PRIMARY PLASMA CELL LEUKAEMIA

*Abotaleb Alia A. MD

ABSTRACT

Background: Plasma cell leukemia (PCL) is a rare and aggressive form of plasma cell disorder characterized by the presence of circulating plasma cells. It is classified as either primary PCL (pPCL) or as secondary PCL (sPCL). Intensive chemotherapy regimens and bortezomib-based regimens are recommended followed by high-dose therapy with autologous stem-cell transplantation (HDT/ASCT) if feasible. Because the incidence of PCL is relatively low and fulminant presentation, we are reporting this case. Case Summery: A 52-year-old Saudi woman with a history of hypertension, diabetes mellitus, dyslipidemia and morbid obesity presented with symptoms of anemia and recurrent community acquired pneumonia.Laboratory evaluation showed normocytic anemia, leukocytosis with 46% atypical lymphocytes, and normal platelets. Peripheral smear and flow cytometry confirmed PCL with 30% plasma cells. Bone marrow biopsy demonstrated 50% plasma cells (38+, 138+, 117+, 10-, 19-, 20-, 56-) and hypercellularity. Fish on bone marrow showed p53 in 40% of cells, deletion 13q in 30% of cells, duplication 1q and deletion 1p.VCD therapy was started then upgraded to VD-PACE regimen to be followed by autologous bone marrow transplantation but unfourtionatly deteriorated and died due to septic shock and multi-organ failure. Conclusion: PCL is the most aggressive variant of monoclonal gammopathy that carry a poor prognosis despite of all available treatment modalities. From what described in the literatures and by looking to our case, pPCL sometimes is difficult to be differentiated from sPCL, so we need more biological and immunohistochemistry tools in addition to more molecular studies to help in distinguish between these two forms. Multi-center studies and clinical trials should be conducted to develop accurate criteria for the early diagnosis and prompt treatment of this disease.

Keywords: Case report, multiple myeloma, plasma cell leukemia, VD-PACE, VCD, ASCT.