EBV Negative Aggressive NK-Cell Leukemia: Occurrence in Non-Asian Patients with a Uniformly Dismal Outcome
MT Howard, WG Morice, JP Maciejewski, N Bejanyan, ED Hsi. Mayo Clinic, Rochester, MN; Cleveland Clinic, Cleveland, OH
Background: Aggressive NK-cell leukemia (ANKL) is a rare NK-cell neoplasm almost exclusively found in Asian populations and which is typically Epstein-Barr virus (EBV) associated. EBV negative ANKL has been sporadically reported and it has been suggested that such cases have a relatively favorable prognosis. We report 4 patients from the midwestern United states with EBV negative ANKL.
Design: Records were reviewed at Cleveland Clinic and Mayo Clinic from 2003-2009 to find cases of EBV negative aggressive NK-cell leukemia.
Results: The 4 patients had a mean age of 38.5 years, and 3 were female. Splenomegaly was present in all patients. No patients were Asian. All patients followed an aggressive clinical course, and median survival time was 13 months (Table 1).
|Patient||Age (years)||Splenomegaly||Hepatomegaly||Absolute Lymphocyte Count||Absolute Neutrophil Count||Hgb||Survival (months)|