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

Abstract

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 × 109 [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]. Primary extra medullary plasmacytomas of the gastrointestinal tract represents <5% of all extra medullary plasmacytomas [8]. Any segment of the gastrointestinal tract may be the site of plasma cell infiltration. The small bowel is the most commonly involved site, followed by stomach, colon and esophagus [9]. The most common presenting symptoms are abdominal pain and gastrointestinal bleeding. A few case reports of solitary gastric plasmacytomas have been described in literature [10], [11]. A case reported a solitary gastric plasmacytoma in a 46-year-old male who presented with melena and was treated with partial gastrectomy, progressed to having local recurrence and respiratory obstruction due to large mediastinal deposits after 18 months. Eventually transforming into lambda light chain type multiple myeloma with multiple visceral plasmacytomas within a few months [11]. There is only one case reported in literature of a non-secretory PCL with gastric involvement, where a 49 year old male was diagnosed with non-secretory Ig A PPCL, co-expressing CD38 and CD13 on immunophenotyping. He presented with epigastric discomfort and was found to have multiple submucosal masses with ulcerations in the body of the stomach on endoscopy. On biopsy confirmed to be gastric involvement by PCL and he eventually died of cerebral hemorrhage [12]. Here we present a case of a 79-year-old Caucasian male who presented with symptomatic anemia, and was diagnosed with an aggressive type of plasma cell leukemia and a large ulcerated and hemorrhagic gastric plasmacytoma.