Survival of twenty-two months in a patient with primary plasma cell leukaemia treated with melphalan and prednisolone

Abstract

Summary In the majority of cases plasma cell leukaemia is a rapidly fatal disease with a mean survival time of five months. There have been reports of increased survival using various regimens of chemotherapy although most cases eventually relapse. We describe a patient with primary plasma cell leukaemia who responded to a combination of oral melphalan and prednisolone with control of the disease in the bone marrow but relapsed with extramedullary disease in the central nervous system and testes, and died 22 months after diagnosis. Melphalan poorly penetrates the central nervous system and its testicular penetration is unknown.

Primary plasma cell leukemia followed by testicular plasmacytoma

“…In the past, only two other cases of similar localizations in primary PCL are reported [12,13]; both of patients developed a testicular relapse, and, similarly to our case, Extramedullary plasma cell leukemia 225 they had a poor prognosis. It has been suggested that the lower temperature in the scrotum creates a non-anatomical blood-testes barrier, making this organ a sanctuary site for disease from chemotherapeutic attack during initial treatment [12].…”

“…It has been suggested that the lower temperature in the scrotum creates a non-anatomical blood-testes barrier, making this organ a sanctuary site for disease from chemotherapeutic attack during initial treatment [12]. Our patient obtained a complete hematological remission after induction with a therapeutic regimen based on the association of steroids with a single alkylating agent, cyclophosphamide at intermediate doses, whose administration is not well described; on the contrary more information are available about melphalan, a different alkylating agent, used at standard or intensive doses in primary PCL, but often with unsatisfactory results.…”