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Prepublished online as a Blood First Edition Paper on May 17, 2002; DOI 10.1182/blood-2001-12-0312.
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Blood, 15 September 2002, Vol. 100, No. 6, pp. 1972-1976
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Clinicobiological 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 Maria Nosari,
Enrico Maria Pogliani,
Marco Vignetti,
Giuseppe Visani,
Vittorina Zagonel,
Giuseppe Leone, and
Franco Mandelli
From the Cellular Biotechnology and Hematology
Department, "La Sapienza" University, the Hematology Department,
Catholic University, and the Epidemiology and Biostatistic Laboratory,
Istituto Superiore di Sanità, Rome, Italy; the Hematology
Department, San Bortolo Hospital, Vicenza, Italy; the Clinica Medica 2, Policlinico San Matteo, Pavia, Italy; the Hematology Department,
Policlinico di Careggi, Firenze, Italy; Haematology Department
"Molinette," San Giovanni Battista Hospital, Torino, Italy; the
Hematology Department, "Casa sollievo della sofferenza" Hospital,
San Giovanni Rotondo, Italy; the "Talamona" Hematology Department,
Niguarda "Ca' Granda" Hospital, Milano, Italy; the Hematology
Department, "San Gerardo" Hospital, Monza, Italy; the
"Seragnoli" Hematology Department, Policlinico "San Orsola,"
Bologna, Italy; and the Centro di Riferimento Oncologico, Aviano,
Italy.
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  (PML/RAR)
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.
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