Spontaneous Hepatic Rupture Associated With Epstein-Barr Virus Negative Aggressive Natural Killer Cell Leukemia

Aggressive natural killer cell leukemia (ANKL) is a rare subtype of large granular lymphocyte (LGL) leukemia, which typically presents in young adults of Asian descent. It is an aggressive disease, characterized initially by fever, pancytopenia and hepatosplenomegaly, which rapidly progresses to organ failure and death over the course of months. Spontaneous hemorrhagic complications have been reported to occur in ANKL in a handful of case reports, including lethal intestinal and cerebral hemorrhage as well as splenic rupture. Here, we present a case of a 49-year-old man with Epstein-Barr virus (EBV)-negative ANKL who developed fatal spontaneous hepatic rupture approximately 4 months after initial diagnosis. To the best of our knowledge, this is first reported case of hepatic rupture associated with ANKL.