TREATMENT PECULIARITIES OF NON-HODGKIN?S LYMPHOMAS IN CHILDREN WITH NIJMEGEN BREAKAGE SYNDROME
(Abstract release date: 05/19/16)
EHA Library. Kozlova O. 06/09/16; 134614; PB1714
Dr. Olena Kozlova
Contributions
Contributions
Abstract
Abstract: PB1714
Type: Publication Only
Background
Nijmegen breakage syndrome (NBS) is rare autosomal recessive disorder, typically characterized by increased chromosome fragility phenomenon, immune deficiency, hypersensitivity to radiation and high predisposition to malignancy.
Aims
To investigate the effectiveness of cytostatic treatment of non-Hodgkin lymphomas (NHL) in children with NBS.
Methods
1992 through 2014 one hundred children in the Hematology Department of Lviv Children hospital were first diagnosed with, 9 (9%) out them with NBS. The median age of this children group was 8 years and 8 months (from 4 years 4 months to 14 years 11 months old). Male to female ratio was 3:2. The diagnosis of NBS was based on specific phenotype features, results of clinical, genetic (657del5 NBN gene mutation) and laboratory investigations. The diagnosis of NHL was based on clinical symptoms, morphological and immunological analysis of tumor substrate. Patients received treatment according to BFM-group protocols (NHL-BFM-90/95, NHL-DGLLU-2000).
Results
The NHL types were established as follows: lymphoblastic lymphoma (LBL) in 3 patients, diffuse large B-cell lymphoma (DLBCL) in 6 patients. In most cases the primary manifestations were nodal: peripheral lymph nodes - in 9 patients, mediastinal – in 7, abdominal lymph nodes - in 3, and spleen – in 7 children. Involved extranodal sites included liver (6 patients), lung and pleura (5), skull bones (2), kidney (1), CNS (1). Atypical cells (L1/L2 and L3-type lymphoblasts according to FAB-classification) in the bone marrow were found in 2 patients with LBL, 1 – with DLBCL. Cytostatic treatment was initiated in 8 patients. One patient was in the terminal state and died before the specific treatment. Three patients died upon the completion of first chemotherapy cycles, due to serious infectious complications (pneumonia, pneumothorax, enteropathy, necrotic stomatitis, palatal fistula). In a 4 year old boy with DLBCL, after 2 cycles, the tumor didn’t go into regression. The salvage therapy (ICE + rituximab) was not effective; the child died of lymphoma progression. In 4 patients the cytostatic treatment was successful. Three children with LBL received a full course of protocol treatment without reducing the doses of cytostatic and are in remission at present (duration of remission in months – 215, 39, 30). In one girl with DLBCL the treatment was discontinued after the 5th cycle (AA N2) of NHL-DGLLU-2000 protocol, because of coccyx area soft tissue necrosis, thus the last BB cycle was not conducted. After 11 years of remission in this patient a secondary tumor (meningioma) was diagnosed, her treatment with the CyberKnife radiosurgery was effective.
Conclusion
The complexity of the clinical course of NHL in NBS patients was preconditioned by the presence of concomitant infections on the background of combined immunodeficiency, which caused death of 4 patients at the beginning of the treatment. One patient died from lymphoma progression. Specific NHL treatment in NBS patients is possible and effective, given the individual correction of doses and schedules of applied cytostatic agents, and if adequate supportive therapy is provided concomitantly. Nonetheless, after successful NHL treatment in children with NBS, the risks of secondary tumor development still run high.
Session topic: E-poster
Keyword(s): Chemotherapy, Children, Immunodeficiency, Non-Hodgkin's lymphoma
Type: Publication Only
Background
Nijmegen breakage syndrome (NBS) is rare autosomal recessive disorder, typically characterized by increased chromosome fragility phenomenon, immune deficiency, hypersensitivity to radiation and high predisposition to malignancy.
Aims
To investigate the effectiveness of cytostatic treatment of non-Hodgkin lymphomas (NHL) in children with NBS.
Methods
1992 through 2014 one hundred children in the Hematology Department of Lviv Children hospital were first diagnosed with, 9 (9%) out them with NBS. The median age of this children group was 8 years and 8 months (from 4 years 4 months to 14 years 11 months old). Male to female ratio was 3:2. The diagnosis of NBS was based on specific phenotype features, results of clinical, genetic (657del5 NBN gene mutation) and laboratory investigations. The diagnosis of NHL was based on clinical symptoms, morphological and immunological analysis of tumor substrate. Patients received treatment according to BFM-group protocols (NHL-BFM-90/95, NHL-DGLLU-2000).
