EPSTEIN-BARR VIRUS-ASSOCIATED T-LYMPHOPROLIFERATIVE DISORDERS IN ADULTS: A CASE SERIES OF 13 PATIENTS IN A SINGLE INSTITUTION AND REVIEW OF LITERATURE
(Abstract release date: 05/19/16)
EHA Library. shi P. 06/09/16; 135374; LB2263
Dr. Pengfei shi
Contributions
Contributions
Abstract
Abstract: LB2263
Type: Eposter Presentation
Background
Epstein-Barr virus (EBV) usually infected B lymphocytes, sometimes also infected T- or natural killer (NK)-cells. EBV-associated T/NK cell lymphoproliferative disease (EBV-T/NK-LPD) represents a continuous spectrum of diseases, ranging from benign hyperplasia of reactivity to clonal and malignant lymphoproliferations. Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorder in children and young adults is a systemic disease, and features with EBV infection of T or NK cells cloned proliferation.
Aims
To understand the clinical features of EBV-associated T/NK cell lymphoproliferative disease and its poor prognosis, recognize the importance of early diagnosis and timely treatment.
Methods
Retrospective analysis of 13 patients with clinical features, EB virus spectrum, treatment and prognosis
Results
we report 13 adult cases of systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorder including 9 males and 4 females with a median age of 41 years old (range,24-75). the most common clinical symoptoms were fever 92.3% (12/13), splenomegaly 100% (13/13), hepatomegaly 30.8% (4/13), lymph node enlargement 38.5% (5 /13), blood cells decrease 92.3% (12/13), abnormal liver function 76.9% (10/13), elevated lactate dehydrogenase 76.9% (10/13), coagulant function abnormality 61.5% (8/13). All patients had significantly increased serum EBV-DNA levels (3.41×104-6.61×106 copy/ml). EBER were positive by Fluorescence in situ hybridization (FISH) (5 cases lymph node and 8 cases bone marrow). T lymphocytes were involved in this group, 7 cases were diagnosed as chronic active EB virus infection (CAEBV), and 6 cases were diagnosed as EBV-associated hemophagocytic lymphohistiocytosis (HLH).One paient with CAEBV had no fever and blood cells decreases, keeping stable disease with prednisone treatment; and another one patient with CAEBV progress for aggressive NK cell leukemia after 4 months diagnosis, with EPOCH regimen combined L-asparaginase chemotherapy. The remaining 11 patients were treated with immunochemotherapy containing etoposide, but the effect is not good. In a median follow-up time of 18 months, one patient was free of disease, one was alive with disease, and eleven died of disease in 5 months, respectively.
Conclusion
Adult systemic EBV + T/NK-LPDs was relatively rare disease and the prognosis is poor. Our report adds to the understanding of these rare disease with early diagnosis and intervention.
Session topic: E-poster
Keyword(s): EBV
Type: Eposter Presentation
Background
Epstein-Barr virus (EBV) usually infected B lymphocytes, sometimes also infected T- or natural killer (NK)-cells. EBV-associated T/NK cell lymphoproliferative disease (EBV-T/NK-LPD) represents a continuous spectrum of diseases, ranging from benign hyperplasia of reactivity to clonal and malignant lymphoproliferations. Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorder in children and young adults is a systemic disease, and features with EBV infection of T or NK cells cloned proliferation.
Aims
To understand the clinical features of EBV-associated T/NK cell lymphoproliferative disease and its poor prognosis, recognize the importance of early diagnosis and timely treatment.
Methods
Retrospective analysis of 13 patients with clinical features, EB virus spectrum, treatment and prognosis
Results
we report 13 adult cases of systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorder including 9 males and 4 females with a median age of 41 years old (range,24-75). the most common clinical symoptoms were fever 92.3% (12/13), splenomegaly 100% (13/13), hepatomegaly 30.8% (4/13), lymph node enlargement 38.5% (5 /13), blood cells decrease 92.3% (12/13), abnormal liver function 76.9% (10/13), elevated lactate dehydrogenase 76.9% (10/13), coagulant function abnormality 61.5% (8/13). All patients had significantly increased serum EBV-DNA levels (3.41×104-6.61×106 copy/ml). EBER were positive by Fluorescence in situ hybridization (FISH) (5 cases lymph node and 8 cases bone marrow). T lymphocytes were involved in this group, 7 cases were diagnosed as chronic active EB virus infection (CAEBV), and 6 cases were diagnosed as EBV-associated hemophagocytic lymphohistiocytosis (HLH).One paient with CAEBV had no fever and blood cells decreases, keeping stable disease with prednisone treatment; and another one patient with CAEBV progress for aggressive NK cell leukemia after 4 months diagnosis, with EPOCH regimen combined L-asparaginase chemotherapy. The remaining 11 patients were treated with immunochemotherapy containing etoposide, but the effect is not good. In a median follow-up time of 18 months, one patient was free of disease, one was alive with disease, and eleven died of disease in 5 months, respectively.
