VITREORETINAL LYMPHOMA: A DESCRIPTIVE STUDY OF A VERY RARE OCCURRENCE FROM A SINGLE INSTITUTION IN ITALY.
(Abstract release date: 05/19/16)
EHA Library. Ferrari A. 06/09/16; 132534; E985
Dr. Angela Ferrari
Contributions
Contributions
Abstract
Abstract: E985
Type: Eposter Presentation
Background
The vitreoretinal lymphoma is a rare subset of primary central nervous system lymphoma (PCNSL) with a poor prognosis. Because of its rarity and the difficult of collecting tumor simples, this entity remains poorly understood and the optimal management is still unknow.
Aims
to describe a single insitution experience with Vitreoretinal lymphomas.
Methods
The 2011-2015 archives of the hematology and oftalmology units of the Reggio Emilia Hospital Lymphoma were searched for cases with vitreoretinal lymphoma. Cases were classified as primary vitreoretinal lymphoma (PVRL) of systemic lymphomas with concurrent Vitreoretinal involvement (CVRL). We defined stage IE as mono or bilateral involvement of the vitreum. We provide a complete description of pathological, clinical, and therapeutic findings.
Results
We identified 7 patients with either PVRL (3) or with CVRL (4). Four patient were female, the median age at diagnosis was 62 years (range 45-74), three had a stage IE and four a stage IVA. All patients were HIV negative. Among the 4 CVRL 2 patients showed brain involvement, one patient nodal involvement, one patient gastric involvement. All patients were initially referred with ocular symptoms mimicking uveitis. The median time between the onset of the ocular symptoms and the lymphoma diagnosis was 13 months (range 2-30 months). All patients underwent a diagnostic vitrectomy. The cytopathological analysis was positive in five cases and uncertain in two cases (CVRL). The immunoistochemical analysis showed atypical CD20+ lymphocytes in three cases. Vitreous supernatant was submitted for IL-10 and IL-6 measurement by ELISA using the IL-10 to IL-6 ratio as a diagnostic marker. Elevation of IL-10 levels and IL-10:IL-6 ratio greather than 1 was found in all cases. Molecular analysis of the specimens detected IgH gene rearrangements in 4 cases. Notably the three PCR negative samples displayed a sharp unbalanced IL-10:IL-6 ratio and atypical CD20+ lymphoid large cells. Cerebral Spine Fluid (CSF) analysis was negative in all patients.All patients were treated as CNS lymphomas and received combined high-dose systemic chemotherapy with Rituximab, High dose ARA-C and High dose Methotrexate, and intravitreal methotrexate iniection (Intravitreal methotrexate 400 microg/0,1 ml twice weekly for 4 weeks, once weekly for 8 weeks, and then once montly for 9 months for a total of 25 injection).All 7 patients achieved a complete remission (CR) at the end of therapy. Four patients underwent autologous stem cell transplant (ASCT) in first remission.The median observation period was 16 months (range, 2-48 months). All patients were alive at this time point except for one patient who relapsed four months after ASCT and died one month later.
Conclusion
This single center retrospective series highlights the insidious onset of both PVRL and CVRL and the difficulties in diagnosing this particularly rare lymphoma occurrence.
Session topic: E-poster
Keyword(s): Diagnosis, Lymphoma
Type: Eposter Presentation
Background
The vitreoretinal lymphoma is a rare subset of primary central nervous system lymphoma (PCNSL) with a poor prognosis. Because of its rarity and the difficult of collecting tumor simples, this entity remains poorly understood and the optimal management is still unknow.
Aims
to describe a single insitution experience with Vitreoretinal lymphomas.
Methods
The 2011-2015 archives of the hematology and oftalmology units of the Reggio Emilia Hospital Lymphoma were searched for cases with vitreoretinal lymphoma. Cases were classified as primary vitreoretinal lymphoma (PVRL) of systemic lymphomas with concurrent Vitreoretinal involvement (CVRL). We defined stage IE as mono or bilateral involvement of the vitreum. We provide a complete description of pathological, clinical, and therapeutic findings.
