Key result
HLH-directed immunosuppression prompts complete recovery of LV function and sinus node activity by day 10.
Why the study?
Cardiac conduction-system abnormalities in hemophagocytic lymphohistiocytosis are exceedingly rare and poorly described.
Does HLH-directed immunosuppressive therapy improve cardiomyopathy and sinus node dysfunction in a patient with HLH?
Case Report (n=1)
Does HLH-directed immunosuppressive therapy improve cardiomyopathy and sinus node dysfunction in a patient with HLH?
Severe sinus node dysfunction and cardiomyopathy can present as reversible manifestations of HLH-related cytokine storm that fully resolve with prompt immunosuppressive therapy.
HLH may feature transient cardiomyopathy with sinus arrest; extends cardiac phenotype but remains hypothesis-generating.
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammation syndrome causing multiorgan failure. While cardiac involvement is recognized, associated conduction-system abnormalities are exceedingly rare and poorly described. CASE SUMMARY: A 60-year-old male presented with fever, pancytopenia, and progressive shock, meeting diagnostic criteria for HLH. His clinical course was acutely complicated by transient cardiomyopathy and frequent episodes of sinus arrest. Following the initiation of HLH-directed therapy, the patient experienced a complete recovery of both systolic function and sinus node conduction by day 10. DISCUSSION: This case identifies severe sinus node dysfunction as a reversible manifestation of HLH-related cytokine storm. Prompt initiation of immunosuppressive therapy led to complete recovery of left ventricular function and sinus node activity, highlighting the reversible nature of the HLH-related myocardial injury. TAKE-HOME MESSAGE: This case highlights the importance of early identification of HLH-related cardiac involvement as it is critical to prevent irreversible damage and improve outcomes in these patients.
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Nunez et al. (2026) conducted a case report in Hemophagocytic lymphohistiocytosis (n=1). HLH-directed immunosuppressive therapy was evaluated on Recovery of systolic function and sinus node conduction. HLH-directed immunosuppressive therapy led to complete recovery of left ventricular function and sinus node activity by day 10 in a patient with HLH-related cytokine storm.
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