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MULTICENTER RETROSPECTIVE ANALYSIS OF CLINICAL OUTCOME OF ADULT PATIENS WITH MIXED-PHENOTYPE ACUTE LEUKEMIA (MPAL) DIAGNOSED AND TREATED IN THE LAST TEN YEARS. A CAMPUS-ALL STUDY.
Author(s): ,
Davide Lazzarotto
Affiliations:
Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,Italie;Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,Italien;Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,Italia;Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,Italy;Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,It
,
Ilaria Tanasi
Affiliations:
Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italie;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italien;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italia;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italy;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona
,
Antonella Vitale
Affiliations:
Ematologia, Dipartimento di Medicina Traslazionale e di Precisione,"Sapienza" Università di Roma,Roma,Italie;Ematologia, Dipartimento di Medicina Traslazionale e di Precisione,"Sapienza" Università di Roma,Roma,Italien;Ematologia, Dipartimento di Medicina Traslazionale e di Precisione,"Sapienza" Università di Roma,Roma,Italia;Ematologia, Dipartimento di Medicina Traslazionale e di Precisione,"Sapi
,
Matteo Piccini
Affiliations:
SOD Ematologica,AOU Careggi,Firenze,Italie;SOD Ematologica,AOU Careggi,Firenze,Italien;SOD Ematologica,AOU Careggi,Firenze,Italia;SOD Ematologica,AOU Careggi,Firenze,Italy;SOD Ematologica,AOU Careggi,Firenze,Italia;SOD Ematologica,AOU Careggi,Firenze,Italië;SOD Ematologica,AOU Careggi,Firenze,Itália;SOD Ematologica,AOU Careggi,Firenze,Италия;SOD Ematologica,AOU Careggi,Firenze,Italien
,
Michelina Dargenio
Affiliations:
S.C. Ematologia,Ospedale Vito Fazzi,Lecce,Italie;S.C. Ematologia,Ospedale Vito Fazzi,Lecce,Italien;S.C. Ematologia,Ospedale Vito Fazzi,Lecce,Italia;S.C. Ematologia,Ospedale Vito Fazzi,Lecce,Italy;S.C. Ematologia,Ospedale Vito Fazzi,Lecce,Italia;S.C. Ematologia,Ospedale Vito Fazzi,Lecce,Italië;S.C. Ematologia,Ospedale Vito Fazzi,Lecce,Itália;S.C. Ematologia,Ospedale Vito Fazzi,Lecce,Италия;S.C. Ema
,
Fabio Giglio
Affiliations:
Unità di Ematologia e Trapianto di Midollo Osseo,IRCCS Ospedale San Raffaele,Milano,Italie;Unità di Ematologia e Trapianto di Midollo Osseo,IRCCS Ospedale San Raffaele,Milano,Italien;Unità di Ematologia e Trapianto di Midollo Osseo,IRCCS Ospedale San Raffaele,Milano,Italia;Unità di Ematologia e Trapianto di Midollo Osseo,IRCCS Ospedale San Raffaele,Milano,Italy;Unità di Ematologia e Trapianto di M
,
Fabio Forghieri
Affiliations:
S.C. Ematologia,Azienda Ospedaliero Universitaria di Modena,Modena,Italie;S.C. Ematologia,Azienda Ospedaliero Universitaria di Modena,Modena,Italien;S.C. Ematologia,Azienda Ospedaliero Universitaria di Modena,Modena,Italia;S.C. Ematologia,Azienda Ospedaliero Universitaria di Modena,Modena,Italy;S.C. Ematologia,Azienda Ospedaliero Universitaria di Modena,Modena,Italia;S.C. Ematologia,Azienda Ospeda
,
Nicola Fracchiolla
Affiliations:
U.O. Ematologia,IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano,Milano,Italie;U.O. Ematologia,IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano,Milano,Italien;U.O. Ematologia,IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano,Milano,Italia;U.O. Ematologia,IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano,Milano,Italy;U.O. Ematologia,IRCCS Ca' Granda Ospedale Maggiore P
,
Marco Cerrano
Affiliations:
S.C. Ematologia 2,AO Città della Salute e della Scienza,Torino,Italie;S.C. Ematologia 2,AO Città della Salute e della Scienza,Torino,Italien;S.C. Ematologia 2,AO Città della Salute e della Scienza,Torino,Italia;S.C. Ematologia 2,AO Città della Salute e della Scienza,Torino,Italy;S.C. Ematologia 2,AO Città della Salute e della Scienza,Torino,Italia;S.C. Ematologia 2,AO Città della Salute e della Sc
,
Elisabetta Todisco
Affiliations:
Divisione di Ematoncologia,Istituto Europeo di Oncologia,Milano,Italie;Divisione di Ematoncologia,Istituto Europeo di Oncologia,Milano,Italien;Divisione di Ematoncologia,Istituto Europeo di Oncologia,Milano,Italia;Divisione di Ematoncologia,Istituto Europeo di Oncologia,Milano,Italy;Divisione di Ematoncologia,Istituto Europeo di Oncologia,Milano,Italia;Divisione di Ematoncologia,Istituto Europeo d
,
Cristina Papayannidis
Affiliations:
Dipartimento di Oncologia e Ematologia,Policlinico S. Orsola-Malpighi,Bologna,Italie;Dipartimento di Oncologia e Ematologia,Policlinico S. Orsola-Malpighi,Bologna,Italien;Dipartimento di Oncologia e Ematologia,Policlinico S. Orsola-Malpighi,Bologna,Italia;Dipartimento di Oncologia e Ematologia,Policlinico S. Orsola-Malpighi,Bologna,Italy;Dipartimento di Oncologia e Ematologia,Policlinico S. Orsola
,
Matteo Leoncin
Affiliations:
