Cookies on this website
We use cookies to ensure that we give you the best experience on our website. If you click 'Continue' we'll assume that you are happy to receive all cookies and you won't see this message again. Click 'Find out more' for information on how to change your cookie settings.

© 2009 by Informa Healthcare USA, Inc. The 5q- syndrome was first described as a “distinct haematological disorder with deletion of long arm of no. 5 chromosome” by Herman van Den Berghe and his colleagues in Leuven, Belgium, in 1974 (1). The syndrome was described in more detail in a subsequent report in 1975 by Gerard Sokal and colleagues, also in Leuven (2). The principal features described in these early reports were a moderate-to-severe macrocytic anemia, with slight leukopenia but normal or elevated platelet count. The bone marrow showed a depressed erythroid series with an occasional excess of myeloblasts. Most of the megakaryocytes had a nonlobulated nucleus. It was recognized early that patients with the 5q- syndrome had a female preponderance, and a good prognosis with a low transformation rate to acute myeloid leukemia (AML) (3). The 5q- syndrome quickly became linked with the myelodysplastic syndromes (MDS). The World Health Organization (WHO) definition of the 5q- syndrome (see chap. 9) is part of the WHO’s MDS classification and includes a more stringent requirement for the patient to be in the refractory anemia (RA) subgroup of MDS, excluding patients with over 5% blasts. Those patients who fall outside the WHO definition of the 5q- syndrome may have some of the hematological features of the 5q- syndrome, but nevertheless do not share its good prognosis (4).

Type

Chapter

Book title

Myelodysplastic Syndromes: Pathobiology and Clinical Management, Second Edition

Publication Date

01/01/2008

Pages

267 - 284