ObjectiveChronic lymphoproliferative disorder of NK cells(CLPD-NK), which is a malignant lymphoproliferative disorders that derived from mature NK cell, is the new disease type of the recently updated World Health Organization classification (WHO 2008), and it is extremely rare in clinical findings. The typical immuno-phenotype is CD2+, sCD3-, CD16+, with weak expression of CD56, and the cytotoxic molecules,such as TIA, granzyme B and granzyme M are positive. Generally, it is not complicated with Epstein-Barr virus infection. The clinical courses of most patients are inert, without any symptoms. Few patients can transform into aggressive NK-cell leukemia, which was known as chronic NK large granular lymphocyte leukemia in the past. Since it is a rare disease, and is the new type of recently updated WHO classification, we reportacase that our hospital diagnosed and review of literature. |