U lympho tế bào T dạng viêm mỡ dưới da ở trẻ em

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Tác giả: Thị Thúy Nga Đỗ, Ngọc Thạch Hoàng, Anh Văn Nguyễn, Văn Lâm Nguyễn, Hồng Điệp Phó, Thị Thúy Trần

Ngôn ngữ: Vie

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Thông tin xuất bản: Y học lâm sàng 2021

Mô tả vật lý: 99-105

Bộ sưu tập: Metadata

ID: 410114

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Chủ đề

U lympho tế bào T dạng viêm mỡ dưới da (Subcutaneous panniculitis - like T - cell lymphoma - SPTCL) là một u lympho hiếm gặp với tổn thương chủ yếu ở bề mặt da và tổ chức mỡ dưới da. SPTCL có thể xảy ra ở cả người lớn và trẻ nhỏ, độ tuổi trung bình được chẩn đoán là 30 tuổi, nữ nhiều hơn nam. Trong SPTCL, bệnh nhân có nhiều nốt tổn thương ở mô mỡ dưới da và có thể gây loét bề mặt da. Tổn thương này được biết đến như viêm mỡ dưới da. Cơ chế bệnh sinh chưa rõ ràng nhưng có sự tham gia của yếu tố di truyền. Chẩn đoán SPTCL vẫn còn là thách thức, chủ yếu dựa vào sinh thiết da với sự bộc lộ của các dấu ấn hóa mô miễn dịch (HMMD). Điều trị SPTCL chưa thống nhất, phụ thuộc vào triệu chứng nhưng thường sử dụng steroid hoặc các thuốc ức chế miễn dịch (ƯCMD). Một số bệnh nhân với SPTCL tiến triển cần điều trị hóa chất. Về tiên lượng, SPTCL thường tiến triển chậm, tuy nhiên khoảng 20% bệnh nhân có hội chứng thực bào máu (Hemophagocytic lymphohistiocytosis - HLH), những bệnh nhân này kém đáp ứng với điều trị và có tiên lượng xấu hơn. Chúng tôi báo cáo một bé trai 13 tháng tuổi vào viện vì sốt và nhiều khối tổn thương dưới da. Bệnh nhân được chẩn đoán viêm mô tế bào và điều trị kháng sinh 2 tuần nhưng bệnh không thuyên giảm. Sau đó, bệnh nhân được sinh thiết tổn thương dưới da, dựa trên kết quả nhuộm HMMD, chúng tôi kết luận phù hợp với SPTCL., Tóm tắt tiếng anh, Subcutaneous panniculitis - like T - cell lymphoma (SPTCL) is a rare type of lymphoma that mainly affects the skin and subcutaneous fat tissue. SPTCL can occur in both adults and children. The average age at diagnosis is 30 years old, with more women than men. In SPTCL, the patient has multiple nodules in the fat layer under the skin, and these can cause swelling. This is known as panniculitis. The pathogenesis is unknown, but genetic factors may be involved. Diagnosis of SPTCL is challenging, mainly based on skin biopsies with staining of immunohistochemical markers. Treatment of SPTCL is inconsistent, depending on symptoms but usually using steroids or immunosuppressive drugs (ACEIs). Some patients with advanced SPTCL require chemotherapy. In terms of patients who have hemophagocytic lymphohistiocytosis (HLH), which is less responsive to treatment and has a worse prognosis. We report a 13 - month - old boy admitted to the hospital with fever and multiple subcutaneous lesions. The patient was diagnosed with cellulitis and treated with antibiotics for 2 weeks, but the disease did not improve. Then, the patient had a skin lesion biopsy. Based on the results of immunohistochemical staining, we concluded that it was suitable for SPTCL.

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