title: Central nervous system relapse in CD56+, FLT3/ITD+ promyelocytic leukemia.
abstract: Central nervous system (CNS) involvement in acute promyelocytic leukemia (APL) is rare and tends to be seen mostly following treatment with all-trans retinoic acid (ATRA), due to prolonged patient survival and poor penetration of the drug in the CNS. At least 10% of extramedullary relapses in APL involve the CNS, and associated factors include an increased age, the BCR isoform, the development of differentiation syndrome, a high white cell count at presentation and hemorrhage into the CNS during induction therapy. We present the case of a patient with high-risk APL, CD56+, CD2+ in whom a CNS relapse was diagnosed through the presence of a PML/RARα rearrangement on PCR of the cerebrospinal fluid (CSF).
authors.affiliation: Institute of Hematology, Clinical Center for Serbia, Koste Todorovića 2, 11000 Belgrade, Serbia. natasacolovic73@gmail.com
authors.forename: N
authors.initials: N
authors.lastname: Colovic
authors.forename: D
authors.initials: D
authors.lastname: Tomin
authors.forename: A
authors.initials: A
authors.lastname: Vidovic
authors.forename: N
authors.initials: N
authors.lastname: Tosic
authors.forename: H D
authors.initials: HD
authors.lastname: Atkinson
authors.forename: Milica D
authors.initials: MD
authors.lastname: Colovic
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D019002:CD56 Antigen / Q000235:genetics / Q000378:metabolism; D016543:Central Nervous System Neoplasms / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000188:drug therapy; D017809:Fatal Outcome; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000134:cerebrospinal fluid / Q000188:drug therapy / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000134:cerebrospinal fluid* / Q000235:genetics; D016133:Polymerase Chain Reaction; D014212:Tretinoin; D051941:fms-Like Tyrosine Kinase 3 / Q000235:genetics / Q000378:metabolism
chemical_list: D000970:Antineoplastic Agents; D019002:CD56 Antigen; D015514:Oncogene Proteins, Fusion; C093476:promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein; D014212:Tretinoin; C497970:FLT3 protein, human; D051941:fms-Like Tyrosine Kinase 3