PROGNOSTIC FACTORS AND TREATMENT OUTCOME OF PEDIATRIC ANAPLASTIC LARGE CELL LYMPHOMA TREATED AT THE CHILDREN CANCER HOSPITAL EGYPT
(Abstract release date: 05/19/16)
EHA Library. Abdelrahman H. 06/09/16; 134644; PB1744
Hany Abdelrahman
Contributions
Contributions
Abstract
Abstract: PB1744
Type: Publication Only
Background
Anaplastic large cell lymphoma (ALCL) belongs to the group of high-grade non-Hodgkin’s lymphomas (NHLs) and typically presents as an aggressive systemic disease, with or without extranodal involvement. ALCL cells are characterized by the expression of the CD30/Ki-1 and more often by a T-cell phenotype. Frequently; ALCL is associated with the t(2;5)(p23;q35) chromosomal translocation, which gives rise to the fusion gene NPM–ALK. Recent data suggest that t(2;5) positive ALCL respond better to therapy and have a higher survival rate than translocation negative ALCL, thus implying that NPM–ALK expression may have a significant prognostic impact
Aims
The aim of the current study is to report the clinico-epinediologic data, prognostic factors and treatment outcome of pediatric ALCL treated at the Children Cancer Hospital Egypt during 8 years period.
Methods
A retrospective study including all patients diagnosed and treated as ALCL from july 2007 till July 2014 at CCHE. The diagnosis of ALCL was based on established morphologic and immunohistochemical criteria. ALK1 antibody directed to the NPM/ALK protein was used to detect the t(2;5) translocation.
Results
Forty-three patients were enrolled in our study, forming 5.4% of all NHL patients treated at CCHE within this period. They were 26 males (60.5%), and 17 (39.5%) females. Mean age was 11.5 years, median 11.7, range (3.7 to 17.2 years).The most common tumor primary site was generalized lymphadenopathy (62%).ALK status was available in 65% of the cohort (28 patients), of which 75% (21 patients) was positive and 25% negative. Bone marrow was free in all patients, while 5 patients (11.6%) had CNS involvement. Stage II and III were 37.2% each (16 patients), while stage IV was 14% (6 patients). Evaluation of tumor response post induction showed 25 (58%) of patients in complete remission (CR), 15 (34.9%) had partial remission (PR), 2 (4.7%) were progressive (PD), and 1 (2.4%) stationary disease (SD), while at the end of treatment 83.7% were in CR, 11.6% PD and 4.7% relapsed. At the end of the study period, 81.4% (36 patients) were alive, and (8 patients) 18.6% died. The mean FU period was 50months (range 12-96). The 4 years OS and EFS is 80.9% and 71.1% respectively.
Conclusion
Results of treatment of ALCL in our center are comparable to most of the reported studies. Salvage for relapsing and progressing patients is difficult, and the outcome is extremely poor, hence the need to identify biologic or clinical prognostic factors including minimal residual tumor to better evaluate chemotherapy response.
Session topic: E-poster
Keyword(s): Anaplastic large cell lymphoma, Pediatric
Type: Publication Only
Background
Anaplastic large cell lymphoma (ALCL) belongs to the group of high-grade non-Hodgkin’s lymphomas (NHLs) and typically presents as an aggressive systemic disease, with or without extranodal involvement. ALCL cells are characterized by the expression of the CD30/Ki-1 and more often by a T-cell phenotype. Frequently; ALCL is associated with the t(2;5)(p23;q35) chromosomal translocation, which gives rise to the fusion gene NPM–ALK. Recent data suggest that t(2;5) positive ALCL respond better to therapy and have a higher survival rate than translocation negative ALCL, thus implying that NPM–ALK expression may have a significant prognostic impact
Aims
The aim of the current study is to report the clinico-epinediologic data, prognostic factors and treatment outcome of pediatric ALCL treated at the Children Cancer Hospital Egypt during 8 years period.
Methods
A retrospective study including all patients diagnosed and treated as ALCL from july 2007 till July 2014 at CCHE. The diagnosis of ALCL was based on established morphologic and immunohistochemical criteria. ALK1 antibody directed to the NPM/ALK protein was used to detect the t(2;5) translocation.
Results
Forty-three patients were enrolled in our study, forming 5.4% of all NHL patients treated at CCHE within this period. They were 26 males (60.5%), and 17 (39.5%) females. Mean age was 11.5 years, median 11.7, range (3.7 to 17.2 years).The most common tumor primary site was generalized lymphadenopathy (62%).ALK status was available in 65% of the cohort (28 patients), of which 75% (21 patients) was positive and 25% negative. Bone marrow was free in all patients, while 5 patients (11.6%) had CNS involvement. Stage II and III were 37.2% each (16 patients), while stage IV was 14% (6 patients). Evaluation of tumor response post induction showed 25 (58%) of patients in complete remission (CR), 15 (34.9%) had partial remission (PR), 2 (4.7%) were progressive (PD), and 1 (2.4%) stationary disease (SD), while at the end of treatment 83.7% were in CR, 11.6% PD and 4.7% relapsed. At the end of the study period, 81.4% (36 patients) were alive, and (8 patients) 18.6% died. The mean FU period was 50months (range 12-96). The 4 years OS and EFS is 80.9% and 71.1% respectively.
Conclusion
Results of treatment of ALCL in our center are comparable to most of the reported studies. Salvage for relapsing and progressing patients is difficult, and the outcome is extremely poor, hence the need to identify biologic or clinical prognostic factors including minimal residual tumor to better evaluate chemotherapy response.
