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Hemophagocytic Lymphohistiocytosis [PDF]

Annual Review of Pathology: Mechanisms of Disease, 2018
Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of widespread inflammation due to massive amounts of cytokines released from activated macrophages. The most common trigger for HLH is infection from a virus, most commonly Epstein-Barr virus. Here we report an adult case of this rare and life-threatening syndrome.
Hanny Al-Samkari
exaly   +5 more sources

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Hemophagocytic lymphohistiocytosis

The Indian Journal of Pediatrics, 1999
Hemophagocytic lymphohistiocytosis (HLH) is a rare condition in children associated with immunodeficiency, life threatening infections and malignancy. Infection associated hemophagocytosis responds well to appropriate antimicrobioal therapy and rarely to steroids when the infective agent is suspected to be of viral origin.
N, Biswal, S, Shareef, P, Nalini, S, Srinivasan, D, Basu   +4 more
openaire   +4 more sources

Hemophagocytic Lymphohistiocytosis

AACN Advanced Critical Care, 2019
Hemophagocytic lymphohistiocytosis is a life-threatening condition associated with hyperinflammation and multiple organ dysfunction. It has many causes, symptoms, and outcomes. Early recognition is critical for treatment. Fever, cytopenias, coagulopathy, and hepatosplenomegaly are hallmark findings.
Janice, Skinner, Benedicta, Yankey, Brenda K, Shelton   +2 more
openaire   +2 more sources

Hemophagocytic Lymphohistiocytosis in Children

The Indian Journal of Pediatrics, 2020
To study the profile of children with Hemophagocytic Lymphohistiocytosis (HLH) in a tertiary care hospital for children.A retrospective analysis of case records of 52 children diagnossed with HLH was performed.Of the 52 children 13% (n = 7) had Familial HLH and 87% (n = 45) had secondary HLH (sHLH). Common manifestations were fever (100%), organomegaly
Divya Nandhakumar, Ajeitha Loganathan, Meena Sivasankaran, Somu Sivabalan, Deenadayalan Munirathnam   +4 more
openaire   +2 more sources

Hemophagocytic lymphohistiocytosis in adults

Leukemia & Lymphoma, 2018
Hemophagocytic lymphohistiocytosis (HLH), a rare but life-threatening condition characterized by uncontrolled inflammation, is increasingly recognized in adults. The management of adult onset HLH is challenging, in part due to gaps in current state of knowledge on etiology, clinical presentation, diagnosis, and management.
Neel S, Bhatt, Benjamin, Oshrine, Julie, An Talano   +2 more
openaire   +2 more sources

An Overview of Hemophagocytic Lymphohistiocytosis

Pediatric Annals, 2017
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by a dysregulated hyperinflammatory response associated with aberrant activation of lymphocytes and macrophages that results in hypercytokinemia. It is classically divided into two types: (1) primary or familial HLH and (2) secondary HLH.
Ysabella M, Esteban, Jill L O, de Jong, Melissa S, Tesher   +2 more
openaire   +2 more sources

Pathogenesis of Hemophagocytic Lymphohistiocytosis

Hematology/Oncology Clinics of North America, 2015
Hemophagocytic Lymphohistiocytosis (HLH), an inherited life-threatening inflammatory disorder, has gained growing recognition not only in children but also increasingly in adults over the past 2 decades. HLH involves inborn defects in lymphocytes, which normally mediate control of infectious and inflammatory conditions within the immune system and in ...
Alexandra H, Filipovich, Shanmuganathan, Chandrakasan   +1 more
openaire   +2 more sources

Hemophagocytic Lymphohistiocytosis in Adults

Hematology/Oncology Clinics of North America, 2015
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal syndrome of pathologic immune dysregulation characterized by clinical signs and symptoms of extreme inflammation. HLH can occur as a genetic or sporadic disorder and, though seen as an inherited condition affecting primarily a pediatric population, can occur at any age and can be ...
Meghan, Campo, Nancy, Berliner
openaire   +2 more sources

Treatment of hemophagocytic lymphohistiocytosis

Current Opinion in Hematology, 2017
Purpose of review Hemophagocytic lymphohistiocytosis (HLH) is a condition of uncontrolled immune activation with a high mortality rate. The recommended therapeutic guideline for HLH was published by the Histiocyte Society in 1994 and revised in 2004, which greatly improved the survival in patients with HLH.
Yini, Wang, Zhao, Wang
openaire   +2 more sources