Case Report: A role for hemoadsorption in hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory syndrome. HLH can occur as a primary (familial) or secondary (acquired) disorder. Secondary HLH (sHLH) manifests in response to infection, malignancy, autoimmune disorders or drugs. Presentation is usually non-specific, with fever, cytopenias, elevated inflammatory markers and hepatosplenomegaly. A high level of suspicion coupled with the use of diagnostic scores (HScore and HLH-2004) is required for timely diagnosis. Specific studies such as bone marrow biopsy or soluble interleukin-2 (IL-2) receptor (CD25) levels are necessary for distinguishing HLH from other conditions, but should not delay treatment. Corticosteroids, in addition to other immunossupressants, should be initiated promptly in order to reduce morbidity and mortality. In secondary cases, treatment of underlying causes is necessary for complete remission. Long-term immunossupression may be required beyond resolution of the HLH trigger to avoid relapses. We report a case of severe acute HLH in a patient with distributive and cardiogenic shock and multiorgan dysfunction. The patient was successfully treated with CytoSorb^® hemoadsorption therapy. This case highlights diagnostic challenges, therapeutic interventions, and the potential role of hemoadsorption therapy as a complementary treatment option for HLH.