Universität zu Köln

Tefferi, Ayalew, Guglielmelli, Paola ORCID: 0000-0003-1809-284X, Larson, Dirk R., Finke, Christy, Wassie, Emnet A., Pieri, Lisa ORCID: 0000-0003-1812-8072, Gangat, Naseema, Fjerza, Rajmonda, Belachew, Alem A., Lasho, Terra L., Ketterling, Rhett P., Hanson, Curtis A., Rambaldi, Alessandro ORCID: 0000-0002-3739-7502, Finazzi, Guido, Thiele, Juergen, Barbui, Tiziano, Pardanani, Animesh and Vannucchi, Alessandro M. (2014). Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis. Blood, 124 (16). S. 2507 - 2514. WASHINGTON: AMER SOC HEMATOLOGY. ISSN 1528-0020

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Abstract

Janus kinase 2 (JAK2) mutations define polycythemia vera (PV). Calreticulin (CALR) and myeloproliferative leukemia virus oncogene (MPL) mutations are specific to JAK2-unmutated essential thrombocythemia (ET) and primarymyelofibrosis (PMF). We examined the effect of these mutations on long-term disease outcome. One thousand five hundred eighty-one patients from the Mayo Clinic (n = 826) and Italy (n = 755) were studied. Fifty-eight percent of Mayo patients were followed until death; median survivals were 19.8 years in ET (n = 292), 13.5 PV (n = 267; hazard ratio [HR], 1.8; 95% confidence interval [CI], 1.4-2.2), and 5.9 PMF(n = 267; HR, 4.5; 95% CI, 3.5-5.7). The survival advantage of ET over PV was not affected by JAK2/CALR/MPL mutational status. Survival in ET was inferior to the age-and sex-matched US population (P < .001). In PMF (n = 428), but not in ET (n = 576), survival and blast transformation (BT) were significantly affected by mutational status; outcome was best in CALR-mutated and worst in triple-negative patients: median survival, 16 vs 2.3 years (HR, 5.1; 95% CI, 3.2-8.0) and BT, 6.5% vs 25% (HR, 7.6; 95% CI, 2.8-20.2), respectively. We conclude that life expectancy in morphologically defined ET is significantly reduced but remains superior to that of PV, regardless of mutational status. In PMF, JAK2/CALR/MPL mutational status is prognostically informative.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Tefferi, Ayalew UNSPECIFIED UNSPECIFIED UNSPECIFIED Guglielmelli, Paola UNSPECIFIED orcid.org/0000-0003-1809-284X UNSPECIFIED Larson, Dirk R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Finke, Christy UNSPECIFIED UNSPECIFIED UNSPECIFIED Wassie, Emnet A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Pieri, Lisa UNSPECIFIED orcid.org/0000-0003-1812-8072 UNSPECIFIED Gangat, Naseema UNSPECIFIED UNSPECIFIED UNSPECIFIED Fjerza, Rajmonda UNSPECIFIED UNSPECIFIED UNSPECIFIED Belachew, Alem A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Lasho, Terra L. UNSPECIFIED UNSPECIFIED UNSPECIFIED Ketterling, Rhett P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hanson, Curtis A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Rambaldi, Alessandro UNSPECIFIED orcid.org/0000-0002-3739-7502 UNSPECIFIED Finazzi, Guido UNSPECIFIED UNSPECIFIED UNSPECIFIED Thiele, Juergen UNSPECIFIED UNSPECIFIED UNSPECIFIED Barbui, Tiziano UNSPECIFIED UNSPECIFIED UNSPECIFIED Pardanani, Animesh UNSPECIFIED UNSPECIFIED UNSPECIFIED Vannucchi, Alessandro M. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-425707
DOI:	10.1182/blood-2014-05-579136
Journal or Publication Title:	Blood
Volume:	124
Number:	16
Page Range:	S. 2507 - 2514
Date:	2014
Publisher:	AMER SOC HEMATOLOGY
Place of Publication:	WASHINGTON
ISSN:	1528-0020
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language TYPE-2 CALRETICULIN MUTATIONS; MYELOPROLIFERATIVE NEOPLASMS; SOMATIC MUTATIONS; CALR; JAK2; CLASSIFICATION; DIAGNOSIS; PHENOTYPE; PROGNOSIS; SUBTYPES Multiple languages Hematology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/42570