血红蛋白病相关慢性腿部溃疡中的活体样血管病变。

IF 3 3区 医学 Q2 HEMATOLOGY Annals of Hematology Pub Date : 2024-12-07 DOI:10.1007/s00277-024-06112-0
Harish Eswaran, Samuel Wilson, Jane Little, Stephan Moll
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引用次数: 0

摘要

慢性腿部溃疡是一种使人衰弱的血红蛋白病的表现,最好的管理是不确定的。活体样血管病变(LV)是一种经抗凝治疗的皮肤非炎症性血栓性血管病变,已在血红蛋白病相关的慢性腿部溃疡中发现。然而,大多数患有血红蛋白病相关溃疡的患者没有接受继发原因的检查,并且LV的患病率和相关性尚不清楚。本研究回顾性检查了二次随访的结果。108例与血红蛋白病相关的慢性腿部溃疡患者被确定。15%的患者接受皮肤活检,97%的活检显示非特异性结果。两名患者有左室,抗凝药物均无反应。类活体血管病变是血红蛋白基因疾病中罕见的溃疡原因,在这些病例中抗凝治疗的益处尚不清楚。
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Livedoid vasculopathy in hemoglobinopathy-associated chronic leg ulcers

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.

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来源期刊
Annals of Hematology
Annals of Hematology 医学-血液学
CiteScore
5.60
自引率
2.90%
发文量
304
审稿时长
2 months
期刊介绍: Annals of Hematology covers the whole spectrum of clinical and experimental hematology, hemostaseology, blood transfusion, and related aspects of medical oncology, including diagnosis and treatment of leukemias, lymphatic neoplasias and solid tumors, and transplantation of hematopoietic stem cells. Coverage includes general aspects of oncology, molecular biology and immunology as pertinent to problems of human blood disease. The journal is associated with the German Society for Hematology and Medical Oncology, and the Austrian Society for Hematology and Oncology.
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