Ca bệnh hiếm gặp: u lympho không Hodgkin tế bào T xâm lấn tủy xương trên bệnh nhân hemophilia A=Rare case: bone marrow invasive t-cell non-Hodgkin lymphoma in a patient with Hemophilia A

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Tác giả: Thị Thu Thủy Hoàng, Quang Tùng Nguyễn

Ngôn ngữ: vie

Ký hiệu phân loại:

Thông tin xuất bản: Y học cộng đồng, 2024

Mô tả vật lý: tr.301-305

Bộ sưu tập: Báo, Tạp chí

ID: 242127

Thêm vào giỏ

Hemophilia A is a hereditary blood clotting disorder with a prevalence of 1/6000 in males. According to the GLOBOCAN database of the International Agency for Research on Cancer of the World Health Organization, among 36 different types of cancer, non-Hodgkin lymphoma ranked 8th in males and 10th in females. However, it is very rare for a patient to have both diseases simultaneously. We want to report a case of T-cell non-Hodgkin lymphoma with bone marrow invasion in a patient with severe Hemophilia A. The patient was an 18-year-old male, diagnosed with Hemophilia A at the age of 4, and was regularly treated with recombinant factor VIII. During their most frequent symptoms, the patient had continuous high fever, with axillary lymphadenopathy. The chest wall ulcer gradually spread after the patient had their axillary lymphadenopathy removed, and popular lesions started appearing on their skin. Hemostasis of the ulcerated lesions was difficult despite the patient receiving factor VIII replacement until stable. Biopsy results with specific immune markers for the ulcerated lesions concluded T-cell non-Hodgkin lymphoma with bone biopsy showed bone marrow invasion. The patient was treated with CHOP chemotherapy but went into septic shock during the leukopenia phase and died.Hemophilia A là bệnh lý rối loạn đông máu di truyền với tỷ lệ 1/6000 nam giới mắc bệnh. Theo cơ sở dữ liệu GLOBOCAN của Cơ quan Nghiên cứu ung thư quốc tế thuộc Tổ chức Y tế Thế giới, trong số 36 loại ung thư khác nhau, tỷ lệ mắc u lympho không Hodgkin đứng thứ 8 ở nam giới và thứ 10 ở nữ. Tuy nhiên, việc bệnh nhân bị mắc đồng thời 2 căn bệnh lại rất hiếm. Chúng tôi báo cáo ca bệnh u lympho không Hodgkin tế bào T có xâm lấn tủy xương trên bệnh nhân Hemophilia A thể nặng. Bệnh nhân nam, 18 tuổi chẩn đoán Hemophilia A thể nặng từ năm 4 tuổi, được điều trị thường xuyên bằng yếu tố VIII tái tổ hợp. Đợt bệnh này bệnh nhân sốt cao liên tục kèm hạch nách. Vết loét thành ngực lan dần sau khi bệnh nhân được trích hạch nách, đồng thời xuất hiện tổn thương sẩn cục trên da. Việc cầm máu tổn thương vùng loét rất khó khăn mặc dù bệnh nhân đã được bù yếu tố VIII đến mức ổn định. Kết quả sinh thiết với các dấu ấn miễn dịch đặc hiệu tại tổn thương vùng loét đã kết luận u lympho không Hodgkin tế bào T và sinh thiết tủy xương thấy hình ảnh xâm lấn tủy. Bệnh nhân được điều trị hóa chất với phác đồ CHOP nhưng đã sốc nhiễm khuẩn trong giai đoạn hạ bạch cầu và tử vong.

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