Pretreatment characteristics and clinical outcome of acute promyelocytic leukaemia patients according to the PML-RAR alpha isoforms: a study of the PETHEMA group.
Of 167 newly diagnosed acute promyelocytic leukaemia patients, 83 patients were long (L)-form (50%), eight variable (V)-form (5%) and 76 short (S)-form (45%). The V-form and S-form groups presented a significantly higher percentage of patients with white blood cell counts > 10 x 10(9)/l (P < 0.05). The S-form cases displayed a significantly higher number of cases with M3v microgranular features (P = 0.005) and CD34 expression (P < 0.0001). There were no differences between the three isoforms in complete remission (CR) rate (overall CR 90%), but the 3-year disease-free survival was lower for V-form cases than it was for L- and S-form cases (62% vs. 94% and 89%, P = 0.056). We conclude that the V-form and S-form types are associated with some negative prognostic features at diagnosis. However, our data were only able to demonstrate an association with adverse prognosis in the V-form type and, moreover, as the number of cases was limited, needs to be confirmed in large, uniformly treated series.
González
M
M
Department of Haematology, Hospital Universitario, Salamanca, Spain. margondi@usal.es
Barragán
E
E
Bolufer
P
P
Chillón
C
C
Colomer
D
D
Borstein
R
R
Calasanz
M J
MJ
Gómez-Casares
M T
MT
Villegas
A
A
Marugán
I
I
Román
J
J
Martín
G
G
Rayón
C
C
Debén
G
G
Tormo
M
M
Díaz-Mediavilla
J
J
Esteve
J
J
González-San Miguel
J
J
Rivas
C
C
Pérez-Equiza
K
K
García-Sanz
R
R
Capote
F J
FJ
Ribera
J M
JM
Arias
J
J
León
A
A
Sanz
M A
MA
D000293:Adolescent; D000328:Adult; D000368:Aged; D018952:Antigens, CD34 / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015473:Leukemia, Promyelocytic, Acute / Q000188:drug therapy / Q000276:immunology / Q000378:metabolism*; D007958:Leukocyte Count; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000235:genetics*; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D016133:Polymerase Chain Reaction / Q000379:methods; D011379:Prognosis; D016016:Proportional Hazards Models; D020033:Protein Isoforms / Q000235:genetics; D016896:Treatment Outcome; D014212:Tretinoin / Q000627:therapeutic use
D018952:Antigens, CD34; D009363:Neoplasm Proteins; D015514:Oncogene Proteins, Fusion; D020033:Protein Isoforms; C093476:promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein; D014212:Tretinoin