A Case of Polycythemia Vera with a JAK2V617F Mutation Combined with Smoldering Myeloma

Abstract

The lymphoproliferative disease multiple myeloma and the myeloproliferative disease polycythemia vera have different pathogenic mechanisms and different natural courses. Thus, the concomitant development of these two diseases in the same individual is rare. In most previously reported cases of both diseases, one disease was assumed to be a secondary malignancy caused by chemotherapy for the other primary disease. Our case was diagnosed as smoldering myeloma based on increased bone marrow plasma cell numbers and monoclonal gammopathy during a regular follow-up visit for JAK2V617F mutation-positive polycythemia vera, which had not been treated except with phlebotomy. This case provides useful clues for understanding the pathogenesis of these two hematological malignancies and the association between them. Here, we report a case of polycythemia vera with a JAK2V617F mutation combined with smoldering myeloma and discuss the clinical significance and pathogenic association between these disorders of different lineages, along with a literature review. (Korean J Med 2013;84:308-312)