A NEW MYELOPROLIFERATIVE DISORDER ASSOCIATED WITH CHROMOSOMAL TRANSLOCATIONS INVOLVING 8P11 - A REVIEW

Chromosomal breakpoints associated with malignancy are known to cluste r at particular regions of the karyotype. Based on a review of the lit erature we have identified a novel leukaemia syndrome associated with translocations involving 8p11. This syndrome is distinct from the prev iously described translocation t(8;16)(p11;p13) associated with acute monoblastic leukaemia. We have summarized the clinical and cytogenetic features of 13 case reports which describe a myeloproliferative syndr ome with eosinophilia, lymphadenopathy and a high incidence of T cell non-Hodgkin's lymphoma with progression to acute myeloid leukaemia. Th e translocations involving 8p11 were: either t(8;13)(p11-12;q11-12), t (8;9) (p11;q32-34) or t(6;8)(q27;p12). In two cases of t(8;13) molecul ar studies have mapped the chromosome 13 breakpoint to a 1.5 Mbp regio n, but a full molecular characterization of these translocations is re quired. In view of the striking clinicopathological and karyotypic sim ilarities between these cases we propose that they be considered a sin gle nosological entity and termed '8p11 myeloproliferative syndrome'.