ArticleThe Journal of allergy and clinical immunology2022
Nodular regenerative hyperplasia in X-linked agammaglobulinemia: An underestimated and severe complication.
Article in The Journal of allergy and clinical immunology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.
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Who cites it
12 citing papers in PubMed, 1 synthesis or guideline pooled it, 17 citations in OpenAlex.
- Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management.Hepatology international · 2024Guideline
- The cost-benefit of newborn screening for X-linked agammaglobulinemia and related B-cell lymphopenia.Immunologic research · 2026Review
- RNA Sequencing Addresses a 5' UTR Variant Leading to X-Linked Agammaglobulinemia and Broader Immune Dysregulation.Journal of clinical immunology · 2025Article
- Outcomes of X-Linked Agammaglobulinaemia Patients.Journal of clinical immunology · 2024Article
- A rare differential diagnosis of inflammatory bowel disease in a young male patient; a challenging case report.Gastroenterology and hepatology from bed to bench · 2024Article
- Differential Diagnosis: Hepatic Complications in Inborn Errors of Immunity.Journal of clinical medicine · 2023Review
- Late-onset enteric virus infection associated with hepatitis (EVAH) in transplanted SCID patients.The Journal of allergy and clinical immunology · 2023Article
- Organ-based clues for diagnosis of inborn errors of immunity: A practical guide for clinicians.Immunity, inflammation and disease · 2023Review
- Co-Occurring X-Linked Agammaglobulinemia and X-Linked Chronic Granulomatous Disease: Two Isolated Pathogenic Variants in One Patient.Biomedicines · 2023Article
- Portal hypertension in common variable immunodeficiency disorders - a single center analysis on clinical and immunological parameter in 196 patients.Frontiers in immunology · 2023Article
- Angiosarcoma in Long-Standing Nodular Regenerative Hyperplasia.ACG case reports journal · 2022Article
- Fatal liver mass rupture in a common-variable-immunodeficiency patient with probable nodular regenerating hyperplasia.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2022Article
Corrections and comments
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Authors and funding
18 authors at 4 institutions in 2 countries.
Funding
Abstract
backgroundLate-onset complications in X-linked agammaglobulinemia (XLA) are increasingly recognized. Nodular regenerative hyperplasia (NRH) has been reported in primary immunodeficiency but data in XLA are limited.
objectivesThis study sought to describe NRH prevalence, associated features, and impact in patients with XLA.
methodsMedical records of all patients with XLA referred to the National Institutes of Health between October 1994 and June 2019 were reviewed. Liver biopsies were performed when clinically indicated. Patients were stratified into NRH+ or NRH- groups, according to their NRH biopsy status. Fisher exact test and Mann-Whitney test were used for statistical comparisons.
resultsRecords of 21 patients with XLA were reviewed, with a cumulative follow-up of 129 patient-years. Eight patients underwent ≥1 liver biopsy of whom 6 (29% of the National Institutes of Health XLA cohort) were NRH+. The median age at NRH diagnosis was 20 years (range, 17-31). Among patients who had liver biopsies, alkaline phosphatase levels were only increased in patients who were NRH+ (P = .04). Persistently low platelet count (<100,000 per μL for >6 months), mildly to highly elevated hepatic venous pressure gradient and either hepatomegaly and/or splenomegaly were present in all patients who were NRH+. In opposition, persistently low platelet counts were not seen in patients who were NRH-, and hepatosplenomegaly was observed in only 1 patient who was NRH-. Hepatic venous pressure gradient was normal in the only patient tested who was NRH-. All-cause mortality was higher among patients who were NRH+ (5 of 6, 83%) than in the rest of the cohort (1 of 15, 7% among patients who were NRH- and who were classified as unknown; P = .002).
conclusionsNRH is an underreported, frequent, and severe complication in XLA, which is associated with increased morbidity and mortality.
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