title: Blast transformation in chronic myelomonocytic leukemia: Risk factors, genetic features, survival, and treatment outcome.
abstract: Among 274 patients with chronic myelomonocytic leukemia (CMML) and followed for a median of 17.1 months, blast transformation (BT) occurred in 36 (13%). On multivariable analysis, risk factors for BT were presence of circulating blasts (HR 5.7; 95% CI 2.8-11.9) and female gender (HR 2.6; 95% CI 1.3-5.1); the results remained unchanged when analysis was restricted to CMML-1. ASXL1/SRSF2/SF3B1/U2AF1/SETBP1 mutational frequencies were not significantly different between time of CMML diagnosis and BT. Median survival post-BT was 4.7 months (5-year survival 6%) and better with allogeneic stem cell transplant (SCT) (14.3 months vs. 4.3 months for chemotherapy vs. 0.9 months for supportive care; P = 0.03). Neither karyotype nor mutational status was independently associated with risk of BT or post-BT survival. We conclude that female patients with CMML and those with circulating blasts are at a higher risk of BT. Post-BT survival is dismal and our observations suggest consideration of allogeneic SCT prior to BT.
authors: Division of Hematology, Mayo Clinic, Rochester, Minnesota.
authors: Mrinal M
authors: MM
authors: Patnaik
authors: Emnet A
authors: EA
authors: Wassie
authors: Terra L
authors: TL
authors: Lasho
authors: Curtis A
authors: CA
authors: Hanson
authors: Rhett
authors: R
authors: Ketterling
authors: Ayalew
authors: A
authors: Tefferi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D005260:Female; D015870:Gene Expression; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D059785:Karyotype; D015477:Leukemia, Myelomonocytic, Chronic / Q000188:drug therapy / Q000235:genetics / Q000401:mortality* / Q000473:pathology*; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D059645:Mutation Rate; D009363:Neoplasm Proteins; D011379:Prognosis; D012737:Sex Factors; D016019:Survival Analysis; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D009363:Neoplasm Proteins