7. First CD3/CD45RA depleted haploidentical transplant for severe combined immunodeficiency disease with Rotavirus gastroenteritis in Vietnam

Nguyen Thanh Binh, Dang Anh Duong1, Bui Ngoc Lan, Nguyen Thi Van Anh, Tran Quynh Huong, Le Thi Minh Huong, Cao Viet Tung, Tran Minh Dien, Nguyen Ngoc Quynh Le
1 bệnh viện nhi Trung ương

Main Article Content

Abstract

Severe combined immunodeficiency includes inherited diseases with impairment of T cells development, often associated with profound defects of B and/or NK cells differentiation. Rotavirus infection is responsible for severe acute gastroenteritis and over 400,000 deaths a year in children aged < 5 years, especially in low and middle-income countries. We report a nine-month-old girl presented with failure to thrive left axillary lymphadenitis post-BCG-vaccination, persistent, nonvaccine-associated rotavirus gastroenteritis. An immune evaluation revealed absent T lymphocytes, B lymphocytes, normal NK cells with genetic testing confirmed RAG2 severe combined immunodeficiency. Rotavirus gastroenteritis persisted despite the administration of oral immunoglobulin pre-transplantation. The patient was treated by a haploidentical transplant -from mother as a donor using CD3/CD45RA+ depletion kit, CliniMACs system (Miltenyi). The first transplant was performed without conditioning. One month post-transplant, rotavirus was cleared. The second transplant was performed three months later, with myeloablative conditioning (Bu-Flu-rATG). Neutrophils engrafted on day +10 post-transplant, platelet engrafted on day +45. Whole blood chimerism on day +28 post 2nd transplant was 100%. Serum IgG level has been normalized without IVIG infusion three months post 2nd transplant.

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References

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