Table of Contents
ISRN Hematology
Volume 2011, Article ID 404057, 3 pages
http://dx.doi.org/10.5402/2011/404057
Case Report

Coexistence of Plasma Cell Dyscrasia with Prefibrotic Stage of Primary Myelofibrosis: A Case Report

1Department of Haematology, University Hospital of Heraklion, 71110 Heraklion Crete, Greece
2Department of Nephrology, University Hospital of Heraklion, 71110 Heraklion Crete, Greece

Received 18 February 2011; Accepted 24 March 2011

Academic Editors: Frederick W. Quelle and Jean-Luc Villeval

Copyright © 2011 George Tsirakis et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Introduction. Coexistence of myeloproliferative neoplasms with lymphoproliferative syndromes has been described in the past, whereas plasma cell dyscrasias seem to be the most common cases. Case Presentation. We present a case of a 59-year-old Caucasian female of Greek origin who presented with thrombocytosis. Clinical and laboratory investigation disclosed the presence of a smoldering myeloma with coexisting histological and molecular characteristics of primary myelofibrosis. The patient had the acquired point mutation V617F in the JAK2 gene but not the bcr-abl rearrangement and was treated for myelofibrosis with subsequent improvement of all haematological parameters without evidence of myelomatic evolution. Conclusion. We present the first case in the literature of a smoldering myeloma coexisting with primary myelofibrosis. The underlying pathogenetic mechanism could be either related to the presence of a pluripotent neoplastic stem cell capable to differentiate into both lymphoid and myeloid cells or be related to two separate nosologic entities.