Academic Journal
Fatal liver mass rupture in a common-variable-immunodeficiency patient with probable nodular regenerating hyperplasia.
| Title: | Fatal liver mass rupture in a common-variable-immunodeficiency patient with probable nodular regenerating hyperplasia. |
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| Authors: | Sompornrattanaphan M; Division of Allergy and Clinical Immunology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkok Noi, Bangkok, 10700, Thailand., Tongdee R; Department of Diagnostic Radiology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Wongsa C; Division of Allergy and Clinical Immunology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkok Noi, Bangkok, 10700, Thailand., Jitmuang A; Division of Infectious Diseases and Tropical Medicine, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Thongngarm T; Division of Allergy and Clinical Immunology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkok Noi, Bangkok, 10700, Thailand. torallergy@gmail.com. |
| Source: | Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology [Allergy Asthma Clin Immunol] 2022 Jan 07; Vol. 18 (1), pp. 2. Date of Electronic Publication: 2022 Jan 07. |
| Publication Type: | Journal Article |
| Language: | English |
| Journal Info: | Publisher: Country of Publication: England NLM ID: 101244313 Publication Model: Electronic Cited Medium: Print ISSN: 1710-1484 (Print) Linking ISSN: 17101484 NLM ISO Abbreviation: Allergy Asthma Clin Immunol Subsets: PubMed not MEDLINE |
| Imprint Name(s): | Publication: Oct. 2009- : London : BioMed Central Original Publication: Hamilton, Ont. : BC Decker, c2004- |
| Abstract: | Background: Nodular regenerating hyperplasia (NRH) is the most common liver involvement in common variable immunodeficiency (CVID). Most patients are asymptomatic with gradually increasing alkaline phosphatase (ALP) and mildly elevated transaminase enzymes over the years. We report the first case of fatal liver mass rupture in a CVID patient with probable NRH. Case Presentation: A 24-year-old man was diagnosed with CVID at the age of 1.25 years. Genetic testing revealed a transmembrane activator and calcium-modulator and cyclophilin-ligand interactor (TACI) mutation. He had been receiving intravenous immunoglobulin (IVIg) replacement therapy ever since then. The trough level of serum IgG ranged between 750-1200 mg/dL. However, he still had occasional episodes of lower respiratory tract infection until bronchiectasis developed. At 22 years old, computed tomography (CT) chest and abdomen as an investigation for lung infection revealed incidental findings of numerous nodular arterial-enhancing lesions in the liver and mild splenomegaly suggestive of NRH with portal hypertension. Seven months later, he developed sudden hypotension and tense bloody ascites. Emergency CT angiography of the abdomen showed NRH with intrahepatic hemorrhage and hemoperitoneum. Despite successful gel foam embolization, the patient died from prolonged shock and multiple organ failure. Conclusions: Although CVID patients with NRH are generally asymptomatic, late complications including portal hypertension, hepatic failure, and hepatic rupture could occur. Therefore, an evaluation of liver function should be included in the regular follow-up of CVID patients. (© 2022. The Author(s).) |
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| Contributed Indexing: | Keywords: Common variable immunodeficiency; Hepatic rupture; Immunodeficiency; Nodular regenerating hyperplasia; TACI mutation |
| Entry Date(s): | Date Created: 20220108 Latest Revision: 20220111 |
| Update Code: | 20221216 |
| PubMed Central ID: | PMC8742317 |
| DOI: | 10.1186/s13223-021-00643-1 |
| PMID: | 34996523 |
| Database: | MEDLINE |
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Accede al texto completo | ISSN: | 1710-1484 |
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| DOI: | 10.1186/s13223-021-00643-1 |