Central Nervous System Relapse in Patients with Acute Promyelocytic Leukemia Treated with All-trans Retinoic Acid / 대한내과학회지

ABSTRACT

Acute promyelocytic leukemia (APL) is distinguished from other subtypes of acute myeloid leukemia (AML) by its distinctive morphology, specific chromosomal abnormality, coagulopathy, and unique response to treatment with all-trans retinoic acid (ATRA). Central nervous system (CNS) involvement is rare in APL. Most CNS relapses occur in patients with hyperleukocytosis at initial presentation, and the optimal management of such patients is still controversial. We report five patients with APL who had CNS relapse with or without evidence of cytological and molecular disease of the bone marrow after ATRA treatment. Brain magnetic resonance imaging revealed leptomeningeal infiltration and cerebrospinal fluid examination showed the presence of promyelocytes. Patients were treated with a combination of systemic chemotherapy and radiotherapy with or without intrathecal chemotherapy, and most were subsequently treated with arsenic trioxide (ATO) as maintenance therapy. Among these patients, one received allogeneic stem cell transplantation in second complete remission.