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. 2015 Aug;39(8):822-7.
doi: 10.1016/j.leukres.2015.05.002. Epub 2015 May 12.

Association of lymphoid malignancies and Philadelphia-chromosome negative myeloproliferative neoplasms: Clinical characteristics, therapy and outcome

Lucia Masarova  1 , Kate J Newberry  1 , Sherry A Pierce  1 , Zeev Estrov  1 , Jorge E Cortes  1 , Hagop M Kantarjian  1 , Srdan Verstovsek  2
Affiliations

Association of lymphoid malignancies and Philadelphia-chromosome negative myeloproliferative neoplasms: Clinical characteristics, therapy and outcome

Lucia Masarova et al. Leuk Res. 2015 Aug.

Abstract

The co-occurrence of myeloproliferative and lymphoproliferative neoplasms (MPN/LPN) has been reported, mostly in case reports. The aim of this study was to assess the characteristics and clinical course of the coexistent diseases. Among 9866 patients who presented to our institution from 1960 to 2014, 34 (0.3%) were diagnosed with MPN/LPN. LPN was diagnosed first in 16 patients, second in 15, and at the same time in 3. The time to secondary malignancy was longer when LPN was diagnosed first (119 vs 98 months). Myelofibrosis (41%), polycythemia vera (24%), and essential thrombocythemia (18%) were the most common MPNs, and non-Hodgkin lymphoma (50%) and chronic lymphocytic leukemia (32%) were the most common LPNs. Seventy-three percent of patients treated for MPN and 72% of those treated for LPN achieved a complete response. After a median follow-up from MPN diagnosis of 84 months, 16 patients are alive and 18 died (4 related to MPN and 2 LPN). Coexistent MPN/LPN is a rare event that does not appear to predict worse outcomes. Treatment choice is generally oriented towards controlling the prevalent disease; the other malignancy may influence treatment strategies in selected cases.

Keywords: Clinical course; Concurrent; Lymphoproliferative neoplasm; Myeloproliferative neoplasm.

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Figures

Figure 1
Figure 1
Order of LPN/MPN diagnosis and disease duration during the observation period for all patients (0 year value represents the time of MPN diagnosis).
See this image and copyright information in PMC

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