人类免疫缺陷病毒和继发性中枢神经系统淋巴瘤患者的马尾综合征：病例报告
抽象的
继发性中枢神经系统淋巴瘤 (SCNSL) 是免疫功能低下患者的一种已知并发症，大多数病例涉及脑实质。由 SCNSL 引起的马尾综合征 (CES) 报告极其稀少，因为该解剖区域受累极为罕见。
我们报告了一例 46 岁非洲女性患者，该患者患有人类免疫缺陷病毒 (HIV)，在 SCNSL 背景下患上了 CES。外周血涂片中没有发现胚细胞。我们提供了文献综述、该患者临床进展的讨论以及放射学/组织病理学发现。患者最终对诱导化疗和高剂量甲氨蝶呤反应良好。
本病例报告表明，CES 虽然在临床情况下很少发生，但对于高危患者，尤其是神经系统发现异常的患者，应考虑 CES。及时识别可能可以防止永久性神经损伤，并避免采取更具侵入性的治疗干预措施。
关键点
继发性中枢神经系统淋巴瘤 (SCNSL) 是弥漫性大 B 细胞淋巴瘤 (DLBCL) 中罕见的表现。
CD4 计数低于 200 且有神经系统症状的患者需要进行钆增强 MRI 扫描。
患有急性尿路感染和虚弱的患者需要进行脊柱 MRI 检查以排除马尾综合征 (CES)。
R-CHOP 化疗和高剂量甲氨蝶呤已被证实对 SCNSL 具有死亡率益处。
背景
人类免疫缺陷病毒 (HIV) 患者罹患多种并发症的风险较高，包括中枢神经系统淋巴瘤 (CNSL)，这是一种侵袭性非霍奇金淋巴瘤 (NHL) [1]。CNSL 最常影响脑实质、脊柱、软脑膜和眼睛 [2,3]，但很少影响脊髓。CNSL 预后不良，中位总生存期不到 3 年，老年人的预后更差 [2]。我们介绍了一名成年女性 HIV 患者，她在 CNSL 背景下患上了 CES。
病例介绍
A 46-years old female presented to the emergency room (ER) complaining of bilateral, painful inguinal lymph nodes. She also endorsed progressive fatigue, fever, anorexia, and unintentional weight loss (twenty kilograms in the past month). Her past medical history was significant for Hashimoto’s hypothyroidism (diagnosed in 2010) and HIV (diagnosed in 2011). The patient was unaware she had HIV and was likely diagnosed in her home country of Cameroon. She immigrated to Canada in 2015, and had a medical assessment in 2016 with documentation indicating she had been diagnosed with HIV since 2011. Since at least 2015, the patient had not been on any antiretroviral therapy. To our knowledge, the patient did not have any AIDS defining illness. In terms of her obstetrical history, she was G2P2 and both pregnancies were spontaneous vaginal deliveries. There were no issues with the pregnancies. In Canada, she received social assistance benefits from the government for her living expenses. The patient was not employed but was enrolled in a social program to learn English and French, so that she could participate in the work force. She lived in an apartment with her two children (8 years old male, and 10 years old female). She had no extended family in Canada. Her home medications were levothyroxine (100 mcg PO daily), vitamin D (1000 IU PO daily) and calcium carbonate (500 mg PO BID); she was not on any antiretroviral therapy. The patient denied any smoking, drinking alcohol or using recreational drugs. CT abdomen-pelvis demonstrated enlarged inguinal and intra-abdominal lymph nodes. She was initially discharged from the ER to be seen in hematology clinic however she returned within a few weeks owing to visual and auditory hallucinations. Psychiatric assessment concluded there was an underlying organic cause for her presentation. On assessment, she was alert and oriented to person, place, and time. Her BP was 122/80, her HR 106 regular, her temperature was 36.7, and her oxygen saturation was 96% in room air. Abdominal exam demonstrated significant lymphadenopathy in the inguinal region. Neurological exam demonstrated pyramidal weakness in the right upper extremity as well as both lower extremities. She was hyperreflexive in both triceps and knee reflexes. She had reduced vibration sense in both her halluxes. Labs revealed a normocytic anemia (Hb 107 g/l MCV 90 fL, WBC 5.50 × 109/L, Plt 380 × 109/L) and a mild hypercalcemia (ionized Ca 1.48 mmol/l). LDH was 129 units/liter. Her peripheral blood smear demonstrated a few elliptocytes, rouleaux and polychromasia. It did not demonstrate any blasts. The HIV viral load was 123/mL & CD4 count was 89. She was COVID + but asymptomatic. Her TSH was 3.86 with a normal Free T4. Viral hepatitis panel was negative. Epstein bar virus (EBV) was positive with 6670 copies. Toxoplasmosis IgG was positive with but IgM was negative. Cytomegalovirus (CMV) IgG was positive. Syphilis, cryptococcus and strongyloides serologies were negative. Peripheral blood smear was negative for malaria. CT head, performed prior to lumbar puncture, was unremarkable. Cerebrospinal fluid (CSF) showed 39% lymphocytes and 16% blasts—the report did not state what type of blasts were found therefore the clinical significance of this finding was unclear. CSF cytology was negative for malignancy but showed rare monocytes. Flow cytometry was not performed on CSF. CSF glucose was low [0.4 mmol/L] and protein was high [0.46 g/l]. CSF fluid was negative for tuberculosis, herpes simplex virus (HSV-1 + 2), syphilis, and human polyomavirus two. No bone marrow biopsy was performed. CT chest demonstrated a right upper nodule in the chest with a ground glass appearance, but no thoracic lymphadenopathy. MRI brain with contrast showed significant confluent hyper signal T2 FLAIR within the subcortical and deep ventricular white matter of both cerebral hemispheres, corpus callosum, right posterior limb of the internal capsule and cerebral peduncle (Fig.1). There was also cranial nerve enhancement. Collectively, these findings were suspicious for leptomeningeal dissemination versus CNS infection. As such, the patient was initially started on ceftriaxone, ampicillin, vancomycin and acyclovir but the antibiotics were soon discontinued after further investigations. The patient was also started on nirmatrelvir/ritonavir (Paxlovid) for COVID. She was also started on bictegravir, emtricitabine, and tenofovir (Biktarvy). Despite a week of anti-retroviral therapy (ART) her CD4 count dropped to 49. A left inguinal node biopsy with flow cytometry showed diffuse large B-cell lymphoma (DLBCL). Flow cytometry also showed BL6, myc rearrangement (double-hit rearrangement), and CD20 positive. During her admiss

扫码免登录支付

本文章为付费文章，是否支付1元后完整阅读？

如果您已购买过该文章，[登录帐号]后即可查看

支付1元