Aggressive NK-cell Leukemia

Written by: Wikipedia contributors

Summarized by: Vinh-Khoa Bao

Overview 

Aggressive NK-cell leukemia (ANKL) is a rare and rapidly progressing form of leukemia

involving natural killer (NK) cells, which are a component of the immune system responsible for killing infected and cancerous cells. It is classified by the World Health Organization as a type of mature T-cell and NK-cell neoplasm and is most often diagnosed in young to middle-aged adults, with a slight male predominance. The disease is strongly associated with the Epstein-Barr virus and is most common in East Asian populations.

Signs and Symptoms

Aggressive NK-cell leukemia usually comes with symptoms such as:

• Fever

• Fatigue

• Hepatosplenomegaly (enlarged liver and spleen)

• Lymphadenopathy (swollen lymph nodes)

• Skin rash

• Disseminated intravascular coagulation

• Hemophagocytic syndrome

Diagnosis

Diagnosis of ANKL involves identifying abnormal NK cells in blood, bone marrow, or other tissues using immunophenotyping, which identifies and classifies cells based on the specific antigens or markers present on their surface. These cells typically test positive for CD2, CD56, cytoplasmic CD3ε, and TIA-1, and negative for CD57.

Prognosis

The prognosis for Aggressive NK-cell leukemia is very poor, with a median survival time of only a few months. The disease is resistant to standard chemotherapy and rapidly progresses to multi-organ failure. Long-term survival for this disease is extremely rare, even with access to aggressive treatment.

Treatment

There is no established standard treatment for Aggressive NK-cell leukemia and normal chemotherapy regimens are generally ineffective. Some cases may respond to stem cell transplantation, but outcomes remain poor overall. Because ANKL is extremely rare, it is difficult to conduct large clinical trials. 

Epidemiology 

Aggressive NK-cell leukemia is rare globally but is seen more frequently in Asian countries, especially Japan, Korea, and China. The disease affects both males and females, typically in their 20s to 40s.

Conclusion

Aggressive NK-cell leukemia is a rare and extremely fatal disease that quickly progresses, has poor response to therapy, and a strong Epstein-Barr virus association. Due to its severity and lack of effective treatment options,  ANKL remains an area in need of greater clinical research and improved therapeutic strategies. 

Work Cited

Wikipedia. “Aggressive NK-cell leukemia.” Wikipedia, 2025, https://en.wikipedia.org/wiki/Aggressive_NK-cell_leukemia?utm_. Accessed 10 August 2025.