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Malignancy in Patients With Inborn Errors of Immunity Beyond Infectious Complication: Single Center Experience for 30 Years
DC Field | Value | Language |
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dc.contributor.author | 최준식 | - |
dc.date.accessioned | 2024-07-01T06:58:03Z | - |
dc.date.available | 2024-07-01T06:58:03Z | - |
dc.date.issued | 2023-12 | - |
dc.identifier.issn | 2384-1079 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/199888 | - |
dc.description.abstract | Purpose Cancer incidence is known to be higher in patients with inborn errors of immunity (IEI) compared to the general population in addition to traditionally well-known infection susceptibility. We aimed to investigate cancer occurrence in patients with IEI in a single center. Methods Medical records of IEI patients treated at Samsung Medical Center, Seoul, Korea were retrospectively reviewed from November 1994 to September 2023. Patients with IEI and cancer were identified. Results Among 194 patients with IEI, seven patients (3.6%) were diagnosed with cancer. Five cases were lymphomas, 4 of which were Epstein-Barr virus (EBV)-associated lymphomas. The remaining cases included gastric cancer and multiple myeloma. The median age at cancer diagnosis was 18 years (range, 1–75 years). Among patients with cancer, underlying IEIs included X-linked lymphoproliferative disease-1 (XLP-1, n=3), activated phosphoinositide 3-kinase delta syndrome (APDS, n=2), and cytotoxic T-lymphocyte antigen 4 (CTLA-4) haplo-insufficiency (n=2). Seventy-five percent (3/4) of XLP-1 patients, 40.0% (2/5) of APDS patients, and 50.0% (2/4) of CTLA-4 haplo-insufficiency patients developed cancer. Patients with XLP-1 developed cancer at earlier age (median age 5 years) compared to those with APDS and CTLA-4 (P<0.001). One patient with APDS died during hematopoietic cell transplantation. Conclusions Cancer occurred in 3.6% of IEI patients at a single center in Korea. In addition to infectious complications and inflammation, physicians caring for IEI patients should be aware of the potential risk of cancer, especially in association with EBV infection. | - |
dc.description.statementOfResponsibility | open | - |
dc.language | Korean | - |
dc.publisher | Korean Society of Pediatric Infectious Diseases | - |
dc.relation.isPartOf | Pediatric Infection & Vaccine | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.title | Malignancy in Patients With Inborn Errors of Immunity Beyond Infectious Complication: Single Center Experience for 30 Years | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.department | Dept. of Pediatrics (소아과학교실) | - |
dc.contributor.googleauthor | Doo Ri Kim | - |
dc.contributor.googleauthor | Kyung-Ran Kim | - |
dc.contributor.googleauthor | Hwanhee Park | - |
dc.contributor.googleauthor | Joon-sik Choi | - |
dc.contributor.googleauthor | Yoonsun Yoon | - |
dc.contributor.googleauthor | Sohee Son | - |
dc.contributor.googleauthor | Hee Young Ju | - |
dc.contributor.googleauthor | Jihyun Kim | - |
dc.contributor.googleauthor | Keon Hee Yoo | - |
dc.contributor.googleauthor | Kangmo Ahn | - |
dc.contributor.googleauthor | Hee-Jin Kim | - |
dc.contributor.googleauthor | Eun-Suk Kang | - |
dc.contributor.googleauthor | Junhun Cho | - |
dc.contributor.googleauthor | Su Eun Park | - |
dc.contributor.googleauthor | Kihyun Kim | - |
dc.contributor.googleauthor | Yae-Jean Kim | - |
dc.identifier.doi | 10.14776/piv.2023.30.e22 | - |
dc.contributor.localId | A06293 | - |
dc.relation.journalcode | J02486 | - |
dc.identifier.eissn | 2384-1087 | - |
dc.contributor.alternativeName | Choi, Joon Sik | - |
dc.contributor.affiliatedAuthor | 최준식 | - |
dc.citation.volume | 30 | - |
dc.citation.number | 3 | - |
dc.citation.startPage | 129 | - |
dc.citation.endPage | 138 | - |
dc.identifier.bibliographicCitation | Pediatric Infection & Vaccine, Vol.30(3) : 129-138, 2023-12 | - |
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