ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Aggressive natural killer cell leukemia
Other Resources UpToDate PubMed

Aggressive natural killer cell leukemia

Contributors: Nina Haghi MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Aggressive natural killer leukemia (ANKL) is a proliferation of mature natural killer (NK) cells. It is very rare (fewer than 350 cases reported in the literature), with most cases described in young and middle-aged persons from China, Japan, and South America.

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

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:11/05/2020
Last Updated:11/23/2020
Copyright © 2021 VisualDx®. All rights reserved.
Aggressive natural killer cell leukemia
Print  
Aggressive natural killer cell leukemia
Copyright © 2021 VisualDx®. All rights reserved.