TY - JOUR
T1 - Hypertrophic Obstructive Cardiomyopathy in An Infant with Hemophagocytic Lymphohistiocytosis; Answer to a Riddle
AU - Rabah, Fatma
AU - Beshlawi, Ismail
AU - Wali, Yasser
AU - Al-Rawas, Abdulhakim
AU - Al Senaidi, Khalfan
N1 - Publisher Copyright:
Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2015/8/6
Y1 - 2015/8/6
N2 - 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.
AB - 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.
KW - FHLH
KW - HOCM
KW - hemophagocytic lymphohistiocytosis
KW - high dose corticosteroid therapy
KW - hypertrophic obstructive cardiomyopathy
KW - severe adverse event
UR - http://www.scopus.com/inward/record.url?scp=84938751697&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84938751697&partnerID=8YFLogxK
U2 - 10.1097/MPH.0000000000000257
DO - 10.1097/MPH.0000000000000257
M3 - Article
C2 - 25222062
AN - SCOPUS:84938751697
SN - 1077-4114
VL - 37
SP - 459
EP - 461
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
IS - 6
ER -