ArticleBritish journal of haematology2011
Impaired immune function in children with Fanconi anaemia.
Article in British journal of haematology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
26 citing papers in PubMed, 1 synthesis or guideline pooled it, 39 citations in OpenAlex.
- Longitudinal clinical manifestations of Fanconi anemia: A systematized review.Blood reviews · 2024Pooled it
- Immune status of patients with inherited bone marrow failure syndromes.American journal of hematology · 2015Trial
- Beyond neutropenia: Immunological evaluation of patients with Shwachman-Diamond syndrome.British journal of haematology · 2026Article
- The Molecular and Genetic Mechanisms of Inherited Bone Marrow Failure Syndromes: The Role of Inflammatory Cytokines in Their Pathogenesis.Biomolecules · 2023Review
- Article
- Bone Marrow Failure Syndromes, Overlapping Diseases with a Common Cytokine Signature.International journal of molecular sciences · 2021Review
- NK Cell Development and Function in Patients with Fanconi Anemia.Critical reviews in immunology · 2021Article
- A mathematical solution to Peto's paradox using Polya's urn model: implications for the aetiology of cancer in general.Theory in biosciences = Theorie in den Biowissenschaften · 2019Article
- Natural Killer Cell Degranulation Defect: A Cause for Impaired NK-Cell Cytotoxicity and Hyperinflammation in Fanconi Anemia Patients.Frontiers in immunology · 2019Article
- Review
- Lymphopenia With Clinical and Laboratory Features of Combined Immune Deficiency in an 11-Year-Old Female WithFrontiers in pediatrics · 2018Article
- Limited detection of human polyomaviruses in Fanconi anemia related squamous cell carcinoma.PloS one · 2018Article
- Antibody response to human papillomavirus vaccination and natural exposure in individuals with Fanconi Anemia.Vaccine · 2017Article
- Impaired immune function in children and adults with Fanconi anemia.Pediatric blood & cancer · 2017Article
- Common Variable Immunodeficiency Caused by FANC Mutations.Journal of clinical immunology · 2017Article
- Current Knowledge and Priorities for Future Research in Late Effects after Hematopoietic Cell Transplantation for Inherited Bone Marrow Failure Syndromes: Consensus Statement from the Second Pediatric Blood and Marrow Transplant Consortium International Conference on Late Effects after Pediatric Hematopoietic Cell Transplantation.Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation · 2017Review
- Natural Killer Cells in the Orchestration of Chronic Inflammatory Diseases.Journal of immunology research · 2017Review
- Loss of Fancc Impairs Antibody-Secreting Cell Differentiation in Mice through Deregulating the Wnt Signaling Pathway.Journal of immunology (Baltimore, Md. : 1950) · 2016Article
- Dearth and Delayed Maturation of Testicular Germ Cells in Fanconi Anemia E Mutant Male Mice.PloS one · 2016Article
- Oral human papillomavirus is common in individuals with Fanconi anemia.Cancer epidemiology, biomarkers & prevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology · 2015Article
Corrections and comments
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Authors and funding
11 authors at 4 institutions in 1 country.
Funding
Abstract
Fanconi anaemia is an autosomal recessive or X-linked disease characterized by progressive bone marrow failure, variable congenital abnormalities and a predisposition to malignancy. Reports of immune function in this population are limited, and include only specific areas of immune performance, showing variable defects. We report a cross-sectional immunological assessment in 10 children with FA. Absolute numbers of B cells and natural killer (NK) cells were reduced compared to controls (P = 0·048 and P = 0·0002, respectively), while absolute number of T cells were within normal range. Perforin and granzyme content of NK cells was reduced (P < 0·00001 and P = 0·0057, respectively) along with the NK cell cytotoxicity (P < 0·001). Antigen proliferation in response to tetanus was decreased (P = 0·008) while responses to candida and phytohaemagglutinin were not. Cytotoxic T cell function was also reduced (P < 0·0001). Immunoglobulin G levels were normal in those evaluated. Our series represents the first attempt at a comprehensive quantitative and functional evaluation of immune function in this rare group of patients and demonstrates a significant deficit in the NK cell compartment, a novel quantitative B cell defect, along with abnormal cytotoxic function. These findings may be especially relevant in this patient population with known predisposition to DNA damage and malignancy.
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Registered trials
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