Plasma cell leukaemia (PCL) is a rare and aggressive plasma cell malignancy and is generally considered to be the final stage of multiple myeloma (MM). Although treatment modalities for MM have significantly evolved in the past decades, PCL unfortunately still retains an overall dismal prognosis, with most patients presenting with a highly symptomatic and aggressive disease course. We present a case of a transplant-ineligible patient diagnosed with a primary PCL who had an indolent presentation and achieved a durable complete remission after treatment via bortezomib-lenalidomide-dexamethasone (VRD). The present case illustrates the clinical heterogeneity of plasma cell disorders, and highlights the necessity of careful cytomorphological and flow cytometric analysis of aberrant lymphoid cells, even in the absence of stigmata of MM.

(BELG J HEMATOL 2022;13(7):277–80)