Clinicobiological features and outcome of acute promyelocytic leukemia occurring as a second tumor: the GIMEMA experience.
We analyzed the clinicobiological features and treatment outcome of a series of acute promyelocytic leukemias (APLs) occurring as a second tumor (APL-st's, n = 51) and compared these with a large group of de novo APL cases (n = 641), both observed by the Italian cooperative group GIMEMA. In the APL-st group, 37 patients had received radiotherapy and/or chemotherapy for their primary malignancy (PM), while 14 had been treated by surgery alone. Compared with de novo APL patients, APL-st patients were characterized by a predominance of females (P <.003), higher median age (P <.05), and worse performance status (P <.005). The median time elapsed between PM and APL-st was 36 months, with a longer latency for patients treated with surgery alone. No significant differences were found with regard to karyotypic lesions or type of promyelocytic leukemia/retinoic acid receptor alpha (PML/RARalpha) fusion in the 2 cohorts. A high prevalence of PMs of the reproductive system was observed among the female APL-st population (24 [71%] of 34 patients in this group had suffered from breast, uterine, or ovarian cancer). Thirty-one APL-st and 641 de novo APL patients received homogeneous APL therapy according to the all-trans retinoic acid (ATRA) and idarubicin regimen (the AIDA regimen). The complete remission (CR), 4-year event-free survival (EFS), and 4-year overall survival (OS) rates were 97% and 93%, 65% and 68%, and 85% and 78% in the APL-st and de novo APL groups, respectively. In spite of important clinical differences (older age and poorer performance status), the APL-st group responded as well as the de novo APL group to upfront ATRA plus chemotherapy, probably reflecting genetic similarity.
Cellular Biotechnology and Hematology Department, La Sapienza University, Via Benevento 6, 00161 Rome, Italy. pulsoni@bce.uniroma1.it
Alessandro
A
Pulsoni
Livio
L
Pagano
Francesco
F
Lo Coco
Giuseppe
G
Avvisati
Luca
L
Mele
Eros
E
Di Bona
Rosangela
R
Invernizzi
Franco
F
Leoni
Filippo
F
Marmont
Alfonso
A
Mele
Lorella
L
Melillo
Anna Maria
AM
Nosari
Enrico Maria
EM
Pogliani
Marco
M
Vignetti
Giuseppe
G
Visani
Vittorina
V
Zagonel
Giuseppe
G
Leone
Franco
F
Mandelli
D000293:Adolescent; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015255:Idarubicin / Q000627:therapeutic use; D015994:Incidence; D007223:Infant; D015473:Leukemia, Promyelocytic, Acute / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome; D014212:Tretinoin / Q000627:therapeutic use
D000970:Antineoplastic Agents; D014212:Tretinoin; D015255:Idarubicin