Results
The NHL types were established as follows: lymphoblastic lymphoma (LBL) in 3 patients, diffuse large B-cell lymphoma (DLBCL) in 6 patients. In most cases the primary manifestations were nodal: peripheral lymph nodes - in 9 patients, mediastinal – in 7, abdominal lymph nodes - in 3, and spleen – in 7 children. Involved extranodal sites included liver (6 patients), lung and pleura (5), skull bones (2), kidney (1), CNS (1). Atypical cells (L1/L2 and L3-type lymphoblasts according to FAB-classification) in the bone marrow were found in 2 patients with LBL, 1 – with DLBCL. Cytostatic treatment was initiated in 8 patients. One patient was in the terminal state and died before the specific treatment. Three patients died upon the completion of first chemotherapy cycles, due to serious infectious complications (pneumonia, pneumothorax, enteropathy, necrotic stomatitis, palatal fistula). In a 4 year old boy with DLBCL, after 2 cycles, the tumor didn’t go into regression. The salvage therapy (ICE + rituximab) was not effective; the child died of lymphoma progression. In 4 patients the cytostatic treatment was successful. Three children with LBL received a full course of protocol treatment without reducing the doses of cytostatic and are in remission at present (duration of remission in months – 215, 39, 30). In one girl with DLBCL the treatment was discontinued after the 5th cycle (AA N2) of NHL-DGLLU-2000 protocol, because of coccyx area soft tissue necrosis, thus the last BB cycle was not conducted. After 11 years of remission in this patient a secondary tumor (meningioma) was diagnosed, her treatment with the CyberKnife radiosurgery was effective.
Conclusion
The complexity of the clinical course of NHL in NBS patients was preconditioned by the presence of concomitant infections on the background of combined immunodeficiency, which caused death of 4 patients at the beginning of the treatment. One patient died from lymphoma progression. Specific NHL treatment in NBS patients is possible and effective, given the individual correction of doses and schedules of applied cytostatic agents, and if adequate supportive therapy is provided concomitantly. Nonetheless, after successful NHL treatment in children with NBS, the risks of secondary tumor development still run high.
Session topic: E-poster
Keyword(s): Chemotherapy, Children, Immunodeficiency, Non-Hodgkin's lymphoma
Abstract: PB1714
Type: Publication Only
Background
Nijmegen breakage syndrome (NBS) is rare autosomal recessive disorder, typically characterized by increased chromosome fragility phenomenon, immune deficiency, hypersensitivity to radiation and high predisposition to malignancy.
Aims
To investigate the effectiveness of cytostatic treatment of non-Hodgkin lymphomas (NHL) in children with NBS.
Methods
1992 through 2014 one hundred children in the Hematology Department of Lviv Children hospital were first diagnosed with, 9 (9%) out them with NBS. The median age of this children group was 8 years and 8 months (from 4 years 4 months to 14 years 11 months old). Male to female ratio was 3:2. The diagnosis of NBS was based on specific phenotype features, results of clinical, genetic (657del5 NBN gene mutation) and laboratory investigations. The diagnosis of NHL was based on clinical symptoms, morphological and immunological analysis of tumor substrate. Patients received treatment according to BFM-group protocols (NHL-BFM-90/95, NHL-DGLLU-2000).
Results
The NHL types were established as follows: lymphoblastic lymphoma (LBL) in 3 patients, diffuse large B-cell lymphoma (DLBCL) in 6 patients. In most cases the primary manifestations were nodal: peripheral lymph nodes - in 9 patients, mediastinal – in 7, abdominal lymph nodes - in 3, and spleen – in 7 children. Involved extranodal sites included liver (6 patients), lung and pleura (5), skull bones (2), kidney (1), CNS (1). Atypical cells (L1/L2 and L3-type lymphoblasts according to FAB-classification) in the bone marrow were found in 2 patients with LBL, 1 – with DLBCL. Cytostatic treatment was initiated in 8 patients. One patient was in the terminal state and died before the specific treatment. Three patients died upon the completion of first chemotherapy cycles, due to serious infectious complications (pneumonia, pneumothorax, enteropathy, necrotic stomatitis, palatal fistula). In a 4 year old boy with DLBCL, after 2 cycles, the tumor didn’t go into regression. The salvage therapy (ICE + rituximab) was not effective; the child died of lymphoma progression. In 4 patients the cytostatic treatment was successful. Three children with LBL received a full course of protocol treatment without reducing the doses of cytostatic and are in remission at present (duration of remission in months – 215, 39, 30). In one girl with DLBCL the treatment was discontinued after the 5th cycle (AA N2) of NHL-DGLLU-2000 protocol, because of coccyx area soft tissue necrosis, thus the last BB cycle was not conducted. After 11 years of remission in this patient a secondary tumor (meningioma) was diagnosed, her treatment with the CyberKnife radiosurgery was effective.
