以脊髓压迫症状为表现的Richter综合征1例并文献复习

目的:探讨少见部位侵犯的Richter综合征的临床特点、诊治方法与预后.方法:报道1例具脊髓硬膜外占位病变的Richter综合征,通过病理组织学、骨髓细胞学、免疫组织化学方法的实验室检查,结合文献复习,探讨其发病机制、诊断、治疗与预后特点.结果:患者诊断慢性淋巴细胞白血病(CLL)6年,接受氟达拉滨治疗后第3日出现双下肢瘫痪和排尿困难.经磁共振检查发现患者胸椎段硬脊膜外占位病变,经外科手术切除,病理检查证实为弥漫大B细胞淋巴瘤.结论:此例慢性淋巴细胞白血病进展为恶性度较高的淋巴瘤,即Richter综合征,而此例发生的硬脊膜外的侵犯尤为少见,应加深对其的认识以避免漏诊和误诊.该疾病预后不良.

Richter''''s syndrome with primary manifestation of spinal compression: report of a case and literature review

Objective:To report a case of Richter's syndrome with involvement of a rare site and investigate its clinical feature,diagnosis,therapy and prognosis.Methods:The patient diagnosed as Richter's syndrome with epidural involvement in the vertebral canal was reported.The histology,cellular morphology of bone marrow,immunohistochemical and pathological examinations of this case were detected.Through literature review,the clinical feature,pathogenesis,diagnosis,therapy and prognosis of Richter's symdrome were investigated.Results:A 44-year-old male had previously diagnosed chronic lymphocytic leukemia(CLL),presenting only leucocytosis(lymphocytosis)for 6 years.He began to paralysis on both lower extremities and dysuresia 3 days after receiving therapy of fludarabine.Spinal magnetic resonance imaging(MRI)revealed an expansive process outside the endorachis in thoracic segments.Extirpation of the lesion was conducted and the pathological examination indicated a diffuse large B cell lymphomas.Conclusion:The occurrence of a diffuse large cell non Hodgkin's lymphoma in a CLL patient was known as Richter's syndrome.It is the most serious complication of CLL.Richter's syndrome rarely happened in the vertebral canal.Understanding of this syndrome should be deepened to avoid of missed diagnosis and misdiagnosis.Treatment options for these patients are limited and the prognosis is unfavourable.