Plasma cell leukemia (PCL) is a rare plasma cell disorder. It is the leukemic variant of multiple myeloma. A 52-year-old man with an atypical presentation of primary plasma cell leukemia is reported. The patient presented with paraparesis which progressively worsened to paraplegia. MRI of the spine showed an extradural mass causing cord compression and multiple bony erosions from soft tissue masses. Peripheral blood film examination and bone marrow aspiration showed numerous plasmablasts. Atypical cells expressed surface and cytoplasmic lambda light chain on immunochemical studies, surface CD45 and CD38. To our knowledge, primary PCL presenting with progressive paraplegia has not been reported in the literature.
Plasma cell leukaemia (PCL) is an aggressive haematological malignancy which is classified into primary (pPCL) and secondary PCL. A 39-year-old Indian man presented to the Department of Hematology with a 2-week history of fever and lethargy. Clinically, he was pale and febrile. Haemogram revealed bicytopenia with leucocytosis. The peripheral blood film portrayed rouleax formation with 45% of circulating plasma cells. Serum protein electrophoresis and immunofixation revealed IgG lambda paraproteinaemia of 48 g/L. Bone marrow aspirate, flow cytometry and trephine were consistent with IgG lambda pPCL. He was treated with six cycles of bortezomib, thalidomide and dexamethasone combination chemotherapy followed by high-dose melphalan conditioning and autologous stem cell transplant. Currently, he is in complete remission for the past 18 months and is on oral lenalidomide maintenance therapy. Prognosis is often dismal in pPCL with the median overall survival below 1 year if treatment is delayed.