Báo cáo trường hợp trẻ mắc hội chứng Tafro

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Tác giả: Thị Phượng Lương, Thị Kiên Nguyễn, Thu Hương Nguyễn

Ngôn ngữ: Vie

Ký hiệu phân loại:

Thông tin xuất bản: Nghiên cứu y học (Đại học Y Hà Nội) 2020

Mô tả vật lý: 81-85

Bộ sưu tập: Metadata

ID: 396059

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Hội chứng TAFRO được báo cáo đầu tiên năm 2010 tại Nhật Bản, đăc trưng bởi giảm tiểu cầu, tràn dịch đamàng, xơ hóa tủy xương có reticulin, rối loạn chức năng thận, phì đại cơ quan. Bệnh có thể đe dọa tính mạng nêncần được chẩn đoán sớm và thường đáp ứng với thuốc ức chế miễn dịch. Bệnh thường gặp ở người già hoặctrung niên, rất hiếm gặp ở thanh thiếu niên. Chúng tôi báo cáo một ca bệnh ở trẻ nhỏ đầu tiên được chẩn đoánhội chứng TAFRO và điều trị thành công bằng Cyclospron A tại bệnh viện Nhi Trung ương. Trẻ nam 6 tuổi vàoviện vì sốt, kèm theo phù, khó thở, tràn dịch màng phổi, màng tim, cổ chướng, gan lách to, thiếu máu, giảm tiểucầu, CRP tăng, giảm albumin máu, suy thận cấp, protein niệu nhiều, C3 giảm nhẹ, C4 bình thường, sinh thiết tủyxương có tăng sinh mẫu tiểu cầu. Trẻ được chẩn đoán hội chứng TAFRO, và điều trị prednisone 1mg/kg/ngàyphối hợp cyclosporinA 4mg/kg/ngày được 2 tuần, trẻ hết sốt, hết phù, tiểu cầu máu tăng, chức năng thận cải thiện, Tóm tắt tiếng anh, TAFRO syndrome was first reported in 2010 in Japan. It is characterized by a constellation ofsymptoms: Thrombocytopenia, Anasarca, reticulin Fibrosis of the bone marrow, Renal dysfunction andOrganomegaly (TAFRO). Prompt diagnosis of TAFRO syndrome is critical due to its life threateningconditions. Patients with TAFRO syndrome usually respond to immunosuppressive therapy. TAFROsyndrome often occurs in the middle-aged and the elderly, and is rarely seen in adolescents. Here wereport the first child case diagnosed with TAFRO syndrome, and successfully treated with CyclosporinA in the national children hospital. A 6-year-old boy Vietnamese was referred to us with fever. He hadedema, dyspnea, anasarca, hepatosplenomegaly, anemia, thrombocytopenia, elevated C-reactiveprotein and hypoproteinemia, acute renal failure, high proteinuria, slightly decreased C3, normal C4,bone marrow biopsy reveals increased number of megakaryocytes. He was diagnosed with TAFROsyndrome, treated by prednisone 1mg/kg/day combined with cyclosporin A 4mg/kg/day for 2 weeks.Improvement seen with no fever, no edema, increased platelets, and improved kidney function.

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