Clinicopathological features and treatment of aggressive natural killer cell leukemia: case series and literature review.

Yongan Ni; Lei Li; Yuping Wang; Lirong Sun

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.

HEMATOPOIETIC stem cell transplantation; KILLER cells; HEMOPHAGOCYTIC lymphohistiocytosis; BONE marrow

Turkish Journal of Pediatrics, 2024, Vol 66, Issue 4, p481

0041-4301

Academic Journal

10.24953/turkjpediatr.2024.5072