Plasma cell leukemia - A comprehensive analysis of clinical and pathological features of 7 cases

Abstract

Background: Plasma cell leukemia (PCL) is a rare, yet aggressive plasma cell (PC) neoplasm, variant of multiple myeloma (MM), characterized by high levels of PCs circulating in the peripheral blood. PCL can either originate de novo (primary PCL) or as a secondary leukemic transformation of MM (secondary PCL) and is characterized by circulating PCs >2×109/L in peripheral blood and a peripheral blood plasmacytosis >20%. Aims & Objective: Present study was undertaken to analyze the main clinical & pathological features of PCL. For diagnostic purpose the morphological appearances and confirmation by immunophenotyping are emphasized rather than more sophisticated testing methods that may not be widely available. Material and Methods: A descriptive study was carried out in the department of Pathology, in a tertiary care teaching hospital, Ahmedabad, India during year 2009-2013. We investigated the important clinical characteristics, pathological,biochemical & radiological features, immunophenotype, & prognostic factors of 7 patients of PCL. Results: Common clinical features at diagnosis were anaemia, renal insufficiency, bone pain, splenomegaly or hepatomegaly. Anaemia, leucocytosis, thrombocytopenia & plasmacytosis were seen in peripheral blood. Plasma cell marker - CD 38 & CD 138 were expressed in all cases. Serum β2-microglobulin, serum LDH were increased & serum albumin was decreased in all 7 cases & were associated with poor prognosis. The median survival time from diagnosis was 9 months. Conclusion: Plasma cells have characteristic morphological features which can be easily identified on peripheral blood & bone marrow examination.CD 38 & CD 138 are excellent plasma cell markers. Increased serum β2-microglobulin &serum LDH & decreased serum albumin are potent poor prognosis factors. PCL is aggressive neoplasm with poorresponse to chemotherapy & low median survival time from diagnosis.