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Secondary hemophagocytic lymphohistiocytosis in children (Review).


ABSTRACT: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition characterized by hyperinflammation in an uncontrolled and ineffective immune response. Despite great improvement in diagnosis and treatment, it still represents a challenge in clinical management, with poor prognosis in the absence of an aggressive therapeutic approach. The present literature review focuses on secondary HLH at pediatric age, which represents a heterogeneous group in terms of etiology and therapeutic approach. It summarizes the most recent evidence on epidemiology, pathophysiology, diagnosis, treatment and prognosis, and provides a detailed description and comparison of the major subtypes of secondary HLH. Finally, it addresses the open questions with a focus on diagnosis and new treatment insights.

SUBMITTER: Benevenuta C 

PROVIDER: S-EPMC10433411 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature

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Secondary hemophagocytic lymphohistiocytosis in children (Review).

Benevenuta Chiara C   Mussinatto Ilaria I   Orsi Cecilia C   Timeus Fabio S FS  

Experimental and therapeutic medicine 20230717 3


Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition characterized by hyperinflammation in an uncontrolled and ineffective immune response. Despite great improvement in diagnosis and treatment, it still represents a challenge in clinical management, with poor prognosis in the absence of an aggressive therapeutic approach. The present literature review focuses on secondary HLH at pediatric age, which represents a heterogeneous group in terms of etiology and therapeutic a  ...[more]

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