ISSN 2997-1036
International Journal of Hematology | Vol. 6, No. 7, July 2015 | pp. 49–56
DOI: 10.46882/2015/IJH/000067
Case Report
Title: Extramedullary relapse of acute promyelocytic leukemia in the central nervous system following complete hematological remission
Names of Authors: K. L. Musa¹, M. N. Lawal², O. P. Dikko³
Authors’ Affiliations: ¹Department of Haematology, Federal Medical Centre, Katsina, Nigeria; ²Department of Oncology, Bayero University, Kano, Nigeria; ³Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
Abstract: Extramedullary relapse of acute promyelocytic leukemia is a rare complication following successful differentiation therapy using all-trans retinoic acid. We report a 28-year-old male who achieved sustained complete cytogenetic and hematological remission following induction and consolidation for acute promyelocytic leukemia harboring the t(15;17) PML-RARA rearrangement. Fourteen months post-consolidation, he presented with progressive severe headache, vomiting, and bilateral papilledema, without systemic symptoms. Full blood counts and bone marrow biopsy confirmed persistent marrow remission with normal hematopoietic lineages. However, lumbar puncture revealed elevated opening pressure and a leukocyte count of 140/μl. Cytocentrifuge examination of cerebrospinal fluid demonstrated numerous promyeloblasts with Auer rods. Molecular real-time PCR on cerebrospinal fluid confirmed the presence of the PML-RARA transcript, establishing a central nervous system relapse. Intrathecal chemotherapy using cytarabine and methotrexate was initiated immediately, combined with systemic arsenic trioxide. Complete clearance of blastic clones from the cerebrospinal fluid was achieved after 6 weekly cycles. This case demonstrates that the central nervous system can serve as a pharmacological sanctuary for promyelocytic clones, highlighting the need to investigate neurological symptoms even during complete marrow remission.
Keywords: Acute promyelocytic leukemia, extramedullary relapse, central nervous system, all-trans retinoic acid, cerebrospinal fluid
Manuscript Timeline: Received: April 18, 2015; Revised: May 22, 2015; Accepted: June 10, 2015; Published: July 16, 2015