Results
We identified 7 patients with either PVRL (3) or with CVRL (4). Four patient were female, the median age at diagnosis was 62 years (range 45-74), three had a stage IE and four a stage IVA. All patients were HIV negative. Among the 4 CVRL 2 patients showed brain involvement, one patient nodal involvement, one patient gastric involvement. All patients were initially referred with ocular symptoms mimicking uveitis. The median time between the onset of the ocular symptoms and the lymphoma diagnosis was 13 months (range 2-30 months). All patients underwent a diagnostic vitrectomy. The cytopathological analysis was positive in five cases and uncertain in two cases (CVRL). The immunoistochemical analysis showed atypical CD20+ lymphocytes in three cases. Vitreous supernatant was submitted for IL-10 and IL-6 measurement by ELISA using the IL-10 to IL-6 ratio as a diagnostic marker. Elevation of IL-10 levels and IL-10:IL-6 ratio greather than 1 was found in all cases. Molecular analysis of the specimens detected IgH gene rearrangements in 4 cases. Notably the three PCR negative samples displayed a sharp unbalanced IL-10:IL-6 ratio and atypical CD20+ lymphoid large cells. Cerebral Spine Fluid (CSF) analysis was negative in all patients.All patients were treated as CNS lymphomas and received combined high-dose systemic chemotherapy with Rituximab, High dose ARA-C and High dose Methotrexate, and intravitreal methotrexate iniection (Intravitreal methotrexate 400 microg/0,1 ml twice weekly for 4 weeks, once weekly for 8 weeks, and then once montly for 9 months for a total of 25 injection).All 7 patients achieved a complete remission (CR) at the end of therapy. Four patients underwent autologous stem cell transplant (ASCT) in first remission.The median observation period was 16 months (range, 2-48 months). All patients were alive at this time point except for one patient who relapsed four months after ASCT and died one month later.
Conclusion
This single center retrospective series highlights the insidious onset of both PVRL and CVRL and the difficulties in diagnosing this particularly rare lymphoma occurrence.
Session topic: E-poster
Keyword(s): Diagnosis, Lymphoma
Abstract: E985
Type: Eposter Presentation
Background
The vitreoretinal lymphoma is a rare subset of primary central nervous system lymphoma (PCNSL) with a poor prognosis. Because of its rarity and the difficult of collecting tumor simples, this entity remains poorly understood and the optimal management is still unknow.
Aims
to describe a single insitution experience with Vitreoretinal lymphomas.
Methods
The 2011-2015 archives of the hematology and oftalmology units of the Reggio Emilia Hospital Lymphoma were searched for cases with vitreoretinal lymphoma. Cases were classified as primary vitreoretinal lymphoma (PVRL) of systemic lymphomas with concurrent Vitreoretinal involvement (CVRL). We defined stage IE as mono or bilateral involvement of the vitreum. We provide a complete description of pathological, clinical, and therapeutic findings.
Results
We identified 7 patients with either PVRL (3) or with CVRL (4). Four patient were female, the median age at diagnosis was 62 years (range 45-74), three had a stage IE and four a stage IVA. All patients were HIV negative. Among the 4 CVRL 2 patients showed brain involvement, one patient nodal involvement, one patient gastric involvement. All patients were initially referred with ocular symptoms mimicking uveitis. The median time between the onset of the ocular symptoms and the lymphoma diagnosis was 13 months (range 2-30 months). All patients underwent a diagnostic vitrectomy. The cytopathological analysis was positive in five cases and uncertain in two cases (CVRL). The immunoistochemical analysis showed atypical CD20+ lymphocytes in three cases. Vitreous supernatant was submitted for IL-10 and IL-6 measurement by ELISA using the IL-10 to IL-6 ratio as a diagnostic marker. Elevation of IL-10 levels and IL-10:IL-6 ratio greather than 1 was found in all cases. Molecular analysis of the specimens detected IgH gene rearrangements in 4 cases. Notably the three PCR negative samples displayed a sharp unbalanced IL-10:IL-6 ratio and atypical CD20+ lymphoid large cells. Cerebral Spine Fluid (CSF) analysis was negative in all patients.All patients were treated as CNS lymphomas and received combined high-dose systemic chemotherapy with Rituximab, High dose ARA-C and High dose Methotrexate, and intravitreal methotrexate iniection (Intravitreal methotrexate 400 microg/0,1 ml twice weekly for 4 weeks, once weekly for 8 weeks, and then once montly for 9 months for a total of 25 injection).All 7 patients achieved a complete remission (CR) at the end of therapy. Four patients underwent autologous stem cell transplant (ASCT) in first remission.The median observation period was 16 months (range, 2-48 months). All patients were alive at this time point except for one patient who relapsed four months after ASCT and died one month later.
