Treatment outcomes following leukemic transformation in Philadelphia-negative myeloproliferative neoplasms.
Leukemic transformation (LT) is a rare but fatal complication of Philadelphia-negative myeloproliferative neoplasms (MPNs) for which optimal treatment strategies are not known. At our center, we have adopted a treatment approach for LT where patients within the transplant age group who have a reasonable fitness level are treated with curative intent and offered induction chemotherapy. Subsequently, those who respond and have a suitable donor are considered for allogeneic hematopoietic cell transplantation (HCT). In this study, we evaluated the clinical outcomes of this treatment approach in 75 patients with LT. The 2-year overall survival (OS) from the time of LT was 15%. A total of 39 patients (52%) were treated with curative intent (induction ± HCT) and had a 2-y OS of 26% compared with 3% in those noncuratively treated (P < .0001). In the curative intent group, 18 individuals (46%) achieved complete remission (CR) or CR with incomplete recovery and 12 (31%) reverted to a chronic MPN phase, with 17 patients undergoing HCT. Survival of patients posttransplant was significantly improved compared with those who responded to induction but were not transplanted (2-y OS of 47% vs 15%; P = .03). Thus, induction chemotherapy followed by HCT has the potential for long-term disease control in select patients with LT preceded by a MPN.
Department of Medicine, University Health Network, Toronto, Ontario, Canada.
James A
JA
Kennedy
Eshetu G
EG
Atenafu
Hans A
HA
Messner
Kenneth J
KJ
Craddock
Joseph M
JM
Brandwein
Jeffrey H
JH
Lipton
Mark D
MD
Minden
Aaron D
AD
Schimmer
Andre C
AC
Schuh
Karen W
KW
Yee
Vikas
V
Gupta
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use; D001374:Azacitidine / Q000031:analogs & derivatives / Q000627:therapeutic use; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D003131:Combined Modality Therapy; D003561:Cytarabine / Q000627:therapeutic use; D000077209:Decitabine; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000235:genetics / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D010677:Philadelphia Chromosome; D012042:Registries; D016019:Survival Analysis; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D055815:Young Adult
D000964:Antimetabolites, Antineoplastic; D003561:Cytarabine; D000077209:Decitabine; D001374:Azacitidine