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Aggressive NK-cell leukemia

ORPHA:86873· ICD-10 C94.7

Definition

An extremely rare and highly aggressive neoplasm, usually manifesting in the third to fourth decade of life, affecting males and females equally, and characterized by the onset of high fever, weight loss, jaundice, skin infiltration, lymphadenopathy, hepatosplenomegaly, and severe anemia. It has a fulminant and rapidly fatal disease course with the progressive appearance of multiorgan failure and disseminated intravascular coagulation.

Prevalence
Unknown
Inheritance
Multigenic/multifactorial, Not applicable
Age of onset
Adult