Immunophenotype of neoplastic plasma cells in AL amyloidosis

Abstract

Aim: AL amyloidosis (ALA) is a form of plasma cell (PC) dyscrasia characterised by deposition of insoluble fibrillar deposits in various organs causing organ damage. This is believed to be the first study to evaluate the expression of CD79a, CD20, CD56, cyclin D1 and epithelial membrane antigen (EMA) in neoplastic PCs of ALA.

Methods and results: The study included 36 well-documented cases of ALA. In all cases presence of amyloid deposits had been documented by Congo Red stain in the bone marrow trephine biopsy (BMTB) and/or in other tissues. BMTBs showed varying degrees of PC infiltration (median 12%). In eight of the 36 cases with associated myeloma, the mean (2 x SE) percentage of PCs (PC%) was 34 (18)%, while in the remaining, PC% was 15 (4)%. Expression of CD20, CD79a, CD56, cyclin D1 and EMA was noted in 42%, 86%, 50%, 53% and 83% of cases, respectively. Aberrant antigen expression in the form of CD56 and/or cyclin D1 expression was seen in 79% of cases. Nine of 10 cases with small lymphoid-like PCs were positive for CD20 and all the 10 cases were positive for cyclin D1. On the other hand, among cases without small lymphoid-like morphology, CD20 and cyclin D1 expression was seen in only 6 of 26 and 8 of 26 cases respectively (p = 0.001 and 0.002 respectively). CD20 expression correlated with cyclin D1 expression (p = 0.045). Cytological atypia/pleomorphism was predictive of associated myeloma (p = <0.001).

Conclusion: Marrow involvement by neoplastic PCs in ALA can be identified by their aberrant antigen expression apart from light chain restriction, and rituximab as a possible treatment option may be explored in CD20-positive ALA.