Conclusion
Adult systemic EBV + T/NK-LPDs was relatively rare disease and the prognosis is poor. Our report adds to the understanding of these rare disease with early diagnosis and intervention.
Session topic: E-poster
Keyword(s): EBV
Abstract: LB2263
Type: Eposter Presentation
Background
Epstein-Barr virus (EBV) usually infected B lymphocytes, sometimes also infected T- or natural killer (NK)-cells. EBV-associated T/NK cell lymphoproliferative disease (EBV-T/NK-LPD) represents a continuous spectrum of diseases, ranging from benign hyperplasia of reactivity to clonal and malignant lymphoproliferations. Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorder in children and young adults is a systemic disease, and features with EBV infection of T or NK cells cloned proliferation.
Aims
To understand the clinical features of EBV-associated T/NK cell lymphoproliferative disease and its poor prognosis, recognize the importance of early diagnosis and timely treatment.
Methods
Retrospective analysis of 13 patients with clinical features, EB virus spectrum, treatment and prognosis
Results
we report 13 adult cases of systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorder including 9 males and 4 females with a median age of 41 years old (range,24-75). the most common clinical symoptoms were fever 92.3% (12/13), splenomegaly 100% (13/13), hepatomegaly 30.8% (4/13), lymph node enlargement 38.5% (5 /13), blood cells decrease 92.3% (12/13), abnormal liver function 76.9% (10/13), elevated lactate dehydrogenase 76.9% (10/13), coagulant function abnormality 61.5% (8/13). All patients had significantly increased serum EBV-DNA levels (3.41×104-6.61×106 copy/ml). EBER were positive by Fluorescence in situ hybridization (FISH) (5 cases lymph node and 8 cases bone marrow). T lymphocytes were involved in this group, 7 cases were diagnosed as chronic active EB virus infection (CAEBV), and 6 cases were diagnosed as EBV-associated hemophagocytic lymphohistiocytosis (HLH).One paient with CAEBV had no fever and blood cells decreases, keeping stable disease with prednisone treatment; and another one patient with CAEBV progress for aggressive NK cell leukemia after 4 months diagnosis, with EPOCH regimen combined L-asparaginase chemotherapy. The remaining 11 patients were treated with immunochemotherapy containing etoposide, but the effect is not good. In a median follow-up time of 18 months, one patient was free of disease, one was alive with disease, and eleven died of disease in 5 months, respectively.
Conclusion
Adult systemic EBV + T/NK-LPDs was relatively rare disease and the prognosis is poor. Our report adds to the understanding of these rare disease with early diagnosis and intervention.
Session topic: E-poster
Keyword(s): EBV
Type: Eposter Presentation
Background
Epstein-Barr virus (EBV) usually infected B lymphocytes, sometimes also infected T- or natural killer (NK)-cells. EBV-associated T/NK cell lymphoproliferative disease (EBV-T/NK-LPD) represents a continuous spectrum of diseases, ranging from benign hyperplasia of reactivity to clonal and malignant lymphoproliferations. Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorder in children and young adults is a systemic disease, and features with EBV infection of T or NK cells cloned proliferation.
Aims
To understand the clinical features of EBV-associated T/NK cell lymphoproliferative disease and its poor prognosis, recognize the importance of early diagnosis and timely treatment.
Methods
Retrospective analysis of 13 patients with clinical features, EB virus spectrum, treatment and prognosis
Results
we report 13 adult cases of systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorder including 9 males and 4 females with a median age of 41 years old (range,24-75). the most common clinical symoptoms were fever 92.3% (12/13), splenomegaly 100% (13/13), hepatomegaly 30.8% (4/13), lymph node enlargement 38.5% (5 /13), blood cells decrease 92.3% (12/13), abnormal liver function 76.9% (10/13), elevated lactate dehydrogenase 76.9% (10/13), coagulant function abnormality 61.5% (8/13). All patients had significantly increased serum EBV-DNA levels (3.41×104-6.61×106 copy/ml). EBER were positive by Fluorescence in situ hybridization (FISH) (5 cases lymph node and 8 cases bone marrow). T lymphocytes were involved in this group, 7 cases were diagnosed as chronic active EB virus infection (CAEBV), and 6 cases were diagnosed as EBV-associated hemophagocytic lymphohistiocytosis (HLH).One paient with CAEBV had no fever and blood cells decreases, keeping stable disease with prednisone treatment; and another one patient with CAEBV progress for aggressive NK cell leukemia after 4 months diagnosis, with EPOCH regimen combined L-asparaginase chemotherapy. The remaining 11 patients were treated with immunochemotherapy containing etoposide, but the effect is not good. In a median follow-up time of 18 months, one patient was free of disease, one was alive with disease, and eleven died of disease in 5 months, respectively.
Conclusion
Adult systemic EBV + T/NK-LPDs was relatively rare disease and the prognosis is poor. Our report adds to the understanding of these rare disease with early diagnosis and intervention.
Session topic: E-poster
Keyword(s): EBV
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