinical, biochemical and histopathological data of 33 patients with agnogenic myeloid metaplasia (AMM) were examined with reference to liver disease and dysfunction.Clinical manifestations of hepatic or portal system involvement included hepatomegaly, jaundice, hepatitis, gall stones, ascites and bleeding esophageal varices. The liver was smooth and firm; it became larger with progression of the disease, but to a lesser degree than the spleen. Mild indirect bilirubinaemia occurred in the course of the disease in about half of the patients. Hepatitis was diagnosed in 5 (15 %) patients and in 3 (10 %) cases it was the cause of death. Cholelithiasis was found in 4 (12 %) patients.Biochemical liver dysfunction occurred in 22 (66 %) patients. A progressive increase in serum alkaline phosphatase and a gradual decrease in serum albumin were observed in 4 patients, who were followed for 5 to 15 years.Hepatic myeloid metaplasia was found in all 20 patients examined histologically. The liver architecture was grossly distorted in 4 cases: in 2 this was due to impaction of the sinusoid with haemopoietic cells, and in the remaining 2 to necrosis and haemorrhage following viral hepatitis. Periportal fibrosis was observed in 6 (30 %) of the liver specimens.Ascites, portal hypertension, or both, were found in 7 (64 %) of the 11 autopsies and in 5 (22 %) of the remaining patients. Liver histology, examined in 11 patients, revealed periportal fibrosis in 5 cases and myeloid metaplasia only, in the remaining 5. Patients with ascites or portal hypertension exhibited more frequently increased levels of serum alkaline phosphatase and vitamin B12, and lower levels of plasma prothrombin and serum albumin.The pathogenesis of the hepatic and portal system involvement in agnogenic myeloid metaplasia (AMM) remains uncertain. Mechanical pressure by foci of myeloid metaplasia, chronic passive congestion, hepatitis or combinations of the above may lead to atrophy or necrosis of the parenchyma, followed by reparative fibrosis. Portal hypertension and ascites, a common complication of advanced AMM, could be due to the combined effect of an increased blood flow in the portal system, hypoalbuminaemia and sinusoidal obstruction.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02497.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

3 cases of Fletcher factor deficiency in a family not related to the 6 families already published (Hathaway et al 1965, Hattersley&Hayse 1970, Abildgaard&Harrison 1974) are studied. In the family described here, 3 of 4 siblings have a Fletcher factor level of less than 1 % and the fourth has a level of 46 %; the Fletcher factor level in the father is 48 % and in the mother 38 %. This suggests an autosomal recessive transmission.Clinically they do not present spontaneous bleedings and only one of the siblings required a unit of blood after an amygdalectomy. It is also of interest to emphasize that 3 of the siblings suffered from congenital multiple arthrogryposis and that 2 of them presented the arthrogryposis together with the Fletcher factor deficiency, a circumstance which could have been favored by the consanguinity of the parents. The fact that the family described here is white and of Mediterranean origin contradicts the idea that there exists a special predisposition among members of the black race for this disease.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02498.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Chromosome studies on bone marrow in a patient with chronic myeloid leukaemia (CML) revealed a mosaic picture with a dominant clone of 24, XY, t (9; 22) (q34; q11). This corresponded to a preponderance of minute blasts in smears of bone marrow aspirate. It is suggested that a haploidy event rather than progressive hypodiploid loss, is responsible for the genesis of the miniature blast cells.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02499.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

A female patient with lymphomatoid granulomatosis (LYG) involving lung, liver and spleen is described. Our case presented with signs and laboratory data indicating severe hepatic failure.It is not clear if this disorder represents a distinct pathological or clinical entity. In this report the differences between LYG and the two disease groups which it most resembles, Wegener's granulomatosis and malignant lymphoma are discussed.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02500.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

