Castleman病相关性嗜血细胞综合征一例报道并文献复习

doi:10.12114/j.issn.1007-9572.2019.00.658

摘要/Abstract

摘要： 背景　Castleman病（CD）及嗜血细胞综合征（HPS）均是罕见淋巴增生性疾病，病死率高，迄今尚缺乏标准的治疗方案。目的　加强对CD相关性HPS的认识，提高临床医师对该病的诊疗水平。方法　报道2017-08-22贵州省黔西南州人民医院收治的1例CD相关性HPS的患儿，结合相关文献，总结其诊断及治疗特点。结果　本例患儿表现为反复高热、肝脾大、淋巴结肿大伴全身水肿，曾误诊为败血症、传染性单核细胞增多症、淋巴瘤，并予反复抗感染、大剂量激素、丙种球蛋白等冲击治疗，病情无明显好转；通过正电子发射断层显像/计算机断层成像（PET-CT）检查及颈部淋巴结活检符合淋巴结多中心CD，且骨髓细胞学检查提示，嗜血细胞增多，最终考虑CD相关性HPS。针对白介素6（IL-6）通路靶向治疗后临床症状得到持续缓解。结论　CD是一种较为罕见的疾病，进展迅速，预后较差，且明确诊断需行组织病理学检查，而针对IL-6通路靶向治疗可能成为CD患者有效治疗方案。

关键词: 巨淋巴结增生；淋巴组织细胞增多症, 嗜血细胞性；Castleman病；治疗；病例报告

Abstract: Background　Castleman's disease（CD） and hemophagocytic syndrome（HPS） are rare lymphoid hyperplasia diseases with high mortality rate and no standard treatment currently.Objective　To improve clinicians' understanding，diagnostic and therapeutic levels of CD-related HPS.Methods　A case of CD-related HPS admitted to Qian Xi Nan People's Hospital on August 22，2017 was reported.His diagnosis and treatment were summarized with literature review.Results　This patient with recurrent fever，hepatosplenomegaly，lymphadenectasis and systemic edema was ever misdiagnosed as septicemia，infectious mononucleosis and lymphoma，and treated with pulse therapies including repeated anti-infective agents，high-dose hormones and gamma globulin，but no curative effects were achieved.Results of PET-CT scan and cervical lymph node biopsy indicated multicentric CD involving multiple lymph nodes.Bone marrow cytology showed increased hemophagocytosis.Altogether，CD-related HPS was considered.The patient was treated with IL-6 targeted therapy and continued remission was achieved.Conclusion　CD is a rare disease with rapid progression and poor prognosis.Histopathological examination is necessary for clear diagnosis.For treatment，IL-6 pathway targeted therapies may become an effective therapy for CD patients.

Key words: Giant lymph node hyperplasia；Lymphohistiocytosis, hemophagocytic；Castleman's disease；Treatment；Case reports

陈春江. Castleman病相关性嗜血细胞综合征一例报道并文献复习[J]. 中国全科医学, 2020, 23(14): 1804-1807. DOI: 10.12114/j.issn.1007-9572.2019.00.658.
CHEN Chunjiang. Castleman's Disease-related Hemophagocytic Syndrome：a Case Report and Literature Review　[J]. Chinese General Practice, 2020, 23(14): 1804-1807. DOI: 10.12114/j.issn.1007-9572.2019.00.658.

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