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KMID : 1040320230300030129
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Pediatric Infection & Vaccine
2023 Volume.30 No. 3 p.129 ~ p.138
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Malignancy in Patients With Inborn Errors of Immunity Beyond Infectious Complication: Single Center Experience for 30 Years
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Kim Doo-Ri
Kim Kyung-Ran
Park Hwan-Hee
Choi Joon-Sik
Yoon Yoon-Sun
Son So-Hee
Ju Hee-Young
Kim Ji-Hyun
Yoo Keon-Hee
Ahn Kang-Mo
Kim Hee-Jin
Kang Eun-Suk
Cho Jun-Hun
Park Su-Eun
Kim Ki-Hyun
Kim Yae-Jean
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Abstract
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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) haploinsufficiency (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.
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KEYWORD
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Primary immunodeficiency diseases, Epstein-Barr virus, Malignancy, Lymphoma
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