Clinical and biologic data in thePLZF-RARα-positive patients
| Case4-150 (country) . | Sex/ Age . | WBC, ×109/L . | DIC . | Karyotype . | PLZF-RARα(ZF)/ RARαPLZF4-151 . | ATRA treatment . | Chemotherapy (protocol)‡ . | CR1, mo . | Current status (from time of diagnosis) . | References . |
|---|---|---|---|---|---|---|---|---|---|---|
| 434-153,4-155(France) | M/32 | 11.6 | + | 45,X,−Y,t(11;17) (q23;q21) | +(2ZF)/+ | D1-5 | (APL9362) | +36 | Alive in clinical and molecular CR1 at 37 mo (allo-BMT at 5 mo) | New case |
| 444-153,4-155(France) | M/34 | 2.4 | + | 45,X,−Y,add(2)(q33), t(11;17)(q23;q21)/46,XY | +(2ZF)/+ | D1-31 | Dauno/Ara-C (APL9362), Amsa/Ara-C as 2nd line to achieve 1st CR | 47 | Dead at 56 mo in relapse | Licht et al, 1995 (case 5)37; Koken et al, 199958 |
| 454-153(Belgium) | M/68 | 6.9 | + | 46,XY,t(11;17)(q23;q21)/ 47,idem,+8 | +(2ZF)/+ | D60-75 D150-240 | Dauno D1-3/Ara-C D1-7, Mitox/Ara-C as 2nd line at D240 to achieve 1st CR | 2 | Dead at 15 mo in relapse | Guidez et al, 199459; Licht et al, 1995 (case 2)37 |
| 464-155(UK) | M/53 | 4.5 | ++ | 46,XY,t(11;17)(q23;q21)/ 46,XY | +(2ZF)/+ | D1-31 | Dauno/Ara-C/Eto/G-CSF, 3 consolidation courses (MRC AML 1211) | +42 | Alive in CR1 at 43 mo | Grimwade et al, 1997 (case 7)38; Culligan et al, 199860 |
| 47 (USA) | F/37 | 45.2 | + | 46,XX,t(11;17)(q23;q21) | +(2ZF)/ND | No | (SWOG 860037) | 3 | CR2, lost to follow-up and dead at 11 mo | Scott et al, 1994 (case 16)61; Licht et al, 1995 (case 4)37 |
| 48 (USA) | M/81 | 7.6 | + | 46,XY,t(11;17)(q23;q21) | +(3ZF)/ND | D1-18 | No | No | Dead at day 18 (brain stem hemorrhage) | Licht et al, 1995 (case 3)37 |
| 49 (Italy) | M/43 | 10.4 | + | 46,XY,i(7)(q10),t(11;17) (q23;q21) | +(2ZF)/ND | D1-46 | (AIDA 049363) | 15 | Dead in 2nd relapse at 30 mo (auto-BMT at 23 mo) | New case |
| 504-153,4-155(Italy) | M/34 | 20.0 | − | 46,XY,del(11)(q23)/45, idem,−Y/46,XY | +/ND | D51-60 | Dauno/Ara-C/Eto (EORTC GIMEMA AML 1064), Ara-C/Ida/ATRA as 2nd line, CR1 obtained after HU | +28 | Alive in CR1 at 33 mo (allo-BMT in CR1 at 5 mo) | New case |
| 514-153,4-155(Netherlands) | M/30 | 69.5 | + | 46,XY,t(11;17)(q23;q21) | +(2ZF)/+ | D1-7 | HOVON 29,46 CR2 obtained with ATRA/G-CSF,46consolidation with HIDAC | 11 | Alive in clinical and molecular CR2 at 51 mo (allo-BMT in CR2 at 23 mo) | Jansen et al, 199946 |
| 524-153,4-155(Italy) | M/62 | 9.9 | + | 46,XY.ish ins(11;17)(q23;q21q21) | +(3ZF)/− | No | Ida/Ara-C/Eto (EORTC GIMEMA AML 13), CR obtained after MICE, NOVIA consolidation | +13 | Alive in CR1 at 15 mo | New case |
| 53 (UK) | M/75 | 2.0 | ++ | 46,XY,t(11;17)(q23;q21)/ 46,idem,del(12)(p1?)/46, idem,−6,+r/46,XY | +(3ZF)/+ | D3-23 | DAT 2+7/DAT 2+7/MACE | +16 | Alive in CR1 at 17 mo | New case |
| Case4-150 (country) . | Sex/ Age . | WBC, ×109/L . | DIC . | Karyotype . | PLZF-RARα(ZF)/ RARαPLZF4-151 . | ATRA treatment . | Chemotherapy (protocol)‡ . | CR1, mo . | Current status (from time of diagnosis) . | References . |
|---|---|---|---|---|---|---|---|---|---|---|
| 434-153,4-155(France) | M/32 | 11.6 | + | 45,X,−Y,t(11;17) (q23;q21) | +(2ZF)/+ | D1-5 | (APL9362) | +36 | Alive in clinical and molecular CR1 at 37 mo (allo-BMT at 5 mo) | New case |
