1Department of Hematology, VU University Medical Center, HV Amsterdam, The Netherlands2Department of Neurology, VU University Medical Center, HV Amsterdam, The Netherlands
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A 51-year-old male was diagnosed with chronic phase chronic myeloid leukemia (CML) in May 2005. His Sokal score at diagnosis was 0.95 and his Euro score was 1,363 (both intermediate risk). Cytogenetic examination showed 46XY, t(9;22)(q34;q11.2) in all of 30 metaphases.BCR-ABLPCR was positive for an e14a2 transcript. He was included in the HOVON-51 study (www.hovon.nl) and received two cycles of cytarabine 200 mg/m2 for 7 days and imatinib 800 mg, q.d., continuously. Swiftly, he attained a complete and durable cytogenetic response (at 3 months after start of treatment) and a major molecular response (0.05% compared with diagnosis) already at 3 months after start of treatment that was maintained at further followup. At 12 months after start of treatment, he complained of progressive hearing problems. There was a perceptive and a conductive loss. An magnetic resonance imaging (MRI) scan of the brain did not show any abnormality of the cerebral parenchyma, meninges, or cranial nerves. He was given a hearing aid and middle ear drainage tubes. However, his hearing loss deteriorated so much that he became unable to do his work as a general practitioner. Six months after his hearing loss had started, he developed progressive confusion, ataxia, and word finding disturbances for which he was admitted to the neurology ward in March 2007. He was afebrile and had a normal blood pressure. There was no lymphadenopathy, nor hepatosplenomegaly. On neurological examination, he appeared bradyphrenic and showed reduced attention. There were slight word finding disturbances and some paraphasias. Neck movements were painful in all directions. There was a slight pupillary anisocoria (right > left) and a staggering gait without further neurological abnormalities.