Wen Jingjing, Xu Fang, Li Min, Zhou Qiaoling, Qu Wen, Liu Yiping, Su Jing, Hu Hong
Hematology Department.
Rheumatology Department, Mianyang center hospital, Mianyang, China.
Medicine (Baltimore). 2019 Jul;98(28):e16382. doi: 10.1097/MD.0000000000016382.
Type I monoclonal cryoglobulinemia is usually associated with lymphoproliferative disorders, such as monoclonal gammopathy of undetermined significance (MGUS), myeloma, chronic lymphocytic leukemia (CLL) and lymphoplasmocytic lymphoma (LPL). Clinical symptoms related to Type I cryoglobulin (CG) isotype often include skin, neurological and renal manifestations.
A 42-year-old woman who initially presented urticaria, palpable purpura in both her upper extremities and legs, eosinophilia and Raynaud phenomenon. Skin biopsy revealed eosinophil infiltration. Monoclonal immunoglobulin (Ig) G-κprotein was detected and CG was also positive.
The patient was finally diagnosed as MGUS related Type I CG.
Cyclophosphamide-dexamethasone-thalidomide (CDT) therapy was initiated.
The treatment relieved the skin symptoms efficiently.
To our knowledge, this is a rare case of Type I cryoglobulinemic vasulitis with eosinophilia complicated by MGUS, and the effective treatment of cyclophosphamide combined with thalidomide and prednisone may provide a new therapeutic option for cryoglobulinemic vasulitis.
I型单克隆冷球蛋白血症通常与淋巴增殖性疾病相关,如意义未明的单克隆丙种球蛋白病(MGUS)、骨髓瘤、慢性淋巴细胞白血病(CLL)和淋巴浆细胞淋巴瘤(LPL)。与I型冷球蛋白(CG)同种型相关的临床症状通常包括皮肤、神经和肾脏表现。
一名42岁女性,最初出现荨麻疹、双上肢和腿部可触及的紫癜、嗜酸性粒细胞增多和雷诺现象。皮肤活检显示嗜酸性粒细胞浸润。检测到单克隆免疫球蛋白(Ig)G-κ蛋白,冷球蛋白也呈阳性。
患者最终被诊断为与MGUS相关的I型冷球蛋白血症。
开始使用环磷酰胺-地塞米松-沙利度胺(CDT)治疗。
治疗有效缓解了皮肤症状。
据我们所知,这是一例罕见的伴有嗜酸性粒细胞增多的I型冷球蛋白血症性血管炎合并MGUS的病例,环磷酰胺联合沙利度胺和泼尼松的有效治疗可能为冷球蛋白血症性血管炎提供一种新的治疗选择。