Abstract
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. ©
Original language | English |
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Pages (from-to) | 459-461 |
Number of pages | 3 |
Journal | Journal of Pediatric Hematology/Oncology |
Volume | 37 |
Issue number | 6 |
DOIs | |
Publication status | Published - Aug 6 2015 |
Keywords
- FHLH
- hemophagocytic lymphohistiocytosis
- high dose corticosteroid therapy
- HOCM
- hypertrophic obstructive cardiomyopathy
- severe adverse event
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Oncology
- Hematology
- Medicine(all)