A new myeloproliferative disorder associated with chromosomal translocations involving 8p11: a review

• Published in: Leukemia Vol. 9; no. 10; p. 1628
• Main Authors: Macdonald, D, Aguiar, R C, Mason, P J, Goldman, J M, Cross, N C
• Format: Journal Article
• Language: English
• Published: England 01-10-1995
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Child, Preschool; Chromosomes, Human, Pair 11 - genetics; Chromosomes, Human, Pair 6 - genetics; Chromosomes, Human, Pair 8 - genetics; Chromosomes, Human, Pair 9 - genetics; Female; Humans; Karyotyping; Male; Middle Aged; Myeloproliferative Disorders - genetics; Myeloproliferative Disorders - pathology; Prognosis; Translocation, Genetic - genetics
• ISSN: 0887-6924

Chromosomal breakpoints associated with malignancy are known to cluster at particular regions of the karyotype. Based on a review of the literature we have identified a novel leukaemia syndrome associated with translocations involving 8p11. This syndrome is distinct from the previously 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 syndrome with eosinophilia, lymphadenopathy and a high incidence of T cell non-Hodgkin's lymphoma with progression to acute myeloid leukaemia. The 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) molecular studies have mapped the chromosome 13 breakpoint to a 1.5 Mbp region, but a full molecular characterization of these translocations is required. In view of the striking clinicopathological and karyotypic similarities between these cases we propose that they be considered a single nosological entity and termed '8p11 myeloproliferative syndrome'.