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血红蛋白病相关慢性腿部溃疡中的类脂质渐进性坏死性血管病

Livedoid vasculopathy in hemoglobinopathy-associated chronic leg ulcers.

作者信息

Eswaran Harish, Wilson Samuel, Little Jane, Moll Stephan

机构信息

Division of Hematology, Department of Medicine, UNC Chapel Hill School of Medicine, Chapel Hill, NC, United States of America.

出版信息

Ann Hematol. 2024 Dec;103(12):5235-5239. doi: 10.1007/s00277-024-06112-0. Epub 2024 Dec 7.

Abstract

Chronic leg ulceration is a debilitating manifestation of hemoglobinopathies, and best management is uncertain. Livedoid vasculopathy (LV) is a cutaneous non-inflammatory thrombotic vasculopathy treated with anticoagulation that has been identified in hemoglobinopathy-associated chronic leg ulceration. However, most patients with hemoglobinopathy-associated ulcers do not undergo workup for secondary causes, and the prevalence and relevance of LV is unclear. Outcomes of secondary workup were examined retrospectively in this study. 108 patients with hemoglobinopathy-associated chronic leg ulcers were identified. 15% of patients underwent skin biopsy, and 97% of biopsies showed non-specific findings. Two patients had LV and neither responded to anticoagulants. Livedoid vasculopathy is a rare cause of ulceration in hemoglobin gene disorders and the benefit of anticoagulation in these cases is unclear.

摘要

慢性腿部溃疡是血红蛋白病的一种使人衰弱的表现,最佳治疗方法尚不确定。类脂质渐进性坏死性血管病(LV)是一种经抗凝治疗的皮肤非炎症性血栓性血管病,已在血红蛋白病相关的慢性腿部溃疡中被发现。然而,大多数血红蛋白病相关溃疡患者并未针对继发原因进行检查,LV的患病率及相关性尚不清楚。本研究对二次检查的结果进行了回顾性分析。共纳入108例血红蛋白病相关的慢性腿部溃疡患者。15%的患者接受了皮肤活检,97%的活检显示为非特异性结果。两名患者患有LV,均对抗凝剂无反应。类脂质渐进性坏死性血管病是血红蛋白基因疾病中溃疡形成的罕见原因,在这些病例中抗凝治疗的益处尚不清楚。

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