Primary Plasma Cell Leukaemia

A case history of a patient with primary plasma cell leukaemia is presented. Analysis of serum showed an IgD lambda paraprotein, and lambda-light-chains were found in the urine. Immunofluorescence studies of a bone marrow aspirate revealed intracytoplasmatic IgD of lambda-type in plasma cells. Moreover J-chain could be demonstrated in these plasma cells. A complete remission of the disease, with disappearance of the paraprotein, was obtained following treatment with a combination of cyclophosphamide, vincristine and prednisone. 12 months later, the patient developed multiple extramedullary plasmocytoma lesions in the skin. After treatment with cyclophosphamide, vincristine, doxorubicin and prednisone, another remission was achieved. The literature on the clinical features and the response to chemotherapy of primary plasma cell leukemia is reviewed.