Session topic: E-poster
Keyword(s): Anaplastic large cell lymphoma, Pediatric
Abstract: PB1744
Type: Publication Only
Background
Anaplastic large cell lymphoma (ALCL) belongs to the group of high-grade non-Hodgkin’s lymphomas (NHLs) and typically presents as an aggressive systemic disease, with or without extranodal involvement. ALCL cells are characterized by the expression of the CD30/Ki-1 and more often by a T-cell phenotype. Frequently; ALCL is associated with the t(2;5)(p23;q35) chromosomal translocation, which gives rise to the fusion gene NPM–ALK. Recent data suggest that t(2;5) positive ALCL respond better to therapy and have a higher survival rate than translocation negative ALCL, thus implying that NPM–ALK expression may have a significant prognostic impact
Aims
The aim of the current study is to report the clinico-epinediologic data, prognostic factors and treatment outcome of pediatric ALCL treated at the Children Cancer Hospital Egypt during 8 years period.
Methods
A retrospective study including all patients diagnosed and treated as ALCL from july 2007 till July 2014 at CCHE. The diagnosis of ALCL was based on established morphologic and immunohistochemical criteria. ALK1 antibody directed to the NPM/ALK protein was used to detect the t(2;5) translocation.
Results
Forty-three patients were enrolled in our study, forming 5.4% of all NHL patients treated at CCHE within this period. They were 26 males (60.5%), and 17 (39.5%) females. Mean age was 11.5 years, median 11.7, range (3.7 to 17.2 years).The most common tumor primary site was generalized lymphadenopathy (62%).ALK status was available in 65% of the cohort (28 patients), of which 75% (21 patients) was positive and 25% negative. Bone marrow was free in all patients, while 5 patients (11.6%) had CNS involvement. Stage II and III were 37.2% each (16 patients), while stage IV was 14% (6 patients). Evaluation of tumor response post induction showed 25 (58%) of patients in complete remission (CR), 15 (34.9%) had partial remission (PR), 2 (4.7%) were progressive (PD), and 1 (2.4%) stationary disease (SD), while at the end of treatment 83.7% were in CR, 11.6% PD and 4.7% relapsed. At the end of the study period, 81.4% (36 patients) were alive, and (8 patients) 18.6% died. The mean FU period was 50months (range 12-96). The 4 years OS and EFS is 80.9% and 71.1% respectively.
Conclusion
Results of treatment of ALCL in our center are comparable to most of the reported studies. Salvage for relapsing and progressing patients is difficult, and the outcome is extremely poor, hence the need to identify biologic or clinical prognostic factors including minimal residual tumor to better evaluate chemotherapy response.
Session topic: E-poster
Keyword(s): Anaplastic large cell lymphoma, Pediatric
Type: Publication Only
Background
Anaplastic large cell lymphoma (ALCL) belongs to the group of high-grade non-Hodgkin’s lymphomas (NHLs) and typically presents as an aggressive systemic disease, with or without extranodal involvement. ALCL cells are characterized by the expression of the CD30/Ki-1 and more often by a T-cell phenotype. Frequently; ALCL is associated with the t(2;5)(p23;q35) chromosomal translocation, which gives rise to the fusion gene NPM–ALK. Recent data suggest that t(2;5) positive ALCL respond better to therapy and have a higher survival rate than translocation negative ALCL, thus implying that NPM–ALK expression may have a significant prognostic impact
Aims
The aim of the current study is to report the clinico-epinediologic data, prognostic factors and treatment outcome of pediatric ALCL treated at the Children Cancer Hospital Egypt during 8 years period.
Methods
A retrospective study including all patients diagnosed and treated as ALCL from july 2007 till July 2014 at CCHE. The diagnosis of ALCL was based on established morphologic and immunohistochemical criteria. ALK1 antibody directed to the NPM/ALK protein was used to detect the t(2;5) translocation.
Results
Forty-three patients were enrolled in our study, forming 5.4% of all NHL patients treated at CCHE within this period. They were 26 males (60.5%), and 17 (39.5%) females. Mean age was 11.5 years, median 11.7, range (3.7 to 17.2 years).The most common tumor primary site was generalized lymphadenopathy (62%).ALK status was available in 65% of the cohort (28 patients), of which 75% (21 patients) was positive and 25% negative. Bone marrow was free in all patients, while 5 patients (11.6%) had CNS involvement. Stage II and III were 37.2% each (16 patients), while stage IV was 14% (6 patients). Evaluation of tumor response post induction showed 25 (58%) of patients in complete remission (CR), 15 (34.9%) had partial remission (PR), 2 (4.7%) were progressive (PD), and 1 (2.4%) stationary disease (SD), while at the end of treatment 83.7% were in CR, 11.6% PD and 4.7% relapsed. At the end of the study period, 81.4% (36 patients) were alive, and (8 patients) 18.6% died. The mean FU period was 50months (range 12-96). The 4 years OS and EFS is 80.9% and 71.1% respectively.
Conclusion
Results of treatment of ALCL in our center are comparable to most of the reported studies. Salvage for relapsing and progressing patients is difficult, and the outcome is extremely poor, hence the need to identify biologic or clinical prognostic factors including minimal residual tumor to better evaluate chemotherapy response.
Session topic: E-poster
Keyword(s): Anaplastic large cell lymphoma, Pediatric
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