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Clinical Characteristics Treatment Approaches and Survival Outcomes in Secondary Hemophagocytic Lymphohistiocytosis:
Amratansh Varshney1, Pratibha Satish Phadke2, Anusree Prabhakaran3
1Senior Registrar, Department of Internal Medicine, Deenanath Mangeshkar Hospital, Pune, Maharashtra, India.
Background:
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal syndrome characterized by immune hyperactivation and a cytokine storm, leading to fever, cytopenia, organomegaly, and multiorgan dysfunction. Given its high morbidity and mortality, prompt diagnosis and treatment are essential. Currently, there are no established markers to predict outcomes in secondary hemophagocytic lymphohistiocytosis (sHLH).
Materials And Methods:
This retrospective study aimed to identify prognostic factors in patients with sHLH, specifically evaluating the prognostic role of absolute neutrophil count (ANC) and serum ferritin level. The study also examined the impact of dexamethasone and etoposide on the treatment of these patients.
Results:
HLH remission was achieved in 64 patients (71.1%). Forty-seven out of those 64 patients survived and were discharged home, while 17 patients died in the hospital. HLH remission was not achieved in 26 patients (28.9%), of whom 25 patients died in the hospital. ANC at diagnosis and lower nadir ANC during the hospital stay significantly correlated with mortality (p = 0.001 and 0.017, respectively). Serum ferritin levels, however, were not a significant prognostic marker (p = 0.396). Treatment with dexamethasone was associated with better outcomes in patients with sHLH.
Conclusion:
sHLH should be considered in patients with treatment-resistant bicytopenia or pancytopenia. Lower ANC at diagnosis and nadir ANC during hospitalization are associated with increased mortality, suggesting that ANC is a potential indicator for early immunochemotherapy. Serum ferritin level is not a significant prognostic marker. Early diagnosis and dexamethasone treatment help achieve HLH remission, which improves outcomes in sHLH. These findings highlight the need for prospective studies to better understand sHLH prognostic markers and therapeutic strategies, especially in intensive care settings, where mortality remains high.