CENTRAL NERVOUS SYSTEM RELAPSE OF MULTIPLE MYELOMA

Abstract

Multiple myeloma (MM) is a plasma cell disorder primarily involving bone marrow. Extramedullary involvement is less common, with central nervous system (CNS) myelomatosis being a rare entity and such presentation carries extremely dismal prognosis. We present case of a 40 years old male with MM who was initially treated with 6 cycles of Cyclophosphamide, Thalidomide and Dexamethasone resulting in complete response. 2 years later he presented with CNS myelomatosis and scrotal involvement and was initially treated with Bortezomib and dexamethasone, cranial irradiation and intrathecal Methorexate, Cytarabine, Hydrocortisone (TRIO IT), along with radical orchiectomy and testicular radiation during the course of treatment. However, after initial response his disease showed clinical and radiological progression after 4 months of therapy. He was switched to high dose Methotrexate (HD-MTX) with TRIOIT
and later Lenalidamide and dexamethasone (Len/dex) was added to the above regimen. He continued to show good clinical response but his cytology remained persistently positive, therefore, HD-MTX was discontinued in the later course of treatment. Subsequently he was started on best supportive care only, when his neurological status deteriorated further. He survived almost 9 months after a diagnosis of CNS myelomatosis. Patients with multiple myeloma, presenting with neurological symptoms should always be investigated for the possibility of CNS MM. CNS relapse is a fatal disease with poor prognosis. Recommended treatment must include a systemic anti-MM regimen that crosses the BBB (ideally Immunomodulatory drugs (IMiDs) IMiDs-dexamethasone based therapy), CNS irradiation and intrathecal chemotherapy.

Key words: Multiple myeloma, central nervous system myelomatosis, therapy