Difficulties in Diagnosis of Hemophagocytic Lymphohistocytosis: Overdiagnosis or Underdiagnosis; A Case Report

Hemophagocytic lymphohistocytosis (hlh) is a potentially life threatening condition occurring as a familial disease (fhlh) or secondary to marked immunological activation during viral, bacterial and parasitic infections, malignancies, rheumatologic conditions or immune deficiencies with cytotoxic t and/or nk-cell dysfunction 1, 2. although fhlh is an autosomal recessive disease that affects immune regulation, sporadic cases with no obvious family inheritance occur1. all organ systems might be affected in hlh.