分析EB(Epstein-Barr virus)病毒相关性肺淋巴瘤样肉芽肿病(pulmonary lymphomatoid granulomatosis, PLG)合并噬血细胞综合征(hemophagocytic syndrome, HPS)的临床症状、诊断。

回顾性分析1例EB病毒相关性肺淋巴瘤样肉芽肿病合并噬血细胞综合征患者的临床症状、辅助检查和治疗过程，并进行文献复习。

患者，女性，66岁，因发热、咳嗽伴皮肤结节样瘀斑就诊，完善检查提示双肺多发结节灶、纵隔淋巴结肿大、脾大；血常规：白细胞(white blood cell)2.15×10⁹/L，血红蛋白(hemoglobin)77 g/L，血小板(blood platelets)67×10⁹/L；C-反应蛋白(C-reactive protein, CRP)24.98 mg/L，红细胞沉降率(erythrocyte sedimentation rate, ESR)35 mm/1 h、铁蛋白(ferritin)>2 000.00 μg/L、白介素2受体(Interleukin-2 receptor, IL-2R)7 500.00 U/ml、EB病毒定量阳性，乙肝表面抗原、乙肝e抗体、乙肝核心抗体阳性，肺泡灌洗液(bronchoalveolar lavage fluid, BALF)，二代测序(next-generation sequencing, NGS)提示EB病毒阳性，骨髓穿刺见PLT减少骨髓象。肺组织活检免疫组化提示CD20(＋)，Ki -67(＋)；特殊染色：PAS (－)，PASM(－)；原位杂交：EBER(＋)，病理诊断为淋巴瘤样肉芽肿不能除外。结合病史，最终诊断为噬血细胞综合征、肺淋巴样肉芽肿病、EB病毒感染、乙型肝炎小三阳、肺炎，予抗感染、抗炎、抗病毒等治疗后患者症状好转出院。患者出院后，症状反复，多次合并肺炎住院治疗，发病10个月后因发热、咳嗽至我科治疗，完善肺部结节样病灶穿刺提示B细胞淋巴瘤，转入血液科进一步治疗。

本文纳入EB病毒相关性肺淋巴瘤样肉芽肿病合并噬血细胞综合征患者为罕见病例，发病10个月后病情进展为B细胞淋巴瘤，并进行文献复习，旨在加深临床工作者对该病的认识。

To investigate the clinical manifestations and diagnosis of Epstein-Barr virus (EBV)-associated pulmonary lymphomatoid granulomatosis (PLG) complicated with hemophagocytic syndrome (HPS).

We retrospectively analyzed the clinical features, laboratory findings, and treatment course of a patient with EBV-associated PLG and HPS, along with a literature review.

A 66-year-old female presented with fever, cough, and cutaneous nodular ecchymosis. The comprehensive examination revealed multiple nodules in both lungs, mediastinal lymphadenopathy, and splenomegaly; blood routine test: white blood cell count 2.15×10⁹/L, hemoglobin 77 g/L, platelet count 67×10⁹/L; C-reactive protein (CRP) 24.98 mg/L, erythrocyte sedimentation rate 35mm/1 h, ferritin>2 000.00μg/L, interleukin-2 receptor 7 500.00 U/ml, Epstein-Barr virus quantification positive, hepatitis B surface antigen, hepatitis B e antibody, and hepatitis B core antibody positive; bronchoalveolar lavage fluid next-generation sequencing(NGS) indicated Epstein-Barr virus positivity; bone marrow aspiration showed decreased platelet count and a bone marrow picture. Lung tissue biopsy immunohistochemistry (IHC) shows CD20(+ ) and Ki-67(+ ). Special stains: PAS (-) and PASM (-). In situ hybridization: EBER (+ ). The final pathological diagnosis suggests lymphomatoid granulomatosis cannot be excluded. The final diagnosis included HPS, PLG, EBV infection, chronic hepatitis B, and pulmonary infection. Initial treatment with anti-infectives, anti-inflammatory agents, and antivirals led to symptomatic improvement. However, the patient experienced recurrent pulmonary infections post-discharge. Ten months after onset, she was readmitted for fever and cough, and repeat lung biopsy confirmed B-cell lymphoma, prompting referral to hematology.

This report describes a rare case of EBV-associated PLG progressing to B-cell lymphoma with concurrent HPS. The findings aim to enhance clinicians′awareness of this disease entity.