Acute bilateral Proptosis and ophthalmoplegia as Isolated Ocular manifestation of accelerated phase chronic myeloid leukaemia on Nilotinib

Abstract

Chronic myeloid leukaemia (CML) is a myeloproliferative neoplasm characterized by Philadelphia chromosome with excessive tyrosine activity leading to uncontrolled proliferation of mature and maturing granulocytes, predominantly neutrophils. If left untreated CML goes though chronic phase, accelerated phase and ultimately Blast Crises. Accelerated phase is characterized by features of disease progression without meeting criteria for chronic phase or blast crises. We present a case of young male with CML who presented with acute bilateral proptosis and rapidly rising white cell count. On work up he was found to have progressed to accelerated phase of CML on the basis of rapidly rising WCC and clonal evolution with new cytogenetic finding of 3q deletion in Philadelphia positive cells. He was treated with high dose Cytarabine with complete resolution of proptosis and hematologic remission.