Aggressive natura killer cell leukemia presenting with hemophagocytic lymphohistiocytosis

Center for Children's Cancer & Blood Disorders, Sydney Children's Hospital, Randwick, New South Wales, Australia.
Pediatric Blood & Cancer (Impact Factor: 2.39). 03/2008; 50(3):654-7. DOI: 10.1002/pbc.21358
Source: PubMed


Aggressive natural killer cell leukemia (ANKL) is a very rare condition and when reported occurs almost exclusively in adults. We report a pediatric case of ANKL that presented with hemophagocytic syndrome, preceding the onset of leukemia by 12 weeks. Clinical and laboratory findings are discussed, along with morphology, immunophenotyping and cytogenetics, as well as the association with Epstein-Barr virus (EBV). This case is noteworthy for the expression of CD8 on the malignant cells, the cytogenetic findings that include abnormalities of chromosomes 6 and 7, as well as the age of the patient.

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    • "This hypothesis is mainly based on the similar ethnic background (Asian), morphology, immunophenotype, and genotype (lack of T-cell receptor gene rearrangements) of both entities. Moreover, EBV is detected in over 75% of aggressive NK-cell leukemia cases and near 100% in nasal NK/T cell lymphoma [3] [4]. "
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