Description of the disease * EnglishPolish Pobierz sekcję do PDF 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. Disease data Classification Disease Synonyms ANKCL Agresywny chłoniak z komórek NK ANKCL Białaczka limfocytarna z dużych ziarnistych komórek NK NK-cell LGL leukemia Aggressive NK-cell lymphoma NK-cell LGL leukemia NK-cell large granular lymphocyte leukemia ORPHA code 86873 OMIM code - ICD10 code C94.7 ICD11 code 2A90.3 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl