Recent Advances in the Diagnosis and Treatment of Natural Killer Cell Malignancies
Natural killer (NK)/T-cell lymphomas are aggressive malignancies. Epstein–Barr virus (EBV) infection in lymphoma cells is invariable. NK/T-cell lymphomas are divided into nasal, non-nasal, and disseminated subtypes. Nasal NK/T-cell lymphomas involve the nasal cavity and the upper aerodigestive tract...
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| Format: | Article |
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MDPI AG
2022-01-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/14/3/597 |
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| author | Eric Tse Yok-Lam Kwong |
| author_facet | Eric Tse Yok-Lam Kwong |
| author_sort | Eric Tse |
| collection | DOAJ |
| description | Natural killer (NK)/T-cell lymphomas are aggressive malignancies. Epstein–Barr virus (EBV) infection in lymphoma cells is invariable. NK/T-cell lymphomas are divided into nasal, non-nasal, and disseminated subtypes. Nasal NK/T-cell lymphomas involve the nasal cavity and the upper aerodigestive tract. Non-nasal NK/T-cell lymphomas involve the skin, gastrointestinal tract, testis and other extranodal sites. Disseminated NK/T-cell lymphoma involves multiple organs, rarely presenting with a leukaemic phase. Lymphoma cells are positive for CD3ε (not surface CD3), CD56, cytotoxic molecules and EBV-encoded small RNA. There is a predilection for Asian and Central/South American populations. Genome-wide association studies have identified lymphoma susceptibility loci in Asian patients. Positron emission tomography computed tomography and plasma EBV DNA quantification are crucial evaluations at diagnosis and follow-up. Stage I/II patients typically receive non-athracycline regimens containing asparaginse, together with sequential/concurrent radiotherapy. Anthracycline-containing regimens are ineffective. Stage III/IV patients are treated with asparaginase-containing regimens, followed by allogeneic haematopoietic stem cell transplantation (HSCT) in suitable cases. Autologous HSCT does not improve outcome. In relapsed/refractory patients, novel approaches are needed, involving PD1/PD-L1 targeting, EBV-specific cytotoxic T-cells, and monoclonal antibodies. Small molecules including histone deacetylase inhibitors may be beneficial in selected patients. Future strategies may include targeting of signalling pathways and driver mutations. |
| first_indexed | 2024-03-10T00:07:02Z |
| format | Article |
| id | doaj.art-3b3b06b4e3c347c18fae0617418b0224 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-10T00:07:02Z |
| publishDate | 2022-01-01 |
| publisher | MDPI AG |
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| series | Cancers |
| spelling | doaj.art-3b3b06b4e3c347c18fae0617418b02242023-11-23T16:05:30ZengMDPI AGCancers2072-66942022-01-0114359710.3390/cancers14030597Recent Advances in the Diagnosis and Treatment of Natural Killer Cell MalignanciesEric Tse0Yok-Lam Kwong1Department of Medicine, Queen Mary Hospital, Hong Kong, ChinaDepartment of Medicine, Queen Mary Hospital, Hong Kong, ChinaNatural killer (NK)/T-cell lymphomas are aggressive malignancies. Epstein–Barr virus (EBV) infection in lymphoma cells is invariable. NK/T-cell lymphomas are divided into nasal, non-nasal, and disseminated subtypes. Nasal NK/T-cell lymphomas involve the nasal cavity and the upper aerodigestive tract. Non-nasal NK/T-cell lymphomas involve the skin, gastrointestinal tract, testis and other extranodal sites. Disseminated NK/T-cell lymphoma involves multiple organs, rarely presenting with a leukaemic phase. Lymphoma cells are positive for CD3ε (not surface CD3), CD56, cytotoxic molecules and EBV-encoded small RNA. There is a predilection for Asian and Central/South American populations. Genome-wide association studies have identified lymphoma susceptibility loci in Asian patients. Positron emission tomography computed tomography and plasma EBV DNA quantification are crucial evaluations at diagnosis and follow-up. Stage I/II patients typically receive non-athracycline regimens containing asparaginse, together with sequential/concurrent radiotherapy. Anthracycline-containing regimens are ineffective. Stage III/IV patients are treated with asparaginase-containing regimens, followed by allogeneic haematopoietic stem cell transplantation (HSCT) in suitable cases. Autologous HSCT does not improve outcome. In relapsed/refractory patients, novel approaches are needed, involving PD1/PD-L1 targeting, EBV-specific cytotoxic T-cells, and monoclonal antibodies. Small molecules including histone deacetylase inhibitors may be beneficial in selected patients. Future strategies may include targeting of signalling pathways and driver mutations.https://www.mdpi.com/2072-6694/14/3/597NK/T-cell lymphomasEBVasparaginaseradiotherapyhaematopoietic stem cell transplantationPD1 |
| spellingShingle | Eric Tse Yok-Lam Kwong Recent Advances in the Diagnosis and Treatment of Natural Killer Cell Malignancies Cancers NK/T-cell lymphomas EBV asparaginase radiotherapy haematopoietic stem cell transplantation PD1 |
| title | Recent Advances in the Diagnosis and Treatment of Natural Killer Cell Malignancies |
| title_full | Recent Advances in the Diagnosis and Treatment of Natural Killer Cell Malignancies |
| title_fullStr | Recent Advances in the Diagnosis and Treatment of Natural Killer Cell Malignancies |
| title_full_unstemmed | Recent Advances in the Diagnosis and Treatment of Natural Killer Cell Malignancies |
| title_short | Recent Advances in the Diagnosis and Treatment of Natural Killer Cell Malignancies |
| title_sort | recent advances in the diagnosis and treatment of natural killer cell malignancies |
| topic | NK/T-cell lymphomas EBV asparaginase radiotherapy haematopoietic stem cell transplantation PD1 |
| url | https://www.mdpi.com/2072-6694/14/3/597 |
| work_keys_str_mv | AT erictse recentadvancesinthediagnosisandtreatmentofnaturalkillercellmalignancies AT yoklamkwong recentadvancesinthediagnosisandtreatmentofnaturalkillercellmalignancies |