| 444-153,4-155(France) | M/34 | 2.4 | + | 45,X,−Y,add(2)(q33), t(11;17)(q23;q21)/46,XY | +(2ZF)/+ | D1-31 | Dauno/Ara-C (APL9362), Amsa/Ara-C as 2nd line to achieve 1st CR | 47 | Dead at 56 mo in relapse | Licht et al, 1995 (case 5)37; Koken et al, 199958 |
| 454-153(Belgium) | M/68 | 6.9 | + | 46,XY,t(11;17)(q23;q21)/ 47,idem,+8 | +(2ZF)/+ | D60-75 D150-240 | Dauno D1-3/Ara-C D1-7, Mitox/Ara-C as 2nd line at D240 to achieve 1st CR | 2 | Dead at 15 mo in relapse | Guidez et al, 199459; Licht et al, 1995 (case 2)37 |
| 464-155(UK) | M/53 | 4.5 | ++ | 46,XY,t(11;17)(q23;q21)/ 46,XY | +(2ZF)/+ | D1-31 | Dauno/Ara-C/Eto/G-CSF, 3 consolidation courses (MRC AML 1211) | +42 | Alive in CR1 at 43 mo | Grimwade et al, 1997 (case 7)38; Culligan et al, 199860 |
| 47 (USA) | F/37 | 45.2 | + | 46,XX,t(11;17)(q23;q21) | +(2ZF)/ND | No | (SWOG 860037) | 3 | CR2, lost to follow-up and dead at 11 mo | Scott et al, 1994 (case 16)61; Licht et al, 1995 (case 4)37 |
| 48 (USA) | M/81 | 7.6 | + | 46,XY,t(11;17)(q23;q21) | +(3ZF)/ND | D1-18 | No | No | Dead at day 18 (brain stem hemorrhage) | Licht et al, 1995 (case 3)37 |
| 49 (Italy) | M/43 | 10.4 | + | 46,XY,i(7)(q10),t(11;17) (q23;q21) | +(2ZF)/ND | D1-46 | (AIDA 049363) | 15 | Dead in 2nd relapse at 30 mo (auto-BMT at 23 mo) | New case |
| 504-153,4-155(Italy) | M/34 | 20.0 | − | 46,XY,del(11)(q23)/45, idem,−Y/46,XY | +/ND | D51-60 | Dauno/Ara-C/Eto (EORTC GIMEMA AML 1064), Ara-C/Ida/ATRA as 2nd line, CR1 obtained after HU | +28 | Alive in CR1 at 33 mo (allo-BMT in CR1 at 5 mo) | New case |
| 514-153,4-155(Netherlands) | M/30 | 69.5 | + | 46,XY,t(11;17)(q23;q21) | +(2ZF)/+ | D1-7 | HOVON 29,46 CR2 obtained with ATRA/G-CSF,46consolidation with HIDAC | 11 | Alive in clinical and molecular CR2 at 51 mo (allo-BMT in CR2 at 23 mo) | Jansen et al, 199946 |
| 524-153,4-155(Italy) | M/62 | 9.9 | + | 46,XY.ish ins(11;17)(q23;q21q21) | +(3ZF)/− | No | Ida/Ara-C/Eto (EORTC GIMEMA AML 13), CR obtained after MICE, NOVIA consolidation | +13 | Alive in CR1 at 15 mo | New case |
| 53 (UK) | M/75 | 2.0 | ++ | 46,XY,t(11;17)(q23;q21)/ 46,idem,del(12)(p1?)/46, idem,−6,+r/46,XY | +(3ZF)/+ | D3-23 | DAT 2+7/DAT 2+7/MACE | +16 | Alive in CR1 at 17 mo | New case |
D indicates day. DIC indicates disseminated intravascular coagulation: −, none; +, moderate; ++, severe; ish, in situ hybridization.
Only cases 44 and 46 could be classified as classic M3 and basophilic M3, respectively; all cases could be classified as M3r according to the accompanying manuscript by Sainty et al.1
Breakpoints in PLZF intron 2 lead to the retention of 2 zinc fingers (2ZF) in the PLZF-RARa fusion, whereas breakpoints in intron 3 lead to retention of 3 zinc fingers (3ZF); introns numbered according to reference 47.
Dauno indicates daunorubicin; Ara-C, cytosine arabinoside; Amsa, m-amsacrine; Mitox, mitoxantrone; Eto, etoposide; G-CSF, granulocyte colony-stimulating factor; ida, idarubicin; HU, hydroxyurea.
No terminal granulocytic morphologic differentiation with in vitro ATRA alone.
Wild-type pattern with PML immunofluorescence.