Secondary hemophagocytic lymphohistiocytosis syndrome in adults: A case series and review of the literature

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening condition characterized by immune dysregulation and benign proliferation of phagocytic cells, macrophages, and histiocytes. Lymphocytes (CD8 T-cells and natural killer cells) also play a major role in HLH. The ensuing cytokine storm and blood cells phagocytosis can lead to fatal multiple organ failure. So any diseases that can lead to activation of these cytokines can lead to hemophagocytic syndrome. 5 adult patients presented to Dr. Soliman Fakeeh Hospital, Jeddah, Saudi Arabia during 2014–2016 and fulfill the diagnostic criteria of HLH according to HLH-2004 diagnostic and therapeutic guidelines for HLH , have been included in this case series. Two out of five had viral infection, one patient was diagnosed with tuberculosis, and one patient developed secondary HLH in due to malignancy. The fifth patient did not have an identiable etiology. All of our patients presented with different symptoms and were diagnosed based on the standard approved criteria. Only two Out of five patients survived and remained disease free for a follow-up period of 24 months. As a conclusion, HLH syndrome is a rare condition with a high mortality rate. Aggressive treatment approach and early Intensive Care Unit admission is strongly recommended in patients with the cardinal diagnostic features of this condition.

Keywords

• Hemophagocytic lymphohistiocytosis syndrome
• hemophagocytic syndrome
• lymphohistiocytosis