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Prepublished online as a Blood First Edition Paper on May 17, 2002; DOI 10.1182/blood-2001-12-0312.
Submitted December 21, 2001
Accepted April 18, 2002
CLINICO-BIOLOGICAL FEATURES AND OUTCOME OF ACUTE PROMYELOCYTIC LEUKEMIA OCCURRING AS A SECOND TUMOR. THE GIMEMA EXPERIENCE
Alessandro Pulsoni*, Livio Pagano, Francesco Lo-Coco, Giuseppe Avvisati, Luca Mele, Eros Di Bona, Rosangela Invernizzi, Franco Leoni, Filippo Marmont, Alfonso Mele, Lorella Melillo, Anna M Nosari, Enrico M Pogliani, Marco Vignetti, Giuseppe Visani, Vittorina Zagonel, Giuseppe Leone, and Franco Mandelli
Ematologia, Dipartimento di Biopatologia Umana, ''La Sapienza'', Rome, Italy
Ematologia, Universita Cattolica, Rome, Italy
Ematologia, Ospedale S. Bortolo, Vicenza, Italy
Cilinica Medica, Policlinico S. Matteo, Pavia, Italy
Ematologia, Policlinico di Careggi, Firenze, Italy
Ematologia ''Molinette'', Ospedale S.Giovanni Battista, Torino, Italy
Laboratorio di epidemiologia e biostatistica, Istituto Superiore di Sanita, Rome, Italy
Ematologia, Ospedale ''Casa sollievo della soffrenza'', S. Giovanni Rotondo, Italy
Ematologia ''Talamona'', Ospedale Niguarda ''Ca' Granda'', Milano, Italy
Ematologia, Ospedale ''S. Gerardo'', Monza, Italy
Ematologia ''Seragnoli'', Policlinico ''S.Orsola'', Bologna, Italy
Centro di riferimento oncologico, Aviano, Italy
* Corresponding author; email: pulsoni{at}bce.uniroma1.it.
We analyzed here the clinico-biological features and treatment outcome of a series of acute promyelocytic leukemias occurring as a second tumor (APL-st, n=51), and compared these with a large group of de novo APL cases (n=641), both observed by the Italian GIMEMA cooperative group. In the APL-st group, 37 pts had received radiotherapy and/or chemotherapy for their PM, while 14 had been treated by surgery alone. Compared with de novo APL, APL-st pts were characterized by female predominance (p <0.003), higher median age (p <0.05) and worst performance status (p <0.005). The median time elapsed between PM and sAPL was 36 months, with a longer latency for patients treated with surgery alone. No significant differences were found as regarding karyotypic lesions or type of PML/RAR fusion in the two cohorts. A high prevalence of PMs of the reproductive system was observed among the female APL-st population (24/34, or 71%, pts in this group had suffered from breast, uterus or ovary cancer). Thirty-one APL-st and 641 de novo APL pts received homogeneous APL therapy according to the AIDA (All-trans retinoic acid and idarubicin) regimen. In the two groups, the CR, 4-year EFS and 4-year OS rates were 97% and 93%, 65% and 68%, and 85% and 78%, respectively. In spite of important clinical differences (older age and poorer performance status), APL-st responds equally well to upfront ATRA plus chemotherapy as compared to de novo APL, probably reflecting genetic similarity.
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