Post-Essential Thrombocythemia Myelofibrosis and Multiple Isodicentric Y Chromosomes: A Unique Case among a Rare Association.

Date publication

février 2020

Journal

Cytogenetic and genome research

Auteurs

Membres identifiés du Cancéropôle Est :
Dr FERRAND Christophe , Dr COLLONGE-RAME Marie-Agnès , Pr KUENTZ Paul

Tous les auteurs :
Dahlen E, Sarghi SI, Renosi F, Ferrand C, Collonge-Rame MA, Kuentz P

Lien Pubmed

https://www.ncbi.nlm.nih.gov/pubmed/32008001

Résumé

Multiple isodicentric Y chromosomes [idic(Y)] is a rare cytogenetic abnormality, most exclusively described in constitutional karyotypes. Only recently has this entity been reported in hematologic neoplasms such as myeloid disorders, albeit these cases remain very scarce. The possible involvement of increasing copies of potential proto-oncogenes located on the multiple idic(Y) led to consider one of them, CRLF2, as a target for kinase inhibitors. We report here, to our knowledge, the first case of multiple idic(Y) in a patient with myelofibrosis secondary to essential thrombocythemia. The patient received ruxolitinib therapy with initial good clinical response.

Mots clés

CRLF2, Essential thrombocythemia, FISH, Isodicentric Y chromosomes, Myelofibrosis, Ruxolitinib

Référence

Cytogenet. Genome Res.. 2020 Feb 1;: