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Blood, 15 August 2000, Vol. 96, No. 4, pp. 1287-1296
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
A new morphologic classification system for acute promyelocytic
leukemia distinguishes cases with underlying
PLZF/RARA gene rearrangements
Danielle Sainty,
Vincenzo Liso,
Angelo Cantù-Rajnoldi,
David Head,
Marie-Joëlle Mozziconacci,
Christine Arnoulet,
Laurence Benattar,
Susanna Fenu,
Marco Mancini,
Eliane Duchayne,
François-Xavier Mahon,
Norma Gutierrez,
Françoise Birg,
Andrea Biondi,
David Grimwade,
Marina Lafage-Pochitaloff,
Anne Hagemeijer, and
Georges Flandrin on behalf of Groupe Français
d'Hématologie Cellulaire, Groupe Français de
Cytogénétique Hématologique, UK Cancer
Cytogenetics Group, and BIOMED 1 European Community-Concerted Action
"Molecular Cytogenetic Diagnosis in Haematological Malignancies"
From the Institut Paoli-Calmettes,
Institut National de la Santé et de la Recherche Médicale
(INSERM) U119, IFR 57, and Université de la
Méditerranée, Marseille, France; Hôpital
Necker Enfants Malades, Paris, France; Laboratoire
d'Hématologie, Hôpital Purpan, Toulouse,
France; Laboratoire d'Hématologie et
d'Immunologie, Hôpital Pellegrin, Bordeaux, France;
Cattedra di Ematologica i Policlinico di Bari, Bari,
Italy; Dipartimento di Medicina di Laboratorio, Istituti
Clinici di Perfezionamento, Milan, Italy; Dipartimento di
Biotecnologie Cellulari ed Ematologia, Universita degli Studi "la
Sapienza," Rome, Italy; Centro di Ricerca; M
Tettamanti and Ospedale San Gerardo, Monza, Italy; St Jude
Children's Hospital, Memphis, TN; the Division of Medical and
Molecular Genetics, Guy's, King's and St Thomas' School of Medicine,
London, UK; Center for Human Genetics, Leuven,
Belgium; and Servicio de Hematologia, Hospital
Universitario de Salamanca, Centro de Investigation del Cancer,
Universidad de Salamanca, Salamanca, Spain.
Acute promyelocytic leukemia (APL) is typified by the t(15;17)
translocation, which leads to the formation of the
PML/RARA fusion gene and predicts a
beneficial response to retinoids. However, approximately 10% of all
APL cases lack the classic t(15;17). This group includes (1) cases with
cryptic PML/RARA gene rearrangements and t(5;17) that leads
to the NPM/RARA fusion gene,
which are retinoid-responsive, and (2) cases with t(11;17)(q23;q21)
that are associated with the PLZF/RARA fusion gene, which
are retinoid-resistant. A key issue is how to rapidly distinguish
subtypes of APL that demand distinct treatment approaches. To address
this issue, a European workshop was held in Monza, Italy,
during June 1997, and a morphologic, immunophenotypic, cytogenetic, and
molecular review was undertaken in 60 cases of APL lacking t(15;17).
This process led to the development of a novel morphologic
classification system that takes into account the major nuclear and
cytoplasmic features of APL. There were no major differences observed
in morphology or immunophenotype between cases with the classic
t(15;17) and those with the cryptic PML/RARA
gene rearrangements. Auer rods were absent in the t(5;17)
case expressing NPM/RARA. Interestingly, this
classification system distinguished 9 cases with
t(11;17)(q23;q21) and, in addition, successfully identified 2 cases lacking t(11;17), which were subsequently shown to have
underlying PLZF/RARA fusions. The PLZF/RARA
cases were characterized by a predominance of blasts with regular
nuclei, an increased number of Pelger-like cells, and by expression of
CD56 in 4 of 6 cases tested. Use of this classification system,
combined with an analysis for CD56 expression, should allow early
recognition of APL cases requiring tailored molecular investigations.
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