Hypertrophic Obstructive Cardiomyopathy in An Infant with Hemophagocytic Lymphohistiocytosis; Answer to a Riddle

Fatma Rabah; Ismail Beshlawi; Yasser Wali; Abdulhakim Al-Rawas; Khalfan Al Senaidi

doi:10.1097/MPH.0000000000000257

Hypertrophic Obstructive Cardiomyopathy in An Infant with Hemophagocytic Lymphohistiocytosis; Answer to a Riddle

Fatma Rabah^*, Ismail Beshlawi, Yasser Wali, Abdulhakim Al-Rawas, Khalfan Al Senaidi

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

1 Citation (Scopus)

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	https://doi.org/10.1097/MPH.0000000000000257
Publication status	Published - Aug 6 2015

Keywords

FHLH
HOCM
hemophagocytic lymphohistiocytosis
high dose corticosteroid therapy
hypertrophic obstructive cardiomyopathy
severe adverse event

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Hematology
Oncology

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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.",

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AU - Wali, Yasser

AU - Al-Rawas, Abdulhakim

AU - Al Senaidi, Khalfan

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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.

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KW - severe adverse event

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Hypertrophic Obstructive Cardiomyopathy in An Infant with Hemophagocytic Lymphohistiocytosis; Answer to a Riddle

Abstract

Keywords

ASJC Scopus subject areas

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