Sidana Surbhi, Rajkumar S Vincent, Dispenzieri Angela, Lacy Martha Q, Gertz Morie A, Buadi Francis K, Hayman Suzanne R, Dingli David, Kapoor Prashant, Gonsalves Wilson I, Go Ronald S, Hwa Yi Lisa, Leung Nelson, Fonder Amie L, Hobbs Miriam A, Zeldenrust Steven R, Russell Stephen J, Lust John A, Kyle Robert A, Kumar Shaji K
Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Division of Nephrology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Am J Hematol. 2017 Jul;92(7):668-673. doi: 10.1002/ajh.24745. Epub 2017 May 26.
We describe a series of 102 patients diagnosed from January 1, 1990 to December 31, 2015 with Type 1 monoclonal cryoglobulinemia (MoC). Symptoms were seen in 89 (87%) patients, including: cutaneous symptoms in 64 (63%) patients, with purpura (n = 43, 42%) and ulcers/gangrene (n = 35, 34%) being most common; neurological findings in 33 (32%) patients, most frequently sensory neuropathy (n = 24, 24%); vasomotor symptoms, mainly Raynaud's phenomenon in 25 (25%); arthralgias in 24 (24%); and renal manifestations, primarily glomerulonephritis in 14 (14%) patients. An underlying lymphoproliferative disorder was identified in 94 (92%) subjects; MGUS-39, myeloma-20, lymphoplasmacytic lymphoma-21 and others-14. Treatment was initiated in 73 (72%) patients, primarily for cryoglobulinemia-related symptoms in 57. Treatment regimens consisted of: steroids ± alkylating agents in 29 (40%), novel myeloma therapies in 16 (22%), rituximab with alkylating agents in 12 (16%) and rituximab ± steroids in 11 (15%) patients; 22 patients received plasmapheresis. Six patients underwent autologous stem cell transplant. Cryocrit at treatment initiation, change in cryocrit and time to nadir cryocrit were predictive of symptom improvement. Treatment directed toward the underlying clonal disorder resulted in improvement (n = 47) or stabilization (n = 16) of symptoms in the majority of patients and disappearance of cryoglobulin in over one-half.
我们描述了1990年1月1日至2015年12月31日期间诊断为1型单克隆冷球蛋白血症（MoC）的102例患者。89例（87%）患者出现症状，包括：64例（63%）患者有皮肤症状，最常见的是紫癜（n = 43，42%）和溃疡/坏疽（n = 35，34%）；33例（32%）患者有神经系统表现，最常见的是感觉神经病变（n = 24，24%）；25例（25%）患者有血管舒缩症状，主要是雷诺现象；24例（24%）患者有关节痛；14例（14%）患者有肾脏表现，主要是肾小球肾炎。94例（92%）患者发现有潜在的淋巴增殖性疾病；意义未明的单克隆丙种球蛋白病-39例，骨髓瘤-20例，淋巴浆细胞淋巴瘤-21例，其他-14例。73例（72%）患者开始治疗，主要是针对57例与冷球蛋白血症相关的症状。治疗方案包括：29例（40%）患者使用类固醇±烷化剂；16例（22%）患者使用新型骨髓瘤治疗药物；12例（16%）患者使用利妥昔单抗联合烷化剂；11例（15%）患者使用利妥昔单抗±类固醇；22例患者接受了血浆置换。6例患者接受了自体干细胞移植。治疗开始时的冷球蛋白比容、冷球蛋白比容的变化以及冷球蛋白比容降至最低点的时间可预测症状改善情况。针对潜在克隆性疾病的治疗使大多数患者的症状得到改善（n = 47）或稳定（n = 16），并且超过一半的患者冷球蛋白消失。
