title: Hypertrophic Obstructive Cardiomyopathy in An Infant With Hemophagocytic Lymphohistiocytosis; Answer to a Riddle.
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.
authors.affiliation: Department of Child Health, Sultan Qaboos University Hospital (SQUH), Muscat, Oman.
authors.forename: Fatma
authors.initials: F
authors.lastname: Rabah
authors.forename: Ismail
authors.initials: I
authors.lastname: Beshlawi
authors.forename: Yasser
authors.initials: Y
authors.lastname: Wali
authors.forename: Abdulhakim
authors.initials: A
authors.lastname: Al-Rawas
authors.forename: Khalfan
authors.initials: K
authors.lastname: Al Senaidi
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000473:pathology / Q000628:therapy; D003131:Combined Modality Therapy; D017809:Fatal Outcome; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000473:pathology / Q000628:therapy