AGGRESSIVE
NK-CELL LEUKEMIA (ANKL) (previously called NK-cell leukemia/lymphoma,
recently characterized as aggressive NK-cell leukemia to emphasize the
predominance of the leukemic manifestations)
Epidemiology : occurs in young to middle
age adults and rarely childrenref1,
ref2,
ref3,
ref4,
ref5.
(Kwong YL, Chan ACL, Liang RHS. Natural killer cell lymphoma/leukemia:
pathology and treatment. Hematol Oncol. 1007;15:71-79)
Aetiology : there is variable association
with HHV-4 / EBVref1,
ref2 Symptoms & signs : fever, hepatosplenomegaly
and generalized lymphadenopathyref Laboratory examinations :
neutropenia, anemia and thrombocytopenia with high number of circulating
atypical large granular lymphocytes (some having a blastic appearance)
in the blood
large granular lymphocytes infiltrated in bone marrow aspirate and core
biopsy. The extent of marrow involvement tends to be patchy or minimal
compared to other leukemias. The tumor cells vary from mature-appearing
LGLs to cells with fine chromatin and more prominent nucleoli
immunophenotype: CD2+sCD3-cyCD3+11b+/-16+/-56+57-
no T-cell receptor (TCR) genes rearrangement
no unique karyotypic abnormality, sometimes del (6) (q21q25)
exclusion of other diseases with large granular lymphocytosis
Prognosis : these are very aggressive tumors
with median survival < 2 months
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