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肢端发绀和进行性皮肤坏死为表现的华氏巨球蛋白血症伴 I 型冷球蛋白血症:一例报告。

Acrocyanosis and Progressive Skin Necrosis as Manifestation of Waldenstrom Macroglobulinemia Associated With Type I Cryoglobulinemia: A Case Report.

机构信息

Shohada-E-Tajrish Hospital, Shahid Beheshti Univesity of Medical Science, Tehran, Iran.

Clinical Research Development Unit, Shohada-E-Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

出版信息

Int J Low Extrem Wounds. 2023 Sep;22(3):605-609. doi: 10.1177/15347346211026994. Epub 2021 Jun 24.

Abstract

Waldenstrom macroglobulinemia (WM), a rare malignant disorder, occurs as a result of abnormal proliferation of lymphocytes that produce immunoglobulin M. In rare cases, WM complicates by type I cryoglobulinemia. Type I cryoglobulinemia usually presents with cutaneous manifestations such as Raynaud's phenomenon, purpura, necrosis, and gangrene. Various medical conditions, including thrombotic events, rheumatologic disorders, and malignancies, may present with skin discoloration and necrosis. Patients suffering from malignant diseases who initially present with skin manifestations usually are misdiagnosed by physicians. Here, we describe a 72-year-old man presenting with a 6-month acrocyanosis and progressive skin necrosis who was misdiagnosed by physicians. Finally, he was diagnosed to have WM associated with type I cryoglobulinemia. Though uncommon, hematologic malignancies can present with cutaneous manifestations. In some cases, patients may manifest with skin disorders alone. Early and prompt treatment of these diseases may save the patient life, relieve patient symptoms, and increase life quality.

摘要

华氏巨球蛋白血症(WM)是一种罕见的恶性疾病,是由于产生免疫球蛋白 M 的淋巴细胞异常增殖所致。在极少数情况下,WM 会并发 I 型冷球蛋白血症。I 型冷球蛋白血症通常表现为皮肤症状,如雷诺现象、紫癜、坏死和坏疽。各种医学病症,包括血栓事件、风湿性疾病和恶性肿瘤,可能会出现皮肤变色和坏死。最初表现为皮肤症状的恶性疾病患者通常会被医生误诊。在这里,我们描述了一名 72 岁男性,他出现了 6 个月的肢端发绀和进行性皮肤坏死,被医生误诊。最终,他被诊断为 WM 合并 I 型冷球蛋白血症。虽然不常见,但血液系统恶性肿瘤也可能表现为皮肤症状。在某些情况下,患者可能仅表现为皮肤疾病。早期和及时治疗这些疾病可以挽救患者生命,缓解患者症状,提高生活质量。

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