Persistent Epstein-Barr virus infection associated with monosomy 7 or chromosome 3 abnormality in childhood myeloproliferative disorders

Abstract

This report deals with myeloproliferative disorders associated with chronic, persistent Epstein-Barr virus (EBV) infection and with monosomy 7 and aberrations concerning chromosomes 3 and 5. Altogether 5 children were affected, their age ranging from 1-4 yr at time of clinical diagnosis. Principal symptoms were: hepatomegaly, splenomegaly, recurring upper respiratory tract infection and anemia. The serum IgG level remained persistently increased. Anti-EBV antibody concentrations were measured over a period of 9 months to 6 years, demonstrating persistently increased concentrations of IgG antibodies to viral capsid antigen (VCA) and against early antigen (EA). In 3 patients IgA antibodies were also studied and were found to be elevated. Within 2-5 yr 2 children developed chronic myelomonocytic leukemia from the chronic myeloproliferative syndrome. A 3rd patient who initially was diagnosed as chronic myelomonocytic leukemia developed acute leukemia within a period of 12 mo. A 4th patient with myeloproliferative syndrome went into spontaneous remission after an observation period of 2 yr. A 5th patient, the only one with translocation t(3;5)(q27;q33), displayed symptoms and a clinical course that can best be characterized as juvenile chronic myelocytic leukemia. The clinical, hematological, serological and cytogenetic findings may be related.