Open AccessHemophagocytic lymphohistiocytosis in an infant with multiple triggers!!
Author(s) -
Sataroopa Mishra,
Amitabh Singh,
Lavleen Singh,
Anirban Mandal,
Rahul Jain
Publication year - 2018
Publication title -
asian journal of transfusion science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.262
H-Index - 22
eISSN - 1998-3565
pISSN - 0973-6247
DOI - 10.4103/ajts.ajts_142_16
Subject(s) - hemophagocytic lymphohistiocytosis , medicine , cytokine storm , immunology , immune system , intensive care medicine , pediatrics , immune dysregulation , covid-19 , disease , infectious disease (medical specialty)
Hemophagocytic lymphohistiocytosis (HLH) is a disorder of immune dysregulation secondary to a massive unregulated cytokine storm and its downstream consequences. HLH is being increasingly recognized as a cause of pyrexia of unknown origin, unexplained cytopenias, and hepatic dysfunction. However, this potentially treatable condition is often missed due to lack of suspicion, variable, and nonspecific presentations, inability to fulfil all the diagnostic criteria and availability of diagnostic tests in resource limited settings. Both familial and acquired forms of HLH can be triggered by multiple factors in a susceptible patient. We report a 9-month old infant who developed HLH in association with Stevens-Johnson syndrome following massive blood transfusion.