Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review
Background. Aggressive natural killer cell leukemia (ANKL) is rare and difficult to diagnose in early stages, with no standard treatment and a poor prognosis. Case presentation. Two adolescents with ANKL presented with hemophagocytic lymphohistiocytosis (HLH), with Case-1 presenting as refractory...
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| Language: | English |
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Hacettepe University Institute of Child Health
2024-10-01
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| Series: | The Turkish Journal of Pediatrics |
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| Online Access: | https://turkjpediatr.org/article/view/5072 |
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| author | Yongan Ni Lei Li Yuping Wang Lirong Sun |
| author_facet | Yongan Ni Lei Li Yuping Wang Lirong Sun |
| author_sort | Yongan Ni |
| collection | DOAJ |
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Background. Aggressive natural killer cell leukemia (ANKL) is rare and difficult to diagnose in early stages, with no standard treatment and a poor prognosis.
Case presentation. Two adolescents with ANKL presented with hemophagocytic lymphohistiocytosis (HLH), with Case-1 presenting as refractory HLH and Case-2 with lung involvement. The morphology of bone marrow showed an increase in unidentified cells, which mainly expressed CD56. Cytogenetic analysis showed complex karyotypes. Both patients received intensive combined chemotherapy based on pegaspargase and anthracyclines. Case-1 died of tumor lysis syndrome. Case-2 underwent hematopoietic stem cell transplantation and is currently alive and disease-free.
Conclusions. HLH can serve as the initial manifestation of ANKL. Leukemia cells of ANKL have significant variations in the morphology and mainly express CD56. Intensive combination chemotherapy based on pegaspargase and anthracyclines may be considered for ANKL.
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| format | Article |
| id | doaj-art-e4d3c454b72341d4ba075fe7cc786e7c |
| institution | DOAJ |
| issn | 0041-4301 2791-6421 |
| language | English |
| publishDate | 2024-10-01 |
| publisher | Hacettepe University Institute of Child Health |
| record_format | Article |
| series | The Turkish Journal of Pediatrics |
| spelling | doaj-art-e4d3c454b72341d4ba075fe7cc786e7c2025-08-20T02:55:20ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212024-10-0166410.24953/turkjpediatr.2024.5072Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature reviewYongan Ni0https://orcid.org/0000-0003-0793-7093Lei Li1https://orcid.org/0009-0005-3231-1027Yuping Wang2https://orcid.org/0009-0006-2900-9967Lirong Sun3https://orcid.org/0000-0002-5035-7601Department of Pediatric Hematology and Oncology, the Affiliated Hospital of Qingdao University, Qingdao, Shandong, ChinaDepartment of Respiratory & Cardiovascular Pediatrics, the Affiliated Hospital of Qingdao University, Qingdao, Shandong, ChinaDepartment of Pediatrics, Qingdao Women and Children’s Hospital, Qingdao, Shandong, ChinaDepartment of Pediatric Hematology and Oncology, the Affiliated Hospital of Qingdao University, Qingdao, Shandong, China Background. Aggressive natural killer cell leukemia (ANKL) is rare and difficult to diagnose in early stages, with no standard treatment and a poor prognosis. Case presentation. Two adolescents with ANKL presented with hemophagocytic lymphohistiocytosis (HLH), with Case-1 presenting as refractory HLH and Case-2 with lung involvement. The morphology of bone marrow showed an increase in unidentified cells, which mainly expressed CD56. Cytogenetic analysis showed complex karyotypes. Both patients received intensive combined chemotherapy based on pegaspargase and anthracyclines. Case-1 died of tumor lysis syndrome. Case-2 underwent hematopoietic stem cell transplantation and is currently alive and disease-free. Conclusions. HLH can serve as the initial manifestation of ANKL. Leukemia cells of ANKL have significant variations in the morphology and mainly express CD56. Intensive combination chemotherapy based on pegaspargase and anthracyclines may be considered for ANKL. https://turkjpediatr.org/article/view/5072aggressive NK cell leukemiaasparaginaseanthracyclineshemophagocytic lymphohistiocytosis |
| spellingShingle | Yongan Ni Lei Li Yuping Wang Lirong Sun Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review The Turkish Journal of Pediatrics aggressive NK cell leukemia asparaginase anthracyclines hemophagocytic lymphohistiocytosis |
| title | Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review |
| title_full | Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review |
| title_fullStr | Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review |
| title_full_unstemmed | Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review |
| title_short | Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review |
| title_sort | clinicopathological features and treatment of aggressive natural killer cell leukemia case series and literature review |
| topic | aggressive NK cell leukemia asparaginase anthracyclines hemophagocytic lymphohistiocytosis |
| url | https://turkjpediatr.org/article/view/5072 |
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