BACKGROUND: Knowledge concerning the clinical and biological presentation, as well as the outcome of treatment, of biphenotypic acute leukemia in children is limited. DESIGN AND METHODS: This retrospective review analyzes the clinical features and outcome of children with biphenotypic acute leukemia diagnosed and treated over an 8-year period. According to the EGIL scoring system 24 (3.7%) of 633 patients with acute leukemia were classified as having biphenotypic acute leukemia. The diagnostic work-up and results were reviewed specifically for this study in the light of the newly published WHO criteria for the diagnosis of leukemia of ambiguous lineage. Based on these criteria, 11 (1.7%) patients were categorized according to the new nomenclature as having mixed phenotype acute leukemia. The majority of the patients (58.3%) had a B-lymphoid/myeloid phenotype, followed by the T-lymphoid/myeloid phenotype. The most frequent chromosomal abnormality involved the 14q32 locus. Patients received therapy based on a treatment regimen for acute lymphocytic leukemia regimen, which included myeloid-effective agents. RESULTS: At a median follow up of 4 years (range, 6 month - 7 years) the overall survival rate was 75.7% and the event-free survival rate was 73.5%. The survival of those patients who underwent hematopoietic stem cell transplantation in first complete remission was not different from that of the patients who were treated with chemotherapy alone (overall survival: 70.1% versus 81.1%, respectively, p=0.39; event-free survival: 70.1% versus 76.2%, respectively, p=0.75). The outcome of the 11 patients who were retrospectively classified as having mixed phenotype acute leukemia according to the new WHO criteria was excellent, with no relapses or deaths occurring among these patients. CONCLUSIONS: An acute lymphocytic leukemia type of induction therapy, using agents that are active against lymphoid and myeloid leukemias, appears to be more effective in achieving and maintaining complete remissions regardless of whether the patients are classified according to EGIL criteria or the new WHO criteria. Hematopoietic stem cell transplantation may not be necessary for all patients in first complete remission.
BACKGROUND: Knowledge concerning the clinical and biological presentation, as well as the outcome of treatment, of biphenotypic acute leukemia in children is limited. DESIGN AND METHODS: This retrospective review analyzes the clinical features and outcome of children with biphenotypic acute leukemia diagnosed and treated over an 8-year period. According to the EGIL scoring system 24 (3.7%) of 633 patients with acute leukemia were classified as having biphenotypic acute leukemia. The diagnostic work-up and results were reviewed specifically for this study in the light of the newly published WHO criteria for the diagnosis of leukemia of ambiguous lineage. Based on these criteria, 11 (1.7%) patients were categorized according to the new nomenclature as having mixed phenotype acute leukemia. The majority of the patients (58.3%) had a B-lymphoid/myeloid phenotype, followed by the T-lymphoid/myeloid phenotype. The most frequent chromosomal abnormality involved the 14q32 locus. Patients received therapy based on a treatment regimen for acute lymphocytic leukemia regimen, which included myeloid-effective agents. RESULTS: At a median follow up of 4 years (range, 6 month - 7 years) the overall survival rate was 75.7% and the event-free survival rate was 73.5%. The survival of those patients who underwent hematopoietic stem cell transplantation in first complete remission was not different from that of the patients who were treated with chemotherapy alone (overall survival: 70.1% versus 81.1%, respectively, p=0.39; event-free survival: 70.1% versus 76.2%, respectively, p=0.75). The outcome of the 11 patients who were retrospectively classified as having mixed phenotype acute leukemia according to the new WHO criteria was excellent, with no relapses or deaths occurring among these patients. CONCLUSIONS: An acute lymphocytic leukemia type of induction therapy, using agents that are active against lymphoid and myeloid leukemias, appears to be more effective in achieving and maintaining complete remissions regardless of whether the patients are classified according to EGIL criteria or the new WHO criteria. Hematopoietic stem cell transplantation may not be necessary for all patients in first complete remission.
Authors: A Urbano-Ispizua; E Matutes; N Villamor; J M Ribera; E Feliu; E Montserrat; A Grañena; J L Vives-Corrons; C Rozman Journal: Br J Haematol Date: 1990-06 Impact factor: 6.998
Authors: F M Uckun; P Gaynon; H Sather; D Arthur; M Trigg; D Tubergen; J Nachman; P Steinherz; M G Sensel; G R Reaman Journal: Blood Date: 1997-04-01 Impact factor: 22.113
Authors: Ching-Hon Pui; John T Sandlund; Deqing Pei; Dario Campana; Gaston K Rivera; Raul C Ribeiro; Jeffrey E Rubnitz; Bassem I Razzouk; Scott C Howard; Melissa M Hudson; Cheng Cheng; Larry E Kun; Susana C Raimondi; Frederick G Behm; James R Downing; Mary V Relling; William E Evans Journal: Blood Date: 2004-07-13 Impact factor: 22.113
Authors: Jeong A Park; Thad T Ghim; Keun wook Bae; Ho Joon Im; Seong Soo Jang; Chan Jeong Park; Hyun Sook Chi; Jong Jin Seo Journal: Pediatr Blood Cancer Date: 2009-09 Impact factor: 3.167
Authors: Y Hayashi; C H Pui; F G Behm; A H Fuchs; S C Raimondi; G R Kitchingman; J Mirro; D L Williams Journal: Blood Date: 1990-07-01 Impact factor: 22.113
Authors: Wenbin Xiao; Maheetha Bharadwaj; Max Levine; Noushin Farnhoud; Friederike Pastore; Bartlomiej M Getta; Anne Hultquist; Christopher Famulare; Juan S Medina; Minal A Patel; Qi Gao; Natasha Lewis; Janine Pichardo; Jeeyeon Baik; Brian Shaffer; Sergio Giralt; Raajit Rampal; Sean Devlin; Robert Cimera; Yanming Zhang; Maria E Arcila; Elli Papaemmanuil; Ross L Levine; Mikhail Roshal Journal: Blood Adv Date: 2018-12-11
Authors: Brianna R Murphy; Michael Roth; E Anders Kolb; Todd Alonzo; Robert Gerbing; Robert J Wells Journal: Pediatr Blood Cancer Date: 2019-03-25 Impact factor: 3.167
Authors: Etan Orgel; Thomas B Alexander; Brent L Wood; Samir B Kahwash; Meenakshi Devidas; Yunfeng Dai; Todd A Alonzo; Charles G Mullighan; Hiroto Inaba; Stephen P Hunger; Elizabeth A Raetz; Alan S Gamis; Karen R Rabin; Andrew J Carroll; Nyla A Heerema; Jason N Berman; William G Woods; Mignon L Loh; Patrick A Zweidler-McKay; John T Horan Journal: Cancer Date: 2019-10-29 Impact factor: 6.860