Aggressive natural killer cell leukemia
Alerts and Notices
Synopsis
ANKL is almost always associated with Epstein-Barr virus (EBV) infection. Hemophagocytic lymphohistiocytosis (HLH) is reported in 60% of cases.
The main sites of involvement include the liver and spleen, the bone marrow and peripheral blood, and, less frequently, the nose and skin. Clinically, these patients commonly present with fever and liver dysfunction with a rapidly progressive course.
ANKL has high mortality (median length of survival is approximately 8 weeks).
Codes
ICD10CM:C94.80 – Other specified leukemias not having achieved remission
SNOMEDCT:
721310007 – Aggressive natural killer-cell leukemia
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Hemophagocytic syndrome
- Disseminated intravascular coagulation
- Other types of leukemia
- Extranodal NK/T-cell lymphoma
- Other EBV-associated lymphomas
Best Tests
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Management Pearls
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Therapy
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References
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Last Reviewed:11/05/2020
Last Updated:11/23/2020
Last Updated:11/23/2020