AUTOIMMUNE & INFLAMMATORY NMD: EP.15 Severe Guillain-Barre syndrome associated with nelarabine with good prognosis

Abstract

Nelarabine is a purine nucleoside analogue which has T-cell specificity and is highly active in relapsed and refractory T-cell lymphoma. Neurotoxicity occurs in approximately 20% of patients treated with nelarabine and is predominantly peripheral neuropathy. Reports of recovery are variable. We report a severe nelarabine related neuropathy with quadriparesis and respiratory failure and excellent recovery. Diagnosis of stage III biphenotypic lymphoblastic lymphoma was made at 11 years of age in a previously healthy male who presented with supraclavicular lymphadenopathy. He was treated according to UKALL 2011 schedule B. Two months later, as there had been no response to treatment, he received nelarabine. Within two weeks he developed limb weakness and sensory symptoms, weakness progressed rapidly to quadriparesis and respiratory failure. MRI scan of spine showed diffusely thickened nerve roots with contrast enhancement, CSF was acellular with protein 3.27 g/L, initial nerve conduction study showed attenuated motor responses and absent F-waves. He had five cycles of plasmapheresis and IVIG. He required ventilation for 50 days. Muscle weakness slowly improved. He received a matched unrelated donor stem cell transplant (SCT) 6 months after his neuropathy onset. At review, 21 months post-SCT, he remains in remission from his lymphoma, has only mild residual ankle dorsiflexion weakness and no functional impairment. This case of neuropathy temporally associated with nelarabine administration fulfills diagnostic criteria for GBS and raises the possibility that the neuropathy was immune-mediated rather than due to direct neurotoxicity. GBS like presentation of neuropathy following nelarabine administration may have a better prognosis with implications for continuation of life-sustaining treatment and treatment of the malignancy.