SHM Converge Scientific Abstract ePoster Gallery-Hemophagocytic Lymphohistiocytosis (Hlh) Following Treatment With Trimethoprim/Sulfamethoxazole

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This case report describes a rare episode of hemophagocytic lymphohistiocytosis (HLH) likely triggered by trimethoprim/sulfamethoxazole (TMP/SMX). HLH is a severe hyperinflammatory syndrome caused by overactivation of macrophages and excessive cytokine release. It is usually secondary to malignancy, infection, or autoimmune disease, but medications are an uncommon cause.<br /><br />A 31-year-old man with no significant past medical history presented with high fever, chills, night sweats, and rash one week after incision and drainage of a perirectal abscess, for which he had been started on TMP/SMX. On evaluation, he was found to have pancytopenia, markedly elevated inflammatory markers, acute kidney injury, transaminitis, coagulopathy, and hepatosplenomegaly. Bone marrow aspirate showed hemophagocytosis, supporting the diagnosis of HLH.<br /><br />Because he appeared nontoxic clinically, he was treated with dexamethasone alone rather than etoposide-based therapy. He improved after steroid treatment and completed a five-month taper. His HLH laboratory abnormalities resolved, aside from mild indirect hyperbilirubinemia.<br /><br />The authors emphasize that medication-induced HLH is rare and poorly understood, and that atypical causes should be considered when no evidence of malignancy, infection, or autoimmune disease is found. This case suggests that steroids alone may be effective in selected, clinically stable patients with suspected drug-induced HLH. More research is needed to clarify the mechanism and best treatment approach for medication-associated HLH.

Asset Subtitle

Adam Zimilover

Meta Tag

Author List Adam Zimilover, Isaac Huml

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Medication-Induced Hemophagocytic Lymphohistiocytosis

Concept Fever

Concept Cytopenia

Concept Pancytopenia

Distinguished Non-Finalist

Presenter Organization Zucker School of Medicine at Hofstra University

Presenting Author Adam Zimilover

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

trimethoprim-sulfamethoxazole

TMP/SMX

drug-induced HLH

pancytopenia

hemophagocytosis

dexamethasone

hyperinflammatory syndrome

hepatosplenomegaly

Hemophagocytic Lymphohistiocytosis

Medication-Induced Hemophagocytic Lymphohistiocytosis

Fever

Cytopenia

Pancytopenia