SHM Converge Scientific Abstract ePoster Gallery-Spotted: Secondary Hemophagocytic Lymphohistiocytosis Due to Rocky Mountain Spotted Fever

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This document describes a rare case of hemophagocytic lymphohistiocytosis (HLH) caused by Rocky Mountain spotted fever (RMSF). HLH is a syndrome of severe immune overactivation that can be secondary to infectious, malignant, or rheumatologic triggers. Typical findings include fever, hepatosplenomegaly, cytopenias, elevated ferritin, and hemophagocytosis on biopsy. Secondary HLH is treated by addressing the underlying cause, but if a patient worsens, the HLH-94 regimen with dexamethasone and etoposide is recommended.<br /><br />RMSF is caused by Rickettsia rickettsii and is transmitted most often by the American dog tick. It is a rapidly progressive and potentially fatal illness that causes vascular injury and increased permeability through endothelial activation. Common symptoms include fever, headache, and petechial rash involving the palms and soles, while more severe disease can lead to altered mental status, respiratory failure, and multiorgan dysfunction. Typical laboratory abnormalities include thrombocytopenia, transaminitis, leukocytosis, and hyponatremia. Doxycycline is the treatment of choice.<br /><br />The key point of the report is that although rickettsial infections can trigger HLH, HLH secondary to RMSF is very rarely reported. The authors emphasize that both RMSF and HLH can be life-threatening, making early recognition and treatment essential.

Asset Subtitle

Pinar Arikan

Meta Tag

Author List Bridget B. Hottenstein, Pinar Arikan

Category Clinical Vignettes

Concept Hemophagocytic Lymphohistiocytosis

Concept Secondary hemophagocytic lymphohistiocytosis

Concept Doxycycline

Concept Immune Overactivation

Concept Rocky Mountain Spotted Fever

Distinguished Non-Finalist

Presenter Organization The Miriam Hospital

Presenting Author Pinar Arikan

Track Adult

Keywords

hemophagocytic lymphohistiocytosis

HLH

Rocky Mountain spotted fever

RMSF

Rickettsia rickettsii

doxycycline

fever

thrombocytopenia

petechial rash

dexamethasone etoposide

Hemophagocytic Lymphohistiocytosis

Secondary hemophagocytic lymphohistiocytosis

Doxycycline

Immune Overactivation

Rocky Mountain Spotted Fever