Familial hemophagocytic lymphohistiocytosis (FHLH) is a hereditary hyperinflammatory condition with T-cell and macrophage activation. Treatment consists of immunosuppressive therapy plus bone marrow transplantation. Cardiac manifestations of FHLH were scarcely mentioned in the literature with conflicting pathophysiological explanations. We report a case of hypertrophic obstructive cardiomyopathy associated with FHLH. Guided by such a case, a clear vision regarding the real cause is thought to be obtained in the cloudy landscape of pathophysiology.
- hemophagocytic lymphohistiocytosis
- high dose corticosteroid therapy
- hypertrophic obstructive cardiomyopathy
- severe adverse event
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health