Contributions
Abstract: PB1798
Type: Publication Only
Background
Mediastinal B-cell lymphomas present in the mediastinum and are most frequent in young patients. Nodular sclerosis Hodgkin lymphoma (NS HL) and primary mediastinal B-cell lymphoma (PMBL) are common types, whereas mediastinal grey-zone lymphoma (MGZL) is extremely rare and has pathological features intermediate between NS HL and PMBL. The indeterminate pathobiology of MGZL led to uncertainty regarding therapeutic strategy. Clinical characteristics of MGZL and treatment strategy have not been characterized.
Aims
To describe clinical features of patients with MGZL.
Methods
From 2007 to 2018 years, 20 patients were diagnosed with MGZL (according to WHO criteria) in National Research Center for Hematology Моscow Russian Federation. The median age of patients was 32 years (from 19 to 54). There were 7 males, 13 females. Increased LDH activity was revealed in 69% of pts. Bulky mediastinal disease manifested in 45% of pts. A metachronous development of NS HL and PMBCL was observed in 2 out of 20 pts. Synchronous onset of classical HL and PMBL occurred in 18 out of 20 pts. Тreatment was completed in 16 out of 20 pts.
Therapeutically, the Hodgkin-like pathological features of MGZL was treated like HL. Cases with strong expression of the CD20 were treated with rituximab. Cases with features of PMBL, with rituximab-based regimens as with PMBL. R-DA-EPOCH/m-NHL-BFM-90 were used in 12 out of 16 pts, R-BEACOPP-14 in 4 out of 16 patients. Autologous peripheral blood stem cell transplantation (auto-SCT) with BEAM conditioning regimen after first-line treatment was performed in 6 out of 16 pts.
Results
After R-DA-EPOCH/m-NHL-BFM-90 therapy CR was achieved in 6 out of 16 pts. Four out of 16 pts had PR and underwent 2 R-DHAP courses followed by auto-SCT. Early relapse occurred in 1 out of 16 patient. Two out of 16 were primary resistant and underwent the PD-1 blockade.
After R-BEACOPP-14 therapy CR was achieved in 3 out of 16 pts, 1 out of 16 pts had PR and underwent 2 R-DHAP courses followed by auto-SCT, but early disease progression led to lethal outcome. Median follow up was 32 months.
Conclusion
Our data suggest that intensive treatment could improve outcome of this rare and aggressive disease.
Session topic: 21. Aggressive Non-Hodgkin lymphoma - Clinical
Keyword(s): Lymphoid malignancy
Abstract: PB1798
Type: Publication Only
Background
Mediastinal B-cell lymphomas present in the mediastinum and are most frequent in young patients. Nodular sclerosis Hodgkin lymphoma (NS HL) and primary mediastinal B-cell lymphoma (PMBL) are common types, whereas mediastinal grey-zone lymphoma (MGZL) is extremely rare and has pathological features intermediate between NS HL and PMBL. The indeterminate pathobiology of MGZL led to uncertainty regarding therapeutic strategy. Clinical characteristics of MGZL and treatment strategy have not been characterized.
Aims
To describe clinical features of patients with MGZL.
Methods
From 2007 to 2018 years, 20 patients were diagnosed with MGZL (according to WHO criteria) in National Research Center for Hematology Моscow Russian Federation. The median age of patients was 32 years (from 19 to 54). There were 7 males, 13 females. Increased LDH activity was revealed in 69% of pts. Bulky mediastinal disease manifested in 45% of pts. A metachronous development of NS HL and PMBCL was observed in 2 out of 20 pts. Synchronous onset of classical HL and PMBL occurred in 18 out of 20 pts. Тreatment was completed in 16 out of 20 pts.
Therapeutically, the Hodgkin-like pathological features of MGZL was treated like HL. Cases with strong expression of the CD20 were treated with rituximab. Cases with features of PMBL, with rituximab-based regimens as with PMBL. R-DA-EPOCH/m-NHL-BFM-90 were used in 12 out of 16 pts, R-BEACOPP-14 in 4 out of 16 patients. Autologous peripheral blood stem cell transplantation (auto-SCT) with BEAM conditioning regimen after first-line treatment was performed in 6 out of 16 pts.
Results
After R-DA-EPOCH/m-NHL-BFM-90 therapy CR was achieved in 6 out of 16 pts. Four out of 16 pts had PR and underwent 2 R-DHAP courses followed by auto-SCT. Early relapse occurred in 1 out of 16 patient. Two out of 16 were primary resistant and underwent the PD-1 blockade.
After R-BEACOPP-14 therapy CR was achieved in 3 out of 16 pts, 1 out of 16 pts had PR and underwent 2 R-DHAP courses followed by auto-SCT, but early disease progression led to lethal outcome. Median follow up was 32 months.
Conclusion
Our data suggest that intensive treatment could improve outcome of this rare and aggressive disease.
Session topic: 21. Aggressive Non-Hodgkin lymphoma - Clinical
Keyword(s): Lymphoid malignancy