Fast and Furious: a Case of High Risk IgD Plasma Cell Leukemia

Multiple myeloma (MM) is a malignant plasma cell disorder characterized by clonal proliferation of plasma cells in the bone marrow, leading to excessive monoclonal antibody production and subsequent end-organ damage. Plasma cell leukemia is a rare and aggressive leukemic variant of MM that occurs in approximately 0.5-4% of MM cases, and it is defined by the presence of ≥5% circulating plasma cells and classified as either primary (pPCL) or secondary (sPCL). Within MM, the IgD subtype is exceedingly rare, comprising only about 3% of PCL cases.

Here, we present a case of IgD pPCL in an adult male with rapid onset of anemia and renal dysfunction treated with bortezomib, dexamethasone, doxorubicin, cyclophosphamide, and etoposide following the VD-PACE regimen with omission of cisplatin due to renal impairment.

This case highlights the importance of prompt recognition of plasma cell leukemia and other more aggressive multiple myeloma phenotypes and individualized treatment to prevent long-term end-organ damage and improve survival.