유전구형적혈구증 환자에서 Parvovirus B19에 의한 골수무형성위기 이후 발생한 자가면역용혈빈혈 1예
Autoimmune Hemolytic Anemia after Aplastic Crisis due to Parvovirus B19 Infection in a Patient with Hereditary Spherocytosis
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초록
유전구형적혈구증은 적혈구막의 세포골격층을 구성하는 단백질의 결핍으로 인해 구형적혈구가 생겨 지속적으로 용혈이 일어나는 유전질환이다. 본 질환의 경과 중 parvovirus B19 감염으로 인한골수무형성위기를 동반할 수 있다. Parvovirus B19 감염은 전염성홍반과 관절염 등의 질환뿐 아니라 자가면역용혈빈혈을 포함한 다양한 자가면역질환도 유발하는 것으로 알려져 있다. 이에 본 저자들은 유전구형적혈구증을 가진 11세 남아에서 parvovirus B19 감염에 의한 골수무형성위기 3개월 이후 회복기에서 parvovirus B19IgM이 지속적으로 검출되면서 자가면역용혈빈혈이 추가로 동반된드문 1예를 경험하여 이를 문헌 검토와 함께 보고하고자 한다
Abstract
Hereditary spherocytosis (HS) is a genetic disorder characterized by the production and destruction of spherocytes due to a deficiency of red cell membrane cytoskeletal proteins, resulting in the clinical presentation of chronic hemolytic anemia. This disease can be accompanied by an aplastic crisis due to parvovirus B19 infection. Parvovirus B19 infection causes diseases such as erythema infectiosum and arthritis, and can also trigger various autoimmune diseases, including autoimmune hemolytic anemia (AIHA). Here, we report a rare case of AIHA developing 3 months after an aplastic crisis due to parvovirus B19 infection in an 11-year-old boy with HS and provide the relevant literature review.
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