U.O. di Ematologia,Ospedale dell'Angelo,Mestre,Italie;U.O. di Ematologia,Ospedale dell'Angelo,Mestre,Italien;U.O. di Ematologia,Ospedale dell'Angelo,Mestre,Italia;U.O. di Ematologia,Ospedale dell'Angelo,Mestre,Italy;U.O. di Ematologia,Ospedale dell'Angelo,Mestre,Italia;U.O. di Ematologia,Ospedale dell'Angelo,Mestre,Italië;U.O. di Ematologia,Ospedale dell'Angelo,Mestre,Itália;U.O. di Ematologia,Osp
,
Marzia Defina
Affiliations:
UOC Ematologia,Azienda Ospedaliero Universitaria Senese,Siena,Italie;UOC Ematologia,Azienda Ospedaliero Universitaria Senese,Siena,Italien;UOC Ematologia,Azienda Ospedaliero Universitaria Senese,Siena,Italia;UOC Ematologia,Azienda Ospedaliero Universitaria Senese,Siena,Italy;UOC Ematologia,Azienda Ospedaliero Universitaria Senese,Siena,Italia;UOC Ematologia,Azienda Ospedaliero Universitaria Senese
,
Fabio Guolo
Affiliations:
Ematologia e Terapie Cellulari,IRCCS Policlinico San Martino,Genova,Italie;Ematologia e Terapie Cellulari,IRCCS Policlinico San Martino,Genova,Italien;Ematologia e Terapie Cellulari,IRCCS Policlinico San Martino,Genova,Italia;Ematologia e Terapie Cellulari,IRCCS Policlinico San Martino,Genova,Italy;Ematologia e Terapie Cellulari,IRCCS Policlinico San Martino,Genova,Italia;Ematologia e Terapie Cell
,
Crescenza Pasciolla
Affiliations:
U.O. di Ematologia,IRCCS Istituto Tumori Giovanni Paolo II,Bari,Italie;U.O. di Ematologia,IRCCS Istituto Tumori Giovanni Paolo II,Bari,Italien;U.O. di Ematologia,IRCCS Istituto Tumori Giovanni Paolo II,Bari,Italia;U.O. di Ematologia,IRCCS Istituto Tumori Giovanni Paolo II,Bari,Italy;U.O. di Ematologia,IRCCS Istituto Tumori Giovanni Paolo II,Bari,Italia;U.O. di Ematologia,IRCCS Istituto Tumori Giov
,
Mario Delia
Affiliations:
U.O. Ematologia con Trapianto,Azienda Ospedaliero-Universitaria Consorziale, Policlinico di Bari,Bari,Italie;U.O. Ematologia con Trapianto,Azienda Ospedaliero-Universitaria Consorziale, Policlinico di Bari,Bari,Italien;U.O. Ematologia con Trapianto,Azienda Ospedaliero-Universitaria Consorziale, Policlinico di Bari,Bari,Italia;U.O. Ematologia con Trapianto,Azienda Ospedaliero-Universitaria Consorzi
,
Patrizia Chiusolo
Affiliations:
Servizio di Ematologia,Policlinico Gemelli,Roma,Italie;Servizio di Ematologia,Policlinico Gemelli,Roma,Italien;Servizio di Ematologia,Policlinico Gemelli,Roma,Italia;Servizio di Ematologia,Policlinico Gemelli,Roma,Italy;Servizio di Ematologia,Policlinico Gemelli,Roma,Italia;Servizio di Ematologia,Policlinico Gemelli,Roma,Italië;Servizio di Ematologia,Policlinico Gemelli,Roma,Itália;Servizio di Ema
,
Antonino Mulè
Affiliations:
Divisione di Ematologia ad indirizzo oncologico,A.O. Ospedali Riuniti Villa Sofia-Cervello,Palermo,Italie;Divisione di Ematologia ad indirizzo oncologico,A.O. Ospedali Riuniti Villa Sofia-Cervello,Palermo,Italien;Divisione di Ematologia ad indirizzo oncologico,A.O. Ospedali Riuniti Villa Sofia-Cervello,Palermo,Italia;Divisione di Ematologia ad indirizzo oncologico,A.O. Ospedali Riuniti Villa Sofia
,
Anna Candoni
Affiliations:
Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,Italie;Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,Italien;Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,Italia;Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,Italy;Clinica Ematologica,Azienda Sanitaria Universitaria Friuli Centrale,Udine,It
,
Massimiliano Bonifacio
Affiliations:
Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italie;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italien;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italia;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italy;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona
,
Giovanni Pizzolo
Affiliations:
Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italie;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italien;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italia;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona,Verona,Italy;Dipartimento di Medicina, Sezione di Ematologia,Università di Verona
Robin Foà
Affiliations:
Ematologia, Dipartimento di Medicina Traslazionale e di Precisione,"Sapienza" Università di Roma,Roma,Italie;Ematologia, Dipartimento di Medicina Traslazionale e di Precisione,"Sapienza" Università di Roma,Roma,Italien;Ematologia, Dipartimento di Medicina Traslazionale e di Precisione,"Sapienza" Università di Roma,Roma,Italia;Ematologia, Dipartimento di Medicina Traslazionale e di Precisione,"Sapi
(Abstract release date: 05/12/22) EHA Library. Lazzarotto D. 06/10/22; 357220; P357
Davide Lazzarotto
Davide Lazzarotto
Contributions
Abstract
Presentation during EHA2022: All (e)Poster presentations will be made available as of Friday, June 10, 2022 (09:00 CEST) and will be accessible for on-demand viewing until Monday, August 15, 2022 on the Congress platform.

