以皮损为首发表现的瓦尔登斯特伦巨球蛋白血症  

A case of Waldenstrom's macroglobulinemia with skin lesions as the first manifestation

作　　者：毛丽旦 王鹏[1] 康晓静[1] MAO Lidan;WANG Peng;KANG Xiaojing(Department of Dermatology and Venereology,People's Hospital of Xinjiang Uygur Autonomous Region,Xinjiang Clinical Research Center For Dermatologic Diseases,Xinjiang Key Laboratory of Dermatology Research(XJYS1707),Urumqi 830002,China)

机构地区：[1]新疆维吾尔自治区人民医院皮肤性病科、新疆皮肤病临床医学研究中心、新疆皮肤病研究重点实验室(XJYS1707),新疆乌鲁木齐830002

出　　处：《临床皮肤科杂志》2024年第8期485-487,共3页Journal of Clinical Dermatology

摘　　要：报告1例以皮损为首发表现的瓦尔登斯特伦巨球蛋白血症(Waldenstrom's macroglobulinemia,WM)。患者男,72岁,因周身皮肤肿块3年,淋巴结增大2年入院。皮肤科检查:周身可见弥漫分布的暗褐色至紫红色的斑疹、斑片、斑块及结节样损害,双侧腹股沟区可触及数枚呈串珠样排列的增大淋巴结。患者血清中免疫球蛋白M(IgM)升高,骨髓活检提示有核细胞增生极度活跃。皮损组织病理结果提示真皮全层密集的淋巴样细胞浸润。MYD88基因L265P突变阳性。诊断:WM(Ⅳ期B)。治疗:伊布替尼联合苯达莫司汀+利妥昔单抗(BR方案)化疗。A case of 72-year-old mala presented with Waldenstrom's macroglobulinemia,with skin lesions as the first manifestation.The patient was admitted to the hospital due to a generalized skin mass for three years and enlarged lymph nodes for two years.Dermatological examination showed dark brown to purplish red spots,patches,plaques and nodular lesions all over the body,and several swollen lymph nodes arranged like beads were touched in bilateral groin areas.A diagnosis of WM was established based on the increase of monoclonal immunoglobulin M(IgM)in serum,the extremely active lymphoid cell proliferation in bone marrow biopsy,the positive characteristic immunophenotype in immunohistochemistry,and the positive mutation of MYD88 gene L265P.Diagnosis:WM(stage ⅣB).Treatment:Imbruvica combined with BR regimen chemotherapy.

关 键 词：瓦尔登斯特伦巨球蛋白血症 皮肤肿块 

分 类 号：R55[医药卫生—血液循环系统疾病]