BACKGROUND: Systemic anaplastic large cell lymphoma (ALCL) accounts for 2-8% of non-Hodgkin's lymphoma in adults and 10-15% in children. While there is ample data in the world literature about the clinical features and outcome of this disease, prognosis in Indian patients is largely unknown. OBJECTIVE: To study the clinical, pathologic profile and outcome ALCL. MATERIALS AND METHODS: Fifty patients who had pathologically proven diagnosis of systemic ALCL at our institute from June 2003 to May 2011 were included for retrospective analysis. This included 30 cases of anaplastic lymphoma kinase+ (ALK+), ALCL and 20 cases of anaplastic lymphoma kinase- (ALK-), ALCL. The hospital protocol for treatment of these patients included CHOP chemotherapy regimen in >15 years of age and MCP842 protocol with vinblastine for 1 year in <15 years of age. Event free survival was noted. These outcomes were correlated with ALK status, International Prognostic Index (IPI) score, and stage at presentation. RESULTS: At a median follow-up of 36 months (range: 6-72 months) ALK- ALCL had a poor outcome. The 3 year event free survival in pediatric ALCL was 66.7%. In adults, this was 60% ALK+ ALCL was 60% and 20% in ALK- ALCL. CONCLUSIONS: Systemic ALCL is an aggressive disease. CD3 + positivity is commonly seen in ALK- ALCL and ALK+, epithelial membrane antigen + positivity is seen in ALK+ ALCL. ALK- ALCL, advanced stage III, IV and high IPI score were associated with poor prognosis. The demographic profile and outcome in our study was similar to the world literature. With new drugs like crizotinib and brentuximab vedotin the future looks very promising.
BACKGROUND: Systemic anaplastic large cell lymphoma (ALCL) accounts for 2-8% of non-Hodgkin's lymphoma in adults and 10-15% in children. While there is ample data in the world literature about the clinical features and outcome of this disease, prognosis in Indian patients is largely unknown. OBJECTIVE: To study the clinical, pathologic profile and outcome ALCL. MATERIALS AND METHODS: Fifty patients who had pathologically proven diagnosis of systemic ALCL at our institute from June 2003 to May 2011 were included for retrospective analysis. This included 30 cases of anaplastic lymphoma kinase+ (ALK+), ALCL and 20 cases of anaplastic lymphoma kinase- (ALK-), ALCL. The hospital protocol for treatment of these patients included CHOP chemotherapy regimen in >15 years of age and MCP842 protocol with vinblastine for 1 year in <15 years of age. Event free survival was noted. These outcomes were correlated with ALK status, International Prognostic Index (IPI) score, and stage at presentation. RESULTS: At a median follow-up of 36 months (range: 6-72 months) ALK- ALCL had a poor outcome. The 3 year event free survival in pediatric ALCL was 66.7%. In adults, this was 60% ALK+ ALCL was 60% and 20% in ALK- ALCL. CONCLUSIONS: Systemic ALCL is an aggressive disease. CD3 + positivity is commonly seen in ALK- ALCL and ALK+, epithelial membrane antigen + positivity is seen in ALK+ ALCL. ALK- ALCL, advanced stage III, IV and high IPI score were associated with poor prognosis. The demographic profile and outcome in our study was similar to the world literature. With new drugs like crizotinib and brentuximab vedotin the future looks very promising.
Authors: M Strullu; C Thomas; M-C Le Deley; A Chevance; J Kanold; Y Bertrand; C Jubert; J-H Dalle; C Paillard; A Baruchel; L Lamant; G Michel; L Brugières Journal: Bone Marrow Transplant Date: 2015-03-30 Impact factor: 5.483