Division of Infectious Diseases and Chronic Viral Illness Service, McGill University Health Centre, Royal Victoria Hospital, Glen Site, 1001 boulevard Decarie, Montreal, QC, H4A 3J1, Canada.
Research Institute, McGill University Health Centre, Montreal, QC, Canada.
BMC Infect Dis. 2018 May 8;18(1):210. doi: 10.1186/s12879-018-3128-3.
Angiocentric lymph proliferative disorder (ALPD) is a granulomatous lymphoproliferative condition associated with various primary and secondary immunodeficiency states. ALPD is so rare that its prevalence has not been established. Typically affecting middle-aged adults, this condition is often found in the context of Epstein Bar Virus infection and consists of angiocentric and angioinvasive pulmonary infiltrates. Herein, we present a biopsy-proven case of a patient manifesting with a viral meningoencephalomyelitis-like picture with brain, spinal cord, renal and splenic lesions. The diagnosis was confirmed to be ALPD in the context of newly diagnosed HIV infection.
A 35 year-old homosexual man presented with a 5-week history of headaches followed by a 3-week history of horizontal diplopia, limb weakness and right 6th cranial nerve palsy. Lumbar puncture revealed a lymphocytic pleocytosis, high protein and low glucose. Magnetic Resonance Imaging showed scattered lesions throughout the brain and spinal cord and Computed Tomography of the abdomen and pelvis revealed hypodensities involving the kidneys and spleen. HIV testing was positive, with a viral load of 11,096 copies/mL and CD4 count of 324 cells/μL. Serum Epstein Bar virus PCR was positive with 12,434 copies/ml. Right frontal brain biopsy revealed gray matter containing angiogentric cerebritis with organizing infarction but Epstein Bar Virus-in situ preparations were negative and no viral inclusions were identified. A diagnosis of ALPD (also known as lymphomatoid granulomatosis) was made. The patient was initiated on antiretroviral therapy and treated with intravenous rituximab every 3 weeks for 4 cycles and made progressive improvements. By the time of discharge his strength had improved and he was ambulating again although with a walker. Within 2 months, his HIV viral load was suppressed. Magnetic Resonance Imaging of the brain 6 months later revealed interval improvement. At his most recent follow-up, 34 months later, his neurological symptoms had almost completed resolved.
Albeit rare, ALPD should be considered in the differential diagnosis of central nervous system lesions in persons with HIV once common etiologies have been eliminated. Furthermore, ALPD involving the central nervous system may occur in in the absence of documented EBV infection in the central nervous system.
血管中心性淋巴增生性疾病（ALPD）是一种与各种原发性和继发性免疫缺陷状态相关的肉芽肿性淋巴增生性疾病。由于 ALPD 非常罕见，因此其患病率尚未确定。这种疾病通常影响中年成年人，常发生于 Epstein-Barr 病毒感染的背景下，表现为血管中心性和血管侵袭性肺浸润。在此，我们报告了一例经活检证实的患者，其表现为病毒性脑膜脑炎样表现，伴有脑、脊髓、肾脏和脾脏病变。在新诊断的 HIV 感染背景下，该诊断被确认为 ALPD。
一名 35 岁同性恋男性，因头痛 5 周，随后出现水平复视 3 周，伴四肢无力和右侧第 6 颅神经麻痹而就诊。腰椎穿刺显示淋巴细胞增多症、高蛋白和低糖。磁共振成像显示脑和脊髓有散在病变，腹部和骨盆计算机断层扫描显示肾脏和脾脏密度降低。HIV 检测阳性，病毒载量为 11,096 拷贝/ml，CD4 计数为 324 个/μl。血清 Epstein-Barr 病毒 PCR 阳性，拷贝数为 12,434。右额叶脑活检显示含血管中心性脑炎的灰质伴有组织性梗死，但 Epstein-Barr 病毒原位制备为阴性，未发现病毒包涵体。诊断为 ALPD（也称为淋巴瘤样肉芽肿病）。患者开始接受抗逆转录病毒治疗，并接受静脉注射利妥昔单抗每 3 周 1 次，共 4 个周期，病情逐渐改善。出院时，他的肌力有所改善，能够再次行走，尽管需要使用助行器。2 个月内，他的 HIV 病毒载量得到抑制。6 个月后，脑磁共振成像显示有间隔改善。在他最近的随访中，即 34 个月后，他的神经系统症状几乎完全缓解。
尽管罕见，但在排除常见病因后，应将 ALPD 纳入 HIV 感染者中枢神经系统病变的鉴别诊断中。此外，中枢神经系统受累的 ALPD 可能发生于中枢神经系统无明确 EBV 感染的情况下。
