鼻假瘤病例报告-严重血友病a与高滴度抑制剂的罕见表现

P. Mandal, Malini Garg, Debasis Gantait, U. Jana
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引用次数: 1

摘要

使用抑制剂的血友病患者由于因子VIII替代无效,发病率和死亡率增加。假肿瘤是罕见的,但危险的并发症在这些患者中,鼻假肿瘤更罕见。在这里,我们提出的情况下,一名患有严重血友病a与高滴度抑制剂谁发展鼻假瘤的幼儿。当由于经济限制而无法进行免疫耐受治疗时,他接受了FEIBA预防和利妥昔单抗治疗。假性肿瘤经手术切除处理。我们得出结论,血友病患者鼻出血可能是由于潜在的鼻假肿瘤,并强调使用利妥昔单抗根除抑制剂。
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Case report of nasal pseudotumor – a rare presentation in severe haemophilia A with high titre inhibitors
Abstract Haemophilia patients with inhibitors suffer from increased morbidity and mortality due to the ineffectiveness of factor VIII replacement. Pseudotumors are rare but dangerous complications in these patients, and nasal pseudotumors are even rarer. Here, we present the case of a young child with severe haemophilia A with high titre inhibitors who developed a nasal pseudotumor. When immune tolerance therapy was not possible due to financial constraints, he was treated with FEIBA prophylaxis and rituximab. The pseudotumor was managed with surgical excision. We conclude that epistaxis in haemophiliacs can be due to an underlying nasal pseudotumor, and highlight the use of rituximab for the eradication of inhibitors.
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