Tan-Lim Carol Stephanie C, Castor Mary Anne R
Department of Pediatrics, Section of Allergy and Immunology, Philippine General Hospital, Manila, the Philippines.
Asia Pac Allergy. 2021 Jan 26;11(1):e7. doi: 10.5415/apallergy.2021.11.e7. eCollection 2021 Jan.
Primary immunodeficiency disorders, although rare, pose a significant burden in the quality of life of afflicted patients and their families. The most common of these disorders are caused by B-cell defects. A total of 6 patients were seen and diagnosed in a national tertiary hospital in the Philippines from 1996 to 2018. These patients were admitted due to various infections, and were subsequently diagnosed to have B-cell defects. Four out of the 6 patients have genetic studies confirming the diagnosis of X-linked agammaglobulinemia. One patient succumbed to sepsis at 10 years of age, while the rest are on follow-up at the Philippine General Hospital for intravenous immunoglobulin infusion.
原发性免疫缺陷疾病虽然罕见,但给患病患者及其家庭的生活质量带来了沉重负担。这些疾病中最常见的是由B细胞缺陷引起的。1996年至2018年期间,菲律宾一家国立三级医院共接诊并诊断了6例患者。这些患者因各种感染入院,随后被诊断为B细胞缺陷。6例患者中有4例经基因研究确诊为X连锁无丙种球蛋白血症。1例患者在10岁时死于败血症,其余患者在菲律宾总医院接受随访,进行静脉注射免疫球蛋白治疗。