伴Castleman病的血液学检查阴性POEMS综合征一例并文献复习  

POEMS syndrome coexisting with Castleman disease and negative results of hematological examination: report of one case and review of literature

作　　者：方芳 郭轶先[1] 笪宇威[2] 苏力[1] 孙婉玲[1] Fang Fang;Guo Yixian;Da Yuwei;Su Li;Sun Wanling(Department of Hematology,Xuanwu Hospital,Capital Medical University,Beijing 100053,China;Department of Neurology,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院血液科,北京100053 [2]首都医科大学宣武医院神经内科,北京100053

出　　处：《白血病．淋巴瘤》2018年第11期664-669,共6页Journal of Leukemia & Lymphoma

摘　　要：目的加强对血液学检查阴性的不典型POEMS综合征的认识,提高POEMS综合征罕见病例的临床诊断率。方法回顾性描述2017年11月首都医科大学宣武医院血液科收治的1例罕见的血液学阴性、伴有Castleman病的POEMS综合征患者的诊疗经过,并进行文献复习。结果患者老年男性,临床疑诊POEMS综合征,但血清、尿液及骨髓检测均未发现单克隆浆细胞疾病的证据,确诊困难。全身PET-CT结果显示左侧髂骨存在高代谢病灶。在淋巴结活组织检查明确Castleman病的前提下,再次对患者髂骨病灶进行活组织检查术,术后病理回报为浆细胞瘤(λ轻链型),从而明确了血液学检查阴性POEMS综合征的诊断。文献复习示免疫固定电泳可提高POEMS综合征诊断率。骨髓活组织检查、流式细胞术及全身PET-CT可协助寻找单克隆浆细胞疾病的证据。对于存在Castleman病的POEMS综合征,通过周围神经病变、骨病、治疗反应有助于与独立存在的Castleman病相鉴别。结论当临床疑诊POEMS综合征的强制临床诊断标准不足时,应当积极寻找相关证据。对具有多发病灶的患者,多部位活组织检查是协助诊断的必需手段。Objective To strengthen the recognition of atypical POEMS syndrome in order to improve diagnosis rate of rare cases of POEMS syndrome. Methods The diagnosis and treatment of a rare case of POEMS syndrome coexisting with Castleman disease but without M protein in serum, urine and bone marrow who was admitted to Xuanwu Hospital, Capital Medical University in November 2017 were retrospectively analyzed with review of the literature. Results The elder male was suspected with a diagnosis of POMES syndrome, but absence of monoclonal plasma cell disease that made it difficult to diagnose. Systemic PET-CT found an active metabolic lesion in left iliac bone. Although the lymph node biopsy had been performed for a diagnosis of Castleman disease, a bone biopsy was also done for a definite diagnosis. Pathological result indicated a plasmacytoma which confirmed a diagnosis of POEMS syndrome without M protein in serum, urine and bone marrow. Literature review suggested that the application of immunofixation electrophoresis was helpful to improve the diagnostic rate of POEMS syndrome. For patients with a suspected diagnosis of POEMS syndrome, bone biopsy, flow cytometry and systemic PET-CT may assist in the search for monoclonal plasma cell. Periphery neuropathy, bone lesion and treatment response were helpful in distinguishing Castleman disease coexisting with POEMS syndrome from Castleman disease without POEMS syndrome. Conclusions When a mandatory major criterion of POEMS syndrome is not sufficient, it should be actively sought, especially for patient with a suspected diagnosis of POEMS syndrome. For patients with multiple lesions, multi-site biopsies are necessary to assist in diagnosis.

关 键 词：POEMS综合征 CASTLEMAN病 浆细胞瘤 免疫电泳 活组织检查 

分 类 号：R597[医药卫生—内科学]