Abstract: P357

Type: Poster presentation

Session title: Acute lymphoblastic leukemia - Clinical

Background
Mixed phenotype acute leukemia (MPAL) is a very rare disease in adults. Most data derive from small retrospective series, some including also pediatric patients. Generally, treatment is similar to that of acute lymphoblastic leukemia (ALL), but the outcomes in adults and the role of allogeneic stem cell transplantation (AlloSCT) are not well defined.

Aims
Purpose of this retrospective study was to provide data on a large cohort of adult patients diagnosed and treated in 18 Italian hematology centers in the last 10 years.

Methods
Seventy-seven adult patients diagnosed with MPAL according to the EGIL and WHO criteria in the last 10 years (2011-2021) and treated with curative intent were included in the study. Endpoints were the CR rate after induction, overall survival (OS), disease-free survival (DFS) and rate of AlloSCT.

Results

Forty-eight of the 77 (62%) patients had B/myeloid MPAL, 27 (35%) had T/myeloid MPAL and 2 (3%) had B/T/myeloid MPAL. Median age at diagnosis was 49 years (range 17 - 77); it was lower in patients with T/myeloid MPAL than in B/myeloid MPAL cases (39 vs 54 years, P=0.04). Extramedullary involvement was detected in 26/77 (34%) of patients (mainly lymph nodes and spleen), but none had a central nervous system involvement.

Cytogenetic analysis was normal in 40% of patients; 36% of cases had a complex karyotype, 24% had other cytogenetic abnormalities (including 8% with a BCR-ABL1 rearrangement). FLT-ITD mutation was detected in 14% of patients. No differences were found between B/myeloid and T/myeloid MPAL cases.

Thirty/77 (39%) of patients were treated with an acute myeloid leukemia (AML)-like induction regimen, while 47 (61%) were treated with an ALL-like induction. Globally, CR rate after induction was 62% and 42% of these cases were also MRD negative. Patients treated with an AML-like induction had a higher rate of refractory disease after induction (33% vs 10%, P=0.02). The death during induction (DDI) rate was similar in the 2 groups (7% vs 8%). In univariate analysis an ALL-like therapy was the only variable associated with a better CR rate (P=0.0447). Patients refractory to AML-like induction were mainly salvaged with a subsequent AlloSCT or ALL-like therapy followed by an AlloSCT, and 60% of them achieved a CR.

The median OS and DFS of the entire cohort were respectively 41.9 and 37.6 months, with an OS and DFS at 5 years of 43% and 39%, respectively. Age, type of induction therapy, karyotype, presence of FLT3-ITD mutation, extramedullary involvement and type of MPAL (B/myeloid vs T/myeloid) were tested in univariate analysis for OS and DFS. Age below 60 years was the only variable associated with a better OS (median OS of 67 months vs 26 months, P=0.0138), while no variable impacted on DFS.

