Chronic haemolytic anaemia associated with adenylate kinase (AK) deficiency is very rare and only seven cases in five families have been described. We present six children of one family who are deficient of this enzyme and in three of them a combined G6PD deficiency was found. AK deficiency was transmitted by an autosomal recessive gene and heterozygous state was not accompanied by disease, whereas homozygously affected individuals present a congenital chronic non-spherocytic haemolytic anaemia with haemoglobin levels of 8-9 g/dl. Patients also deficient in G6PD suffer from a more severe haemolytic anaemia with haemoglobin levels around 6 g/dl. The AK-deficient children are also mentally retarded. Splenectomy performed in five of the six patients resulted in complete remission of the haemolytic process.
An Israeli Jewish woman who originated from the Uzbekskaya SSR in the south of the Soviet Union, had anti-Dra in her serum and her red cells were Dr(a-) as were those of three of her four children. All Dr(a-) red cells had weakened expression of their Cra, Tca, Esa, IFC, and other Cromer-related antigens. It is suggested that the Dr(a-) phenotype may result from inheritance of a dominant inhibitor gene, although a relatively common recessive allele of Dra was not excluded. Anti-Dra was inhibited by serums from the Dr(a+), but not the Dr(a-), members of the family.
Before and/or after chemotherapy was administered to patients with Burkitt's lymphoma (BL) or lymphoblastic lymphosarcoma (LLS), their sera and those of matched controls were tested for antibodies to Epstein-Barr virus (EBV) capsid antigen (VCA) and early antigen by the indirect immunofluorescence method. Ten of the 16 BL patients were Arab children and 8 of the 11 LLS patients were Jews of Asian-African origin. Although half the BL patients did not have elevated antibody titers when their disease was diagnosed, significantly higher ones were detected in the BL group as compared with the LLS patients and their matched controls; Arab patients had the highest titers. IgM antibodies specific for VCA were found in 2 patients concurrently with elevated titers. We found no correlation between the clinical course of BL and the patients' antibody titers to EBV.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations –citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.