ArticlePloS one2014
Genetic features of late onset primary hemophagocytic lymphohistiocytosis in adolescence or adulthood.
Article in PloS one, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 47 papers.
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Who cites it
47 citing papers in PubMed, 90 citations in OpenAlex.
- Adult Onset of Type 2 Familial Hemophagocytic Lymphohistiocytosis After SARS-CoV-2 Vaccination with an Unusual Neurological Onset: The Great Mimic.Diagnostics (Basel, Switzerland) · 2025Article
- Late-onset hemophagocytic lymphohistiocytosis associated with monoallelic STX11 mutation in an adult: a case report and rationale for early allogeneic hematopoietic cell transplantation.Annals of hematology · 2025Article
- Germline defects of familial haemophagocytic lymphohistiocytosis-Related genes may represent a predisposing factor for mature T- and natural killer-cell lymphoma.British journal of haematology · 2025Article
- Article
- Clinicopathological and Immunogenetic Characterization in 8 Patients with Familial Hemophagocytic Lymphohistiocytosis Type 2: A Study from North India with Literature Review.Journal of clinical immunology · 2025Review
- Beyond genotype: challenges in predicting disease risk for carriers of biallelic perforin variants.Blood · 2025Article
- Case Report: Pediatric CNS-isolated hemophagocytic lymphohistiocytosis secondary to uniparental disomy ofFrontiers in genetics · 2025Article
- Etoposide Therapy of Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Late-onset Familial Hemophagocytic Lymphohistiocytosis in a survivor of Hodgkin's Lymphoma.Leukemia research reports · 2024Article
- Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis.Cancers · 2023Review
- Review
- Case report: Hemophagocytic lymphohistiocytosis complicated by multiple organ dysfunction syndrome following aseptic encephalitis.Frontiers in immunology · 2023Article
- Hemophagocytic LymphohistiocytosisBalkan medical journal · 2022Article
- Haemophagocytic lymphohistiocytosis during pregnancy: a case presentation and literature review.Case reports in perinatal medicine · 2022Article
- Hemophagocytic Lymphohistiocytosis Secondary to Peripheral T Cell Lymphoma with Rapid Onset and Fatal Progression in a Young Patient: A Case Report and Review of the Literature.The American journal of case reports · 2021Review
- Clinical and genetic features of Epstein-Barr virus-triggered late-onset primary hemophagocytic lymphohistiocytosis: Ten pedigrees study.Clinical and translational medicine · 2021Article
- Adult onset type 2 familial hemophagocytic lymphohistiocytosis withWorld journal of clinical cases · 2021Article
- Article
- [Clinical study of haploidentical hematopoietic stem cell transplantation on 15 cases of adult-onset primary hemophagocytic lymphohistiocytosis].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2020Article
- Genotype characteristics and immunological indicator evaluation of 311 hemophagocytic lymphohistiocytosis cases in China.Orphanet journal of rare diseases · 2020Article
Corrections and comments
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Authors and funding
10 authors at 6 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of uncontrolled immune activation leading to extreme inflammation. Primary HLH was once believed to be a disease that occurred only in infancy or young children, and was rarely diagnosed in adults. It is now understood that patients can develop primary HLH in their adolescence or adulthood. This study included 252 adolescent and adult patients with a clinical diagnosis of HLH from 35 general medical institutions across mainland China. All exons and 50 bp of flanking intronic sequence of six HLH-related genes (PRF1, UNC13D, STX11, STXBP2, SH2D1A, and BIRC4) were sequenced in these patients. We identified mutations in 18/252 (7.1%) of the patients, with changes in PRF1 being most common. Late-onset HLH often features viral infection and other predisposing factors. We conclude that late-onset primary HLH is not as rare as previously thought. Older patients should not be delayed to receive HLH-related genes testing when they are suspected with HLH.
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