Intravascular lymphomatosis presenting as an ascending cauda equina: conus medullaris syndrome: remission after biweekly CHOP therapy.

Abstract

A 63 year old man developed dysaesthesia in the legs followed by a subacute ascending flaccid paraparesis with sacral sensory and autonomic involvement. Intravascular lymphomatosis (IVL) was favoured by the presence of low grade fever and raised serum C reactive protein, CSF pleocytosis, raised lymphoma markers (serum LDH, soluble IL-2 receptor), and steroid responsiveness. Only muscle, among several organ biopsies, confirmed IVL. A cytogenetic study of the bone marrow showed chromosome 6 monosomy, as previously reported. The monosomy of chromosome 19, which bears the intercellular cell adhesion molecule-1, newly found in this case, may be related to the unique tumour embolisation of IVL. The CHOP regimen (six courses in 12 weeks) using granulocyte colony stimulating factor (G-CSF) led to gradual resolution of myeloradiculopathy and laboratory supported remission lasting for more than 13 months. The biweekly CHOP with G-CSF support may be a choice of chemotherapy in averting rapidly fatal IVL.