经典霍奇金淋巴瘤中枢神经系统非典型受累：一例病例报告
抽象的
霍奇金淋巴瘤是一种全身性疾病，常累及颈部、锁骨上和纵隔淋巴结。霍奇金淋巴瘤累及中枢神经系统的情况极为罕见，诊断通常通过活检组织上独特的形态学和免疫组织化学染色来确定。霍奇金淋巴瘤很少累及结外。与复发累及多个部位的患者相比，最初仅累及中枢神经系统或复发的患者预后良好。
我们在此报告一例 35 岁东南亚男性复发性霍奇金淋巴瘤病例。患者出现腮腺病变、颈部淋巴结肿大，体重明显减轻，间歇性盗汗。随着癌细胞扩散到中枢神经系统，患者高度怀疑患有结核病。经颈部淋巴结活检，患者被确诊患有霍奇金淋巴瘤。立即开始治疗，包括阿霉素、博来霉素、长春花碱和达卡巴嗪六个周期的化疗。患者接受了三个周期的化疗，包括异环磷酰胺、卡铂和依托泊苷，但随后失访。五年后，患者遭遇了一场交通事故。经检查，脑部计算机断层扫描发现右顶叶占位性病变，伴有中度脑水肿和中线移位。患者接受脑占位性病变切除术，组织病理学检查符合经典霍奇金淋巴瘤的特征。对此类病变进行细致检查以排除任何继发性疾病过程至关重要。
复发性霍奇金淋巴瘤累及中枢神经系统的情况相对罕见，迄今为止报告的病例仅有二十多例，在发展中国家很少见。因此，应始终检查占位性病变，对此类病变进行活检是确诊的金标准。通过及时适当的治疗，可以实现完全缓解。然而，大规模研究将是明智之举，以探索累及中枢神经系统的霍奇金淋巴瘤患者的症状、生存期和治疗方案。
背景
经典霍奇金淋巴瘤 (HL) 是一种淋巴系统癌症，主要影响淋巴结。与非霍奇金淋巴瘤 (NHL) 相比，HL 中枢神经系统 (CNS) 受累极为罕见，最近有研究表明，HL 患者中枢神经系统 (CNS) 受累的概率不到 0.2-0.5% [1]，而 NHL 患者中枢神经系统 (CNS) 受累的概率高达 5-30% [2]。结节性硬化和混合细胞是复发性疾病中最常见的 HL 组织学亚型。治疗方式包括单独放疗或放疗与化疗联合。我们描述了一名患有经典 HL 的患者，其脑部疾病复发。此类表现罕见且可用报告稀少，这对医生来说是一个难以诊断的问题。此外，这些表现可能会给中低收入国家的医生带来一个令人困惑的难题，在这些国家，及时诊断和管理可以减轻资源不足的医疗保健的现有负担。
病例介绍
A 35-year-old South-East Asian male, nonsmoker, with no known comorbidities, reported to outpatient facility in 2012, when he developed right parotid swelling and was managed conservatively. His swelling later transformed into an ulceration. He took multiple courses of antibiotics prescribed by general physicians and also used medication given by unqualified practitioners . Family history was negative for any malignancies. The patient belonged to a household of low socioeconomic status, being employed as a domestic worker. In a few months’ time, the patient developed progressive cervical lymphadenopathy with significant weight loss and intermittent night sweats. Due to high prevalence of tuberculosis in the local population, the patient underwent antituberculosis treatment (ATT) consisting of rifampicin (10 mg/kg), isoniazid (5 mg/kg), ethambutol (15 mg/kg), and pyrazinamide (25 mg/kg). However, no signs of improvement were observed after treatment for 2 months. During this time period, he developed an ulcerated lesion of the right parotid gland. Biopsy of the cervical lymph node revealed Hodgkin lymphoma, and the patient underwent six cycles of chemotherapy ABVD [adriamycin (25 mg/m2intravenous), bleomycin (10 U/m2intravenous), vinblastine (6 mg/m2intravenous), and dacarbazine (DTIC) (375 mg/m2intravenous)]. Each cycle was repeated every 28 days, and this combination was administered on day 1 and day 15. No significant reduction in size of the lymph nodes was observed after chemotherapy, and his parotid gland lesion remained unchanged. Later in June 2013, a biopsy of the ulcerated parotid gland lesion was performed with findings that were consistent with Hodgkin lymphoma. He received his second-line chemotherapy: three cycles of dexamethasone, high-dose Ara-C, and platinol (DHAP). Staging computed tomography (CT) was performed to evaluate the response of treatment, which showed no interval change in parotid lesion but a significant reduction in the size of cervical lymph nodes. Furthermore, 25 fractions of radiation therapy were administered to the patient at the right side of his face including right side of neck. After 10 months of treatment, disease progression was still observed with complaint of cervical lymphadenopathy. In 2014, the patient received three cycles of chemotherapy consisting of ifosfamide, carboplatin, and etoposide (ICE). The patient demonstrated noncompliance to further treatment options and regular hospital visits because of financial constraints, and was eventually lost to follow-up.
In 2019, the patient suffered a road traffic accident (RTA) after 5 years of disease. The cause of RTA was identified as loss of consciousness (LOC) as confirmed by the patient’s brother, who was with him during the accident. A right parietal well-circumscribed space-occupying lesion of around 6.7 × 4.9 cm with moderate cerebral edema and midline shift was found on CT brain imaging as shown in Fig.1. He regained his consciousness spontaneously after a few hours in the emergency department. On further questioning, the patient described intermittent mild headaches a few months prior to the accident. His general physical examination was normal, with few marks of skin abrasions on his limbs. His lab workup was also unremarkable. He was scheduled for elective surgery and eventually underwent a gross total resection of the space-occupying lesion of brain. The specimen was sent for analysis, and immunohistochemistry revealed atypical cell infiltrates largely positive for CD30, CD15, and weakly positive PAX5 but lack of expression for LCA, CD20, and CD3 as shown in Fig.2. Surprisingly, these features were consistent with classical Hodgkin lymphoma according to the World

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