Staten Island University Hospital, Staten Island, New York, NY, USA.
Int J Gen Med. 2012;5:647-53. doi: 10.2147/IJGM.S32993. Epub 2012 Jul 31.
The prevalence of paraneoplastic neurologic syndrome in cancer is 0.01%. Neurological syndromes can be seen in chronic lymphocytic leukemia (CLL) and mostly present as either leukemic infiltration of the central nervous system (CNS) or progressive multifocal leukoencephalopathy. To our knowledge, this is the first reported case of combined sensory-motor neuropathy, myopathy, and dermatitis in a patient with CLL.
A 61-year-old African American man presented with acute dysphagia, rapidly progressive proximal limb-girdle weakness, and dermatitis. He had a white blood cell (WBC) count of 14,600/mm(3), hemoglobin of 11.4 mg/dL, and a platelet count of 165,000/mm(3). Lymphocytes comprised 15% of the total WBC with an absolute lymphocyte count of 2100/mm(3). Metabolic profile was unremarkable except for a serum creatine phosphokinase (CPK) level of 1056 mg/dL. Serum protein electrophoresis, serologic studies for autoimmune, genetic diseases, and paraneoplastic syndromes were all negative. Electrodiagnostic studies revealed sensorimotor neuropathy with mixed axonal and demyelinating features. Muscle biopsy revealed discrete areas of interstitial fibrosis juxtaposed to areas of intact muscle without any inflammation. At that point, a bone marrow biopsy was done because of anemia and slightly elevated mean corpuscular volume of 103. Bone marrow biopsy revealed minimal involvement with CD5/CD19-positive CLL. Flow cytometry demonstrated monoclonal CD5/CD19/CD20/CD23-positive cells, with dim kappa expression, and negative FMC-7 and CD3. This case doesn't meet the criteria for CLL/small lymphocytic lymphoma. However, considering the possibility of paraneoplastic phenomenon for his symptoms, it was decided to start the patient on CLL-directed therapy with Rituximab and Cyclophosphamide. After only two cycles, the patient experienced a dramatic improvement in his muscle strength with disappearance of the rash.
This case highlights a unique clinical picture of inflammatory dermatitis with electromyography and biopsy findings suggestive of myopathy and combined sensorimotor neuropathy with response to CLL-directed therapy. Also the symptoms started before peripheral lymphocytosis which masked the diagnosis for over a year.
癌性副肿瘤神经综合征在癌症中的患病率为 0.01%。神经综合征可见于慢性淋巴细胞白血病（CLL），主要表现为中枢神经系统（CNS）白血病浸润或进行性多灶性白质脑病。据我们所知，这是首例报道的 CLL 患者同时出现感觉运动神经病、肌病和皮炎的病例。
一名 61 岁的非裔美国男性因急性吞咽困难、进行性近端肢体无力和皮疹就诊。他的白细胞（WBC）计数为 14600/mm³，血红蛋白为 11.4mg/dL，血小板计数为 165000/mm³。淋巴细胞占总 WBC 的 15%，绝对值为 2100/mm³。除血清肌酸磷酸激酶（CPK）水平为 1056mg/dL 外，代谢谱无明显异常。血清蛋白电泳、自身免疫、遗传疾病和副肿瘤综合征的血清学研究均为阴性。电诊断研究显示感觉运动神经病伴混合轴索性和脱髓鞘特征。肌肉活检显示，离散的间质纤维化区域与无炎症的完整肌肉区域相邻。此时，由于贫血和平均红细胞体积稍高（103），进行了骨髓活检。骨髓活检显示 CLL 轻度受累，CD5/CD19 阳性。流式细胞术显示单克隆 CD5/CD19/CD20/CD23 阳性细胞，kappa 表达微弱，FMC-7 和 CD3 阴性。该病例不符合 CLL/小淋巴细胞淋巴瘤的标准。然而，考虑到他的症状可能是副肿瘤现象，决定开始对患者进行 CLL 定向治疗，使用利妥昔单抗和环磷酰胺。仅两个周期后，患者的肌肉力量显著改善，皮疹消失。
本病例突出了一种独特的临床特征，即炎症性皮炎，肌电图和活检结果提示肌病和感觉运动神经病合并，对 CLL 定向治疗有反应。此外，症状在周围淋巴细胞增多之前开始，这掩盖了诊断超过一年。