An unusual case of Philadelphia negative chronic myelocytic leukaemia and an extra chromosome 8 in all bone marrow cells is described. The abnormality was present at diagnosis of the disease and throughout the chronic phase which lasted for somewhat less than 2 years. The patient died soon after the blastic transformation with no other chromosomal abnormalities.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02501.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Colony forming units in agar (CFU‐C) were assayed in both bone marrow and peripheral blood of dogs during haemopoietic recovery after lethal total‐body irradiation (1200 R) and allogeneic transfusion of blood mononuclear cells (MNC) from histocompatible donors. MNC had been collected from the peripheral blood by continuous‐flow centrifugation leucapheresis and cryopreserved at ‐196°C until transfusion.Two groups of dogs were studied. Group 1 dogs (n = 12) were given between 0.39 and 2.76 times 109MNC per kg body wt. Group 2 dogs (n = 14) were transfused with a similar number of MNC, ranging from 0.51 to 1.87 times 109per kg body wt., but in addition underwent immuno‐suppressive therapy with methotrexate. In group 1 dogs, there was a rather good correlation between the number of CFU‐C in the regenerating bone marrow and the recovery of the peripheral blood granulocyte values. The regeneration of the CFU‐C population in the bone marrow of methotrexate‐treated dogs showed a somewhat more heterogeneous picture than in dogs of group 1 and in dogs that, in a previous study, were transfused with autologous MNC. The minimum time interval required for the reconstitution of peripheral blood CFU‐C to normal levels was 2–4 weeks but usually

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02502.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Treatment of adult AKR mice with tissue‐specific mitotic inhibitor mol. wt. 1,000–20,000 extracted from the AKR thymus significantly delayed the onset of spontaneous thymus leukaemia, whereas extracts of other organs were without effect. A slight prolongation of survival time was found with spleen extract when administered to BALB/c mice infected with Rauscher virus, which causes leukaemia starting in the spleen. Thymus extracts of MW 100,000–300,000 caused myocardial degeneration in some leukaemic and nonleukaemic

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02503.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

The colony and cluster forming capacity of bone marrow and mononuclear leucocytes and colony:cluster ratio (CCIR) was studied in 23 patients with resistant anaemia and hypercellular bone marrow. Bone marrow controls included 28 patients with iron deficiency, haemolytic, megaloblastic and secondary anaemia as well as 15 haematologically normal subjects and 13 healthy volunteers. Colony formation by blood leucocytes was studied in aregenerative anaemias and in 13 healthy volun‐Colonies and clusters were grown in soft agar cultures in vitro from all control subjects. In normal volunteers the marrow grew between 24 and 201 colonies and 37 to 212 clusters/105cells with the CCIR of 0.48–2.50. Blood leucocytes produced 6–184 colonies and 6–88 clusters/106mononuclear cells with CCIR 0.43–2.64. Colony formation and CCIR were largely normal in haematologically intact subjects and patients with anaemia of known etiology.Patients with aregenerative anaemia showed abnormal colony formation by bone marrow and blood cells more often (P<0.01) than control groups. Only 7 of 23 patients had colony formation within the normal range. Blood leucocytes in 12 out of 18 aregenerative anaemias failed to grow colonies in vitro. The marrow CCIR was below the normal range in all aregenerative anaemias with defective and in 3 with normal colony formation and was significantly lower than in the controls (P<0.01). Of 19 aregenerative anaemias with defect colony formation 11 died, 7 with acute leukaemia.The in vitro colony formation appears useful for subclassification of anaemic conditions and for identification of patients who may be at higher risk of developing

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02504.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

A 64‐year‐old male developed severe anaemia during procainamide therapy. At the same time granulocytopenia was found. Discontinuation of procainamide led to complete recovery. Severe anaemia due to the use of procainamide is not well known. The available data indicate a maturation block of the erythropoiesis as the possible cause of the anaemia, perhaps in combination with an autoimmune haemoly

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02505.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Expression of predicted and measured red cell mass (RCM) in terms of ml/kg body wt. lacks precision. The use of formulae for prediction of normal mean red cell mass derived from the blood volume prediction (BV) of Nadler et al (1962) has been examined. It is proposed that a more accurate determination of mean normal predicted red cell mass (MNRCM) is obtained by using:0.47 times 0.91 × BV = MNRCM for males0.43 times 0.91 × BV = MNRCM for femalesThe MNRCMs given by these formulae agree with those given by predictions based on lean body mass and surface area.Examination of the ‘95 % confidence limits’ of the ‘Nadler’ prediction indicates that males with measured RCMs greater than 25 % and females with measured RCMs greater than 30 % above their MNRCM may be regarded as having poly

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1978.tb02506.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY