title: Chronic lymphocytic leukemia and myeloproliferative neoplasms concurrently diagnosed: clinical and biological characteristics.
abstract: Chronic lymphocytic leukemia (CLL) and myeloproliferative neoplasms (MPN) may occur concomitantly. However, little is known about the pathobiological characteristics and interaction between the neoplastic clones in these rare cases of coinciding malignancies. We retrospectively examined the clinical and biological characteristics of 13 patients with concomitant CLL and MPN--eight primary myelofibrosis (PMF), three essential thrombocytosis (ET), and two polycythemia vera (PV)--who presented to our institution between 1998 and 2014, and tested all patients for MPN-specific aberrations, such as JAK2, MPL and CALR mutations. Along with epidemiological and molecular characterization of this rare condition, we found that JAK2 mutation can be detected 9 years prior to PMF diagnosis, suggesting that PMF clinical phenotype may require several years to develop and CLL/MPN clinical co-occurrence might be sustained by common molecular events. Some features of these patients suggest that pathobiologies of these diseases might be intertwined.
authors.affiliation: a Department of Leukemia , The University of Texas MD Anderson Cancer Center , Houston , TX , USA.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Todisco
authors.affiliation: a Department of Leukemia , The University of Texas MD Anderson Cancer Center , Houston , TX , USA.
authors.forename: Taghi
authors.initials: T
authors.lastname: Manshouri
authors.affiliation: a Department of Leukemia , The University of Texas MD Anderson Cancer Center , Houston , TX , USA.
authors.forename: Srdan
authors.initials: S
authors.lastname: Verstovsek
authors.affiliation: a Department of Leukemia , The University of Texas MD Anderson Cancer Center , Houston , TX , USA.
authors.forename: Lucia
authors.initials: L
authors.lastname: Masarova
authors.affiliation: a Department of Leukemia , The University of Texas MD Anderson Cancer Center , Houston , TX , USA.
authors.forename: Sherry A
authors.initials: SA
authors.lastname: Pierce
authors.affiliation: a Department of Leukemia , The University of Texas MD Anderson Cancer Center , Houston , TX , USA.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Keating
authors.affiliation: a Department of Leukemia , The University of Texas MD Anderson Cancer Center , Houston , TX , USA.
authors.forename: Zeev
authors.initials: Z
authors.lastname: Estrov
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D053614:Janus Kinase 2 / Q000235:genetics; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009196:Myeloproliferative Disorders / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D009367:Neoplasm Staging; D063868:Patient Outcome Assessment; D011087:Polycythemia Vera; D055728:Primary Myelofibrosis; D053628:Receptors, Thrombopoietin / Q000235:genetics; D012189:Retrospective Studies; D013920:Thrombocythemia, Essential
chemical_list: D007143:Immunoglobulin Heavy Chains; D053628:Receptors, Thrombopoietin; C082218:MPL protein, human; C507924:JAK2 protein, human; D053614:Janus Kinase 2
keywords: CALR; Chronic lymphocytic leukemia; JAK2; MPL; myeloproliferative neoplasms; primary myelofibrosis