AlloSCT was performed in 50 patients (65%), 80% of them (40 patients) as part of the frontline treatment (36/40 patients transplanted in CR1). Among transplanted patients, we observed a 5-year OS of 54% (median not reached), that improved to 69% in patients transplanted as part of the frontline treatment.

Conclusion
This study describes one of the largest cohorts of adult patients with MPAL. These data outline that this disease responds better to ALL-like induction therapy than to AML-like therapy and that consolidation therapy should include, whenever possible, an AlloSCT. Prospective studies are needed to uniform the therapeutic approach and to identify variables capable of predicting outcome and to guide therapy.

Keyword(s): Allogeneic hematopoietic stem cell transplant, Mixed lineage leukemia

Presentation during EHA2022: All (e)Poster presentations will be made available as of Friday, June 10, 2022 (09:00 CEST) and will be accessible for on-demand viewing until Monday, August 15, 2022 on the Congress platform.

Abstract: P357

Type: Poster presentation

Session title: Acute lymphoblastic leukemia - Clinical

Background
Mixed phenotype acute leukemia (MPAL) is a very rare disease in adults. Most data derive from small retrospective series, some including also pediatric patients. Generally, treatment is similar to that of acute lymphoblastic leukemia (ALL), but the outcomes in adults and the role of allogeneic stem cell transplantation (AlloSCT) are not well defined.

Aims
Purpose of this retrospective study was to provide data on a large cohort of adult patients diagnosed and treated in 18 Italian hematology centers in the last 10 years.

Methods
Seventy-seven adult patients diagnosed with MPAL according to the EGIL and WHO criteria in the last 10 years (2011-2021) and treated with curative intent were included in the study. Endpoints were the CR rate after induction, overall survival (OS), disease-free survival (DFS) and rate of AlloSCT.

Results

Forty-eight of the 77 (62%) patients had B/myeloid MPAL, 27 (35%) had T/myeloid MPAL and 2 (3%) had B/T/myeloid MPAL. Median age at diagnosis was 49 years (range 17 - 77); it was lower in patients with T/myeloid MPAL than in B/myeloid MPAL cases (39 vs 54 years, P=0.04). Extramedullary involvement was detected in 26/77 (34%) of patients (mainly lymph nodes and spleen), but none had a central nervous system involvement.

Cytogenetic analysis was normal in 40% of patients; 36% of cases had a complex karyotype, 24% had other cytogenetic abnormalities (including 8% with a BCR-ABL1 rearrangement). FLT-ITD mutation was detected in 14% of patients. No differences were found between B/myeloid and T/myeloid MPAL cases.

Thirty/77 (39%) of patients were treated with an acute myeloid leukemia (AML)-like induction regimen, while 47 (61%) were treated with an ALL-like induction. Globally, CR rate after induction was 62% and 42% of these cases were also MRD negative. Patients treated with an AML-like induction had a higher rate of refractory disease after induction (33% vs 10%, P=0.02). The death during induction (DDI) rate was similar in the 2 groups (7% vs 8%). In univariate analysis an ALL-like therapy was the only variable associated with a better CR rate (P=0.0447). Patients refractory to AML-like induction were mainly salvaged with a subsequent AlloSCT or ALL-like therapy followed by an AlloSCT, and 60% of them achieved a CR.

The median OS and DFS of the entire cohort were respectively 41.9 and 37.6 months, with an OS and DFS at 5 years of 43% and 39%, respectively. Age, type of induction therapy, karyotype, presence of FLT3-ITD mutation, extramedullary involvement and type of MPAL (B/myeloid vs T/myeloid) were tested in univariate analysis for OS and DFS. Age below 60 years was the only variable associated with a better OS (median OS of 67 months vs 26 months, P=0.0138), while no variable impacted on DFS.

AlloSCT was performed in 50 patients (65%), 80% of them (40 patients) as part of the frontline treatment (36/40 patients transplanted in CR1). Among transplanted patients, we observed a 5-year OS of 54% (median not reached), that improved to 69% in patients transplanted as part of the frontline treatment.

Conclusion
This study describes one of the largest cohorts of adult patients with MPAL. These data outline that this disease responds better to ALL-like induction therapy than to AML-like therapy and that consolidation therapy should include, whenever possible, an AlloSCT. Prospective studies are needed to uniform the therapeutic approach and to identify variables capable of predicting outcome and to guide therapy.

Keyword(s): Allogeneic hematopoietic stem cell transplant, Mixed lineage leukemia

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