A Case Study of Refractory Secondary HLH treated with Splenectomy in an Adult Patient

Document Type

Conference Proceeding

Publication Date

5-2026

Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) in adults is a rare disease marked by inappropriate immune activation resulting in pancytopenia. This inappropriate immune activation is attributed to aberrant T-lymphocyte and macrophage activation resulting in phagocytosis of blood cells. Surgical splenectomy has been recommended for establishing diagnosis especially in cases in which diagnosis is not confirmed on bone marrow biopsy, but the therapeutic effect of splenectomy in HLH remains unclear Case: We present the case of a 60-year-old female who presented with recurrent fevers as high as 105F, and pancytopenia found to have hemophagocytic lymphohistiocytosis (HLH). She was treated with dexamethasone, IVIG, etoposide, and anakinra. A bone marrow biopsy was obtained but was without evidence of lymphophagocytosis. Repeat imaging showed worsening splenomegaly up to 21 cm in size and a spontaneous psoas hematoma. Given failure of improvement despite medical investigation and intervention, the patient was taken for a robotic-assisted laparoscopic splenectomy. Postoperatively, tocilizumab was started; however, the patient remained without improvement and thus was transferred to a tertiary care center where she ultimately expired. Discussion: HLH is a hyperinflammatory condition that can ultimately lead to multi-organ failure and death. Treating the underlying etiology while managing the patients inflammatory state remains the hallmark of treating this disease. Unfortunately, there is a large percentage of cases in which an underlying etiology cannot be identified, making treatment more complex especial in refractory cases of secondary HLH like the patient presented here. Splenectomy is known to play a role in diagnosis; however, its therapeutic benefit is not well understood. Refractory cases of secondary HLH remain difficult to diagnose and treat. It comprises an extensive list of differential diagnosis which need to be systematically assessed. In cases that are not responding to medical therapy alone, splenectomy can be considered for further diagnostic potential, but the therapeutic benefit is still debated.

Comments

This abstract was published in Surgical Endoscopy, Volume 40, Issue 2 Supplement.

The published version is available at dx.doi.org/10.1007/s00464-026-12851-0.

Copyright © 2026.

Publication Title

Surgical Endoscopy

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