关键词: dermatitis granulomatous myelodysplasia cutis myelodysplastic syndrome

Mesh : Humans Skin / pathology Myelodysplastic Syndromes / diagnosis genetics Leukemia, Myeloid, Acute / genetics Mutation Hematologic Neoplasms / pathology Dermatitis / diagnosis etiology

来  源:   DOI:10.1111/cup.14571

Abstract:
Myelodysplastic syndromes (MDS) are a group of clonal hematopoietic neoplasms resulting from mutations in stem cells. They carry a risk of transformation to acute myeloid leukemia. Cutaneous manifestations of MDS, including myelodysplasia cutis or infiltration by MDS tumor cells, are rare, but significantly associated with increased risk of progression to high-grade myeloid tumors. The clinical and histopathologic differential diagnosis for myelodysplasia cutis includes interstitial granulomatous dermatitis (IGD), a reactive granulomatous dermatitis (RGD) associated with systemic diseases including rheumatologic diseases, and hematologic malignancy like MDS. We report a patient with MDS who presented with myelodysplasia cutis masquerading as IGD both in a clinical and histopathological manner.
摘要:
骨髓增生异常综合征(MDS)是一组由干细胞突变引起的克隆性造血肿瘤。它们具有转化为急性髓细胞性白血病的风险。MDS的皮肤表现,包括骨髓增生异常角化或MDS肿瘤细胞浸润,是罕见的,但与进展为高级别髓系肿瘤的风险增加显著相关。皮肤骨髓增生异常的临床和组织病理学鉴别诊断包括间质性肉芽肿性皮炎(IGD),与全身性疾病相关的反应性肉芽肿性皮炎(RGD),包括风湿性疾病,和血液恶性肿瘤如MDS。我们报告了一名MDS患者,该患者在临床和组织病理学上都伪装成IGD。
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