ArticlePloS one2013
Occurrence of nodular lymphocyte-predominant hodgkin lymphoma in hermansky-pudlak type 2 syndrome is associated to natural killer and natural killer T cell defects.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 1 of them a synthesis that pooled it.
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Who cites it
23 citing papers in PubMed, 1 synthesis or guideline pooled it, 46 citations in OpenAlex.
- Cancer Trends in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis.Journal of clinical immunology · 2024Pooled it
- A predictive model based on immune related genes for diffuse large B cell lymphoma (DLBCL).Translational cancer research · 2025Article
- Review
- LRRC25 Is a Potential Biomarker for Predicting Immunotherapy Response in Patients with Gastric Cancer.Digestive diseases and sciences · 2025Article
- Nodular lymphocyte-predominant Hodgkin lymphoma: advances in disease biology, risk stratification, and treatment.Haematologica · 2024Review
- Pathogenesis and Therapy of Hermansky-Pudlak Syndrome (HPS)-Associated Pulmonary Fibrosis.International journal of molecular sciences · 2024Review
- Histopathologic Features and Differential Diagnosis in Challenging Cases of Nodular Lymphocyte Predominant B-cell Lymphoma/Nodular Lymphocyte Predominant Hodgkin Lymphoma.Journal of clinical and translational pathology · 2024Article
- Hemophagocytic Lymphohistiocytosis in the Context of Hematological Malignancies and Solid Tumors.Advances in experimental medicine and biology · 2024Review
- Microenvironmental immune cell alterations across the spectrum of nodular lymphocyte predominant Hodgkin lymphoma and T-cell/histiocyte-rich large B-cell lymphoma.Frontiers in oncology · 2023Article
- A novel membrane-bound interleukin-2 promotes NK-92 cell persistence and anti-tumor activity.Oncoimmunology · 2022Article
- Platelet-Related Molecular Subtype to Predict Prognosis in Hepatocellular Carcinoma.Journal of hepatocellular carcinoma · 2022Article
- Manipulating NK cellular therapy from cancer to invasive fungal infection: promises and challenges.Frontiers in immunology · 2022Review
- Role of adaptin protein complexes in intracellular trafficking and their impact on diseases.Bioengineered · 2021Article
- A Novel Likely Pathogenic Variant in theCells · 2021Review
- Pitfalls in the Diagnosis of Nodular Lymphocyte Predominant Hodgkin Lymphoma: Variant Patterns, Borderlines and Mimics.Cancers · 2021Review
- Exploring natural killer cell immunology as a therapeutic strategy in lung cancer.Translational lung cancer research · 2021Review
- Identification of an Immune-Related Long Noncoding RNA Pairs Model to Predict Survival and Immune Features in Gastric Cancer.Frontiers in cell and developmental biology · 2021Article
- NK cells for cancer immunotherapy.Nature reviews. Drug discovery · 2020Review
- Coatopathies: Genetic Disorders of Protein Coats.Annual review of cell and developmental biology · 2019Review
- From Natural Killer Cell Receptor Discovery to Characterization of Natural Killer Cell Defects in Primary Immunodeficiencies.Frontiers in immunology · 2019Review
Corrections and comments
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Authors and funding
15 authors at 6 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hermansky Pudlak type 2 syndrome (HPS2) is a rare autosomal recessive primary immune deficiency caused by mutations on β3A gene (AP3B1 gene). The defect results in the impairment of the adaptor protein 3 (AP-3) complex, responsible for protein sorting to secretory lysosomes leading to oculo-cutaneous albinism, bleeding disorders and immunodeficiency. We have studied peripheral blood and lymph node biopsies from two siblings affected by HPS2. Lymph node histology showed a nodular lymphocyte predominance type Hodgkin lymphoma (NLPHL) in both HPS2 siblings. By immunohistochemistry, CD8 T-cells from HPS2 NLPHL contained an increased amount of perforin (Prf) + suggesting a defect in the release of this granules-associated protein. By analyzing peripheral blood immune cells we found a significant reduction of circulating NKT cells and of CD56(bright)CD16(-) Natural Killer (NK) cells subset. Functionally, NK cells were defective in their cytotoxic activity against tumor cell lines including Hodgkin Lymphoma as well as in IFN-γ production. This defect was associated with increased baseline level of CD107a and CD63 at the surface level of unstimulated and IL-2-activated NK cells. In summary, these results suggest that a combined and profound defect of innate and adaptive effector cells might explain the susceptibility to infections and lymphoma in these HPS2 patients.
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