Infection-associated haemophagocytic lymphohistiocytosis: a case series using steroids only protocol for management

• DOI: 10.1007/s00296-011-2291-2
• Publication Date: 2011-12-22T05:10:14Z
• Authors: Prabhas Prasun Giri; Priyankar Pal; Apurba Ghosh; Rajiv Sinha
• Keywords: Male; Adolescent; India; Infant; Communicable Diseases; Dexamethasone; Lymphohistiocytosis, Hemophagocytic; 3. Good health; 03 medical and health sciences; Treatment Outcome; 0302 clinical medicine; Child, Preschool; Humans; Female; Steroids; Child

Haemophagocytic lymphohistiocytosis (HLH) is a heterogeneous group of clinical syndromes characterised by activation and subsequent uncontrolled non-malignant proliferation of T lymphocytes and macrophages, leading to a cytokine storm that accounts for most of its clinical features such as acute febrile illness, hepatosplenomegaly, multi-organ dysfunction and fulminant pancytopenia-resembling severe sepsis. Here, we present a series of 23 cases of infection-associated HLH diagnosed in our hospital within a time period of last three and half years. Though the presentation and progression of disease was variable, the patients shared some common features like prolonged fever unresponsive to broad spectrum antibiotics, organomegaly and cytopenias. In most of the cases, however, the triggering infectious agent could not be identified. They were treated using a steroid only protocol along with supportive measures and showed an excellent response.