Conclusion
This single center retrospective series highlights the insidious onset of both PVRL and CVRL and the difficulties in diagnosing this particularly rare lymphoma occurrence.
Session topic: E-poster
Keyword(s): Diagnosis, Lymphoma
Type: Eposter Presentation
Background
The vitreoretinal lymphoma is a rare subset of primary central nervous system lymphoma (PCNSL) with a poor prognosis. Because of its rarity and the difficult of collecting tumor simples, this entity remains poorly understood and the optimal management is still unknow.
Aims
to describe a single insitution experience with Vitreoretinal lymphomas.
Methods
The 2011-2015 archives of the hematology and oftalmology units of the Reggio Emilia Hospital Lymphoma were searched for cases with vitreoretinal lymphoma. Cases were classified as primary vitreoretinal lymphoma (PVRL) of systemic lymphomas with concurrent Vitreoretinal involvement (CVRL). We defined stage IE as mono or bilateral involvement of the vitreum. We provide a complete description of pathological, clinical, and therapeutic findings.
Results
We identified 7 patients with either PVRL (3) or with CVRL (4). Four patient were female, the median age at diagnosis was 62 years (range 45-74), three had a stage IE and four a stage IVA. All patients were HIV negative. Among the 4 CVRL 2 patients showed brain involvement, one patient nodal involvement, one patient gastric involvement. All patients were initially referred with ocular symptoms mimicking uveitis. The median time between the onset of the ocular symptoms and the lymphoma diagnosis was 13 months (range 2-30 months). All patients underwent a diagnostic vitrectomy. The cytopathological analysis was positive in five cases and uncertain in two cases (CVRL). The immunoistochemical analysis showed atypical CD20+ lymphocytes in three cases. Vitreous supernatant was submitted for IL-10 and IL-6 measurement by ELISA using the IL-10 to IL-6 ratio as a diagnostic marker. Elevation of IL-10 levels and IL-10:IL-6 ratio greather than 1 was found in all cases. Molecular analysis of the specimens detected IgH gene rearrangements in 4 cases. Notably the three PCR negative samples displayed a sharp unbalanced IL-10:IL-6 ratio and atypical CD20+ lymphoid large cells. Cerebral Spine Fluid (CSF) analysis was negative in all patients.All patients were treated as CNS lymphomas and received combined high-dose systemic chemotherapy with Rituximab, High dose ARA-C and High dose Methotrexate, and intravitreal methotrexate iniection (Intravitreal methotrexate 400 microg/0,1 ml twice weekly for 4 weeks, once weekly for 8 weeks, and then once montly for 9 months for a total of 25 injection).All 7 patients achieved a complete remission (CR) at the end of therapy. Four patients underwent autologous stem cell transplant (ASCT) in first remission.The median observation period was 16 months (range, 2-48 months). All patients were alive at this time point except for one patient who relapsed four months after ASCT and died one month later.
Conclusion
This single center retrospective series highlights the insidious onset of both PVRL and CVRL and the difficulties in diagnosing this particularly rare lymphoma occurrence.
Session topic: E-poster
Keyword(s): Diagnosis, Lymphoma
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