A rare case of a minimally secretory plasma cell leukemia with a hemorrhagic gastric plasmacytoma

摘要

Plasma cell leukemia(PCL) is a rare and aggressive plasma cell neoplasm characterized by clonal plasma cells comprising more than 20% of the leukocytes in the circulation and/or an absolute clonal plasma cell count of more than 2 × 10 9 [1,2]. PCL accounts for 2–4% of plasma cell dyscrasias [3]. PCL is more common in African Americans, compared to Caucasians [3], with more male preponderance [4]. PCL arising de novo without prior evidence of multiple myeloma (MM) is termed primary PCL(PPCL), and PCL evolving from a preexisting MM is termed as secondary PCL(SPCL). According to the literature, 60–70% of PCLs are primary, and 30–40% are secondary [3,5]. The chances of refractory and relapsed MM progressing to SPCL is 1–2% [3,6]. PCL is a rare disease and the precise information regarding its incidence, clinical presentation, and pathological features are unfolding gradually [7].