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副肿瘤性白细胞破碎性血管炎酷似小腿溃疡,为冒烟型骨髓瘤IgG κ轻链型罕见的初始表现。

Paraneoplastic leukocytoclastic vasculitis mimicking ulcus cruris as rare initial manifestation of smoldering myeloma IgG kappa.

作者信息

Brummer Christina, Utpatel Kirsten, Geis Sebastian, Haferkamp Sebastian, Brosig Andreas, Herrmann Markus, Arnreich Chiara, Hahn Joachim, Pukrop Tobias, Herr Wolfgang, Gaerditz Sabine

机构信息

Department of Hematology and Oncology, University Hospital of Regensburg, Regensburg, Germany.

Institute of Pathology, University of Regensburg, Regensburg, Germany.

出版信息

Ann Hematol. 2025 May;104(5):3019-3027. doi: 10.1007/s00277-025-06366-2. Epub 2025 Apr 21.

Abstract

Leukocytoclastic vasculitis (LCV) has been reported as a rare paraneoplastic phenomenon associated with several hematologic disorders, including indolent lymphomas such as Waldenström macroglobulinemia. However, there are very few cases of LCV in the context of plasma cell disorders. We present the case of a 58-year-old female who developed a rapidly progressive, ulceronecrotic skin lesion on her left lower leg due to leukocytoclastic vasculitis. The lesion was initially suspected to be an ulcerative chronic wound (ulcus cruris) but represented an atypical manifestation of leukocytoclastic vasculitis as primary and only clinical sign of smoldering multiple myeloma IgG kappa. After standard induction chemoimmunotherapy with daratumumab, bortezomib, lenalidomide, and dexamethasone, the patient proceeded to high-dose chemotherapy with melphalan, followed by autologous stem cell transplantation for consolidation. Despite a bacterial skin superinfection, myeloma treatment was successfully completed without any major complications. The skin lesion healed concurrently with the reduction in paraprotein levels, and there was no need for plastic surgical intervention. LCV mimicking ulcus cruris can represent a rare and atypical initial manifestation of plasma cell neoplasia. In this case report, systemic myeloma treatment proved to be effective for inducing complete remission of advanced ulceronecrotic skin damage. This case extends the spectrum of reported monocloncal gammopathies of cutaneous significance.

摘要

白细胞破碎性血管炎(LCV)已被报道为一种与多种血液系统疾病相关的罕见副肿瘤现象,包括惰性淋巴瘤,如华氏巨球蛋白血症。然而,在浆细胞疾病背景下的LCV病例非常少见。我们报告一例58岁女性,因白细胞破碎性血管炎在左小腿出现快速进展的溃疡性坏死性皮肤病变。该病变最初被怀疑是慢性溃疡性伤口(小腿溃疡),但却是白细胞破碎性血管炎的非典型表现,是冒烟型多发性骨髓瘤IgG κ型的主要且唯一临床体征。在接受达雷妥尤单抗、硼替佐米、来那度胺和地塞米松的标准诱导化疗免疫治疗后,患者接受了美法仑高剂量化疗,随后进行自体干细胞移植巩固治疗。尽管发生了皮肤细菌二重感染,但骨髓瘤治疗顺利完成,未出现任何重大并发症。皮肤病变随着副蛋白水平的降低而愈合,无需进行整形手术干预。模仿小腿溃疡的LCV可能是浆细胞肿瘤罕见且非典型的初始表现。在本病例报告中,全身性骨髓瘤治疗被证明对诱导晚期溃疡性坏死性皮肤损伤完全缓解有效。该病例扩展了具有皮肤意义的单克隆丙种球蛋白病的报道范围。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1ad8/12141142/69e622f4f563/277_2025_6366_Fig1_HTML.jpg

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