Epstein–Barr virus and hemophagocytic lymphohistiocytosis

RA Marsh - Frontiers in immunology, 2018 - frontiersin.org
Epstein–Barr virus (EBV) is a ubiquitous virus that infects nearly all people worldwide
without serious sequela. However, for patients who have genetic diseases which predispose …

Advances in the pathogenesis of primary and secondary haemophagocytic lymphohistiocytosis: differences and similarities

E Brisse, CH Wouters, P Matthys - British Journal of …, 2016 - Wiley Online Library
Haemophagocytic lymphohistiocytosis (HLH) comprises a heterogeneous spectrum of
hyperinflammatory conditions that are inherited (primary HLH) or acquired in a context of …

Neuroinflammatory disease as an isolated manifestation of hemophagocytic lymphohistiocytosis

A Blincoe, M Heeg, PK Campbell, M Hines… - Journal of Clinical …, 2020 - Springer
Isolated neuroinflammatory disease has been described in case reports of familial
hemophagocytic lymphohistiocytosis (FHL), but the clinical spectrum of disease …

Pediatric CNS-isolated hemophagocytic lymphohistiocytosis

LA Benson, H Li, LA Henderson… - Neurology …, 2019 - neurology.org
Objective To highlight a novel, treatable syndrome, we report 4 patients with CNS-isolated
inflammation associated with familial hemophagocytic lymphohistiocytosis (FHL) gene …

Frequency and spectrum of disease-causing variants in 1892 patients with suspected genetic HLH disorders

V Gadoury-Levesque, L Dong, R Su, J Chen… - Blood …, 2020 - ashpublications.org
This article explores the distribution and mutation spectrum of potential disease-causing
genetic variants in hemophagocytic lymphohistiocytosis (HLH)–associated genes observed …

Malignancy and chemotherapy induced haemophagocytic lymphohistiocytosis in children and adolescents—a single centre experience of 20 years

V Strenger, G Merth, H Lackner, SW Aberle… - Annals of …, 2018 - Springer
Haemophagocytic lymphohistiocytosis (HLH) is a possibly life-threatening syndrome of
immune dysregulation and can be divided into primary (hereditary) and secondary forms …

[HTML][HTML] Perforin 1 in cancer: mechanisms, therapy, and outlook

X Guan, H Guo, Y Guo, Q Han, Z Li, C Zhang - Biomolecules, 2024 - mdpi.com
PRF1 (perforin 1) is a key cytotoxic molecule that plays a crucial role in the killing function of
natural killer (NK) cells and cytotoxic T lymphocytes (CTLs). Recent studies have focused on …

[HTML][HTML] Severe adult hemophagocytic lymphohistiocytosis (HLHa) correlates with HLH-related gene variants

C Bloch, JP Jais, M Gil, M Boubaya… - Journal of Allergy and …, 2024 - Elsevier
Background The contribution of genetic factors to the severity of adult hemophagocytic
lymphohistiocytosis (HLHa) remains unclear. Objective We sought to assess a potential link …

Immunodeficiency with susceptibility to lymphoma with complex genotype affecting energy metabolism (FBP1, ACAD9) and vesicle trafficking (RAB27A)

N Brauer, Y Maruta, M Lisci, K Strege… - Frontiers in …, 2023 - frontiersin.org
Introduction Inborn errors of immunity (IEI) are characterized by a dysfunction of the immune
system leading to increased susceptibility to infections, impaired immune regulation and …

Haemophagocytic lymphohistiocytosis complicating pembrolizumab treatment for metastatic breast cancer in a patient with the PRF1A91V gene polymorphism

H Al-Samkari, GD Snyder, S Nikiforow… - Journal of medical …, 2019 - jmg.bmj.com
Background Immune checkpoint inhibitor therapy is a modern breakthrough in medical
oncology, but it can precipitate inflammatory and autoimmune adverse effects. Among the …