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 |
|---|---|
| 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 |
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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)
Cite this
Hypertrophic Obstructive Cardiomyopathy in An Infant with Hemophagocytic Lymphohistiocytosis; Answer to a Riddle. / Rabah, Fatma; Beshlawi, Ismail; Wali, Yasser; Al-Rawas, Abdulhakim; Al Senaidi, Khalfan.
In: Journal of Pediatric Hematology/Oncology, Vol. 37, No. 6, 06.08.2015, p. 459-461.Research output: Contribution to journal › Article
}
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
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 - hemophagocytic lymphohistiocytosis
KW - high dose corticosteroid therapy
KW - HOCM
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
VL - 37
SP - 459
EP - 461
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
SN - 1077-4114
IS - 6
ER -