From Fever to Resolution: Successful Management of Secondary Hemophagocytic Lymphohistiocytosis with Dexamethasone Monotherapy in a ResourceLimited Setting – A Case Report

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, lifethreatening hyperinflammatory syndrome characterized by uncontrolled immune activation, leading to cytokine storm, multiorgan dysfunction, and high mortality if untreated. Secondary HLH in adults is often triggered by infections, malignancies, or autoimmune conditions, presenting with nonspecific symptoms such as persistent fever, cytopenias, and organomegaly, which pose significant diagnostic challenges in resource limited settings. This case report describes a 35yearold male from Panchkhal, Nepal, who presented with prolonged high grade fever initially misdiagnosed as enteric fever and later complicated by hospital acquired pneumonia. Despite broad spectrum antibiotics, symptoms persisted, prompting transfer to Dhulikhel Hospital. Clinical examination revealed splenomegaly, bicytopenia, and respiratory distress requiring intensive care and mechanical ventilation. Laboratory findings included marked hyperferritinemia, hypertriglyceridemia, and evidence of hemophagocytosis on bone marrow aspiration, fulfilling ≥5 HLH2004 diagnostic criteria, supporting a diagnosis of secondary HLH likely infection triggered.

Standard treatment per HLH2004 protocol involves dexamethasone and etoposide, with cyclosporine in select cases. However, due to financial constraints and limited availability of etoposide in this resource limited setting, dexamethasone monotherapy was initiated (10 mg/m²/day initially, tapered over weeks). The patient showed rapid clinical improvement: fever resolved within days, cytopenias corrected, and organ function normalized. He was extubated, weaned off support, and discharged after full recovery without relapse on followup.

This case highlights the diagnostic hurdles of HLH in low resource environments, where advanced tests (e.g., soluble CD25, NK cell activity, genetic profiling) are unavailable. It also demonstrates that dexamethasone monotherapy can achieve complete remission in select adult secondary HLH cases, particularly infection associated forms, avoiding the toxicity and cost of etoposide. In regions like Nepal, where HLH remains underreported and mortality high due to delayed diagnosis, this approach may offer a feasible alternative, warranting further prospective studies to validate efficacy and identify predictors of response.