Division of Neurosurgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.
BMC Neurol. 2012 Aug 3;12:64. doi: 10.1186/1471-2377-12-64.
Lymphocyte-depleted Hodgkin's lymphoma is the rarest form of classical Hodgkin's lymphoma, accounting for < 1% of all cases. Patients often have advanced-stage disease at the time of presentation with an aggressive clinical course. Even more uncommon is primary extranodal disease and rarely it will be presenting with spinal cord compression.
An 88-year-old Caucasian female presented with a history of upper back pain for several months and new onset bilateral leg numbness and weakness. MRI of the spine showed a dorsal epidural lesion with cord compression at T1-T4 with involvement of the paraspinal muscles. The patient received urgent surgical decompression, with final histopathology showing a lymphocyte-depleted Hodgkin's lymphoma. Systemic work-up did not show evidence of nodal disease. Following surgery, she received a course of radiotherapy with good outcome.
To the best of our knowledge, this is the first reported case of primary lymphocyte-depleted Hodgkin lymphoma presenting as epidural spinal cord compression. Our report, in conjunction with a review of the literature, suggests that surgical intervention is clearly indicated in de novo disease followed by radiotherapy.
淋巴细胞耗竭型霍奇金淋巴瘤是经典霍奇金淋巴瘤中最罕见的一种,占所有病例的<1%。患者在就诊时通常处于晚期疾病,具有侵袭性的临床病程。更为罕见的是原发性结外疾病,且很少会出现脊髓压迫。
一位 88 岁白人女性,因上背部疼痛数月,新出现双侧腿部麻木和无力而就诊。脊柱 MRI 显示 T1-T4 背侧硬膜外病变,脊髓受压,伴脊柱旁肌肉受累。患者接受了紧急手术减压,最终的组织病理学检查显示为淋巴细胞耗竭型霍奇金淋巴瘤。全身检查未发现淋巴结疾病的证据。手术后,她接受了放疗,效果良好。
据我们所知,这是首例报告的原发性淋巴细胞耗竭型霍奇金淋巴瘤表现为硬膜外脊髓压迫的病例。我们的报告结合文献复习表明,对于新发疾病,明确的治疗方案是手术干预后进行放疗。