Conclusion
The complexity of the clinical course of NHL in NBS patients was preconditioned by the presence of concomitant infections on the background of combined immunodeficiency, which caused death of 4 patients at the beginning of the treatment. One patient died from lymphoma progression. Specific NHL treatment in NBS patients is possible and effective, given the individual correction of doses and schedules of applied cytostatic agents, and if adequate supportive therapy is provided concomitantly. Nonetheless, after successful NHL treatment in children with NBS, the risks of secondary tumor development still run high.
Session topic: E-poster
Keyword(s): Chemotherapy, Children, Immunodeficiency, Non-Hodgkin's lymphoma
Type: Publication Only
Background
Nijmegen breakage syndrome (NBS) is rare autosomal recessive disorder, typically characterized by increased chromosome fragility phenomenon, immune deficiency, hypersensitivity to radiation and high predisposition to malignancy.
Aims
To investigate the effectiveness of cytostatic treatment of non-Hodgkin lymphomas (NHL) in children with NBS.
Methods
1992 through 2014 one hundred children in the Hematology Department of Lviv Children hospital were first diagnosed with, 9 (9%) out them with NBS. The median age of this children group was 8 years and 8 months (from 4 years 4 months to 14 years 11 months old). Male to female ratio was 3:2. The diagnosis of NBS was based on specific phenotype features, results of clinical, genetic (657del5 NBN gene mutation) and laboratory investigations. The diagnosis of NHL was based on clinical symptoms, morphological and immunological analysis of tumor substrate. Patients received treatment according to BFM-group protocols (NHL-BFM-90/95, NHL-DGLLU-2000).
Results
The NHL types were established as follows: lymphoblastic lymphoma (LBL) in 3 patients, diffuse large B-cell lymphoma (DLBCL) in 6 patients. In most cases the primary manifestations were nodal: peripheral lymph nodes - in 9 patients, mediastinal – in 7, abdominal lymph nodes - in 3, and spleen – in 7 children. Involved extranodal sites included liver (6 patients), lung and pleura (5), skull bones (2), kidney (1), CNS (1). Atypical cells (L1/L2 and L3-type lymphoblasts according to FAB-classification) in the bone marrow were found in 2 patients with LBL, 1 – with DLBCL. Cytostatic treatment was initiated in 8 patients. One patient was in the terminal state and died before the specific treatment. Three patients died upon the completion of first chemotherapy cycles, due to serious infectious complications (pneumonia, pneumothorax, enteropathy, necrotic stomatitis, palatal fistula). In a 4 year old boy with DLBCL, after 2 cycles, the tumor didn’t go into regression. The salvage therapy (ICE + rituximab) was not effective; the child died of lymphoma progression. In 4 patients the cytostatic treatment was successful. Three children with LBL received a full course of protocol treatment without reducing the doses of cytostatic and are in remission at present (duration of remission in months – 215, 39, 30). In one girl with DLBCL the treatment was discontinued after the 5th cycle (AA N2) of NHL-DGLLU-2000 protocol, because of coccyx area soft tissue necrosis, thus the last BB cycle was not conducted. After 11 years of remission in this patient a secondary tumor (meningioma) was diagnosed, her treatment with the CyberKnife radiosurgery was effective.
Conclusion
The complexity of the clinical course of NHL in NBS patients was preconditioned by the presence of concomitant infections on the background of combined immunodeficiency, which caused death of 4 patients at the beginning of the treatment. One patient died from lymphoma progression. Specific NHL treatment in NBS patients is possible and effective, given the individual correction of doses and schedules of applied cytostatic agents, and if adequate supportive therapy is provided concomitantly. Nonetheless, after successful NHL treatment in children with NBS, the risks of secondary tumor development still run high.
Session topic: E-poster
Keyword(s): Chemotherapy, Children, Immunodeficiency, Non-Hodgkin's lymphoma
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