Uneventful administration of vincristine in CMT 1X

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Pediatr Blood Cancer. 2007 May 18

Uneventful administration of vincristine in Charcot-Marie-Tooth

disease type 1X.

Ajitsaria R, Reilly M, J.

Department of Paediatric Oncology, Great Ormond Street Hospital,

London, UK.

Charcot-Marie-Tooth (CMT) disease is the most common inherited

peripheral neuropathy. Administration of vinca alkaloids is

considered contraindicated in CMT because of the risk of life

threatening acute neuropathy.

We describe a 5-year-old patient with a family history of X-linked

CMT1 who presented with Wilms tumour. Despite confirmation of a

connexin 32 point mutation, the patient was treated with 40.5 mg/m

(2) of vincristine with no clinical evidence of drug-related

neuropathy.

This case describes vincristine being safely administered to a

patient with molecularly proven type CMT 1X, but in type 1A CMT

vincristine is still contraindicated.

An extensive literature review revealed no other series of cases in

which vincristine administration in molecularly proven CMT 1X had

been described.

CMT should be excluded in any patient who develops a profound, acute

neuropathy following vincristine, as many patients in the cases

reviewed were asymptomatic prior to treatment.