Hemophagocytic lymphohistiocytosis: Uncommon systemic inflammatory clinical syndrome

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation characterized by clinical signs and symptoms of extreme inflammation. HLH can be challenging to diagnose because the initial symptoms may mimic common infections. People with HLH usually develop symptoms within the first months or years of life which may include fever, enlarged liver or spleen, cytopenia and neurological abnormalities. HLH can only be diagnosed with the proper blood tests. A sample of bone marrow may be obtained to look for hemophagocytosis. Treatment depends on a number of factors, including the severity of symptoms, onset of age, and the underlying cause of the condition. Patients are usually treated with steroids plus chemotherapy and / or an antibody therapy that destroys the T cells. Patients may receive other medications that suppress the immune system. Many patients must also have their immune systems replaced by means of a bone marrow transplant in order to be cured of HLH. We report the brief description of a case along with treatment algorithm.

Keywords

• cytopenia
• hemophagocytosis
• hemophagocytic lymphohistiocytosis