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We undertook a four year study of 128 thalassaemic patients who had undergone several transfusions, to determine the incidence of hepatitis B virus markers and the activities of transaminases in their sera each month. The results showed that the possibility of these patients contracting hepatitis B virus infection is still high, although on only one occasion was a transient antigenaemia found, indicating low viral replication. Furthermore, the probability of contact with hepatitis B virus increases with the number of transfusions and, therefore, with age. About 25% of these patients were positive for hepatitis B markers and 80% for other hepatitis markers including the case of cytomegalovirus hepatitis. [ABSTRACT FROM AUTHOR]

In 98 children with acute lymphoblastic leukaemia, aged 1 to 12 years, the prognostic significance of radiological bone involvement was studied. The mean duration of remission and of survival was much shorter in cases with multiple bone involvement (3 or more bones) than in those where bone involvement was absent. In those cases presenting with 1 or 2 bone lesions no statement of prognostic significance can be made at this stage. A radiological skeletal survey should be made in all children presenting with leukaemia to identify those (about 15%) having multiple bone lesions and therefore a poor prognosis, in order that they can be given more intensive therapy. [ABSTRACT FROM AUTHOR]

Liver function and the presence of HBsAg and anti-HBsAg were studied in 90 hypertransfused thalassaemic children. Increased serum transaminases were found in 62 patients, and persisted from more than 6 months in 45 cases. Liver biopsy in this latter group led to a diagnosis of 14 cases of chronic persistent hepatitis, 9 cases of aggressive hepatitis, and 3 cases of hepatic fibrosis. In Italy thalassaemic children undergoing hypertransfusion therapy frequently encounter SH virus infection, with a consequent hepatitis that is generally anicteric and unrecognized unless systematically sought. In a liver already stressed by the concomitant iron overload, hepatitis infection might thus play a key role in the evolution of cirrhosis which frequently affects thalassaemics. [ABSTRACT FROM AUTHOR]

The relationship between blood requirement and the mean level of maintained haemoglobin was examined in 392 patients with homozygous beta-thalassaemia. Pre- and post-transfusional haemoglobin levels and the amounts of blood transfused were measured during a 1-year period. No significant differences were noted in the blood requirements of patients (splenectomised or not) irrespective of the haemoglobin level. It may be supposed that if the mean haemoglobin level is high the haematopoietic activity is inhibited, and hence the bone marrow mass and total blood volume are reduced. High haemoglobin levels may thus be obtained with no increase in blood intake. [ABSTRACT FROM AUTHOR]

Liver biopsies were performed in 47 thalassaemic children. 33 of them had laboratory findings that suggested chronic hepatitis; the other 14 patients, without such signs, underwent liver biopsies during splenectomy. Nine patients showed a more or less pronounced fibrosis, 26 showed chronic persistent hepatitis, and 12 had histological evidence of chronic aggressive hepatitis. The last 12 patients who had been treated with corticosteroids, and 9 other patients who showed a worsening of their liver function tests, underwent sequential biopsy. We suggest that chronic liver disease in thalassaemic children can produce inflammatory infiltration even without biochemical or clinical sign of chronic hepatitis, that it can progress towards cirrhosis even in patients with chronic persistent hepatitis, and that there appears to be no benefit in giving corticosteroids. [ABSTRACT FROM AUTHOR]

The presence of circulating soluble immune complexes and the level of complement were investigated in sera from 21 patients with beta-thalassaemia major, including both splenectomised and nonsplenectomised patients. A high level of immune complexes was found in half of these cases. Reduced complement levels were seen less frequently. There was no correlation between the presence of circulating immune complexes, decreased complement levels, and thpresence or absence of the spleen. The level of immune complexes increased with the age ofthe individual, i.e. with the duration of the disease. [ABSTRACT FROM AUTHOR]