Department of Histopathology, PGIMER, Chandigarh, India.
Department of Internal Medicine, PGIMER, Chandigarh, India.
Int J Surg Pathol. 2024 Jun;32(4):838-844. doi: 10.1177/10668969231195068. Epub 2023 Sep 18.
Epstein-Barr virus (EBV) is a nonhepatotropic virus. It causes mild self-limiting illness characterized by fever, oral ulcer, diarrhea, lymphadenopathy, and hepatosplenomegaly. Rarely it causes acute liver failure (ALF). EBV-related ALF (EBV-ALF) accounts for 0.2% of all ALF cases. The prognosis of EBV-ALF is dismal, and it can affect both immunocompromised and immunocompetent individuals. We performed a partial autopsy on a 30-year-old immunocompetent individual with infectious mononucleosis and ALF. The autopsy showed characteristic histomorphology of EBV-ALF in the form of centrizonal confluent hepatocytic necrosis, portal mixed inflammatory infiltrate, sinusoidal lymphocytosis, numerous hemophagocytic figures, and tissue Epstein-Barr encoded RNA-in situ hybridization (EBER-ISH) positivity. Extensive hemophagocytosis was noted in the liver, spleen, lymph node, and bone marrow. Other features include T-zone expansion of lymph nodes and spleen, interstitial pneumonia pattern in the lungs, and exanthematous skin changes. EBV-DNA polymerase chain reaction from the postmortem blood sample yielded 70,200 copies/µL. The index case highlights the uncommon occurrence of EBV-ALF, its histomorphological features, and its putative pathogenesis.
EB 病毒(EBV)是非嗜肝病毒。它引起以发热、口腔溃疡、腹泻、淋巴结病和肝脾肿大为特征的轻度自限性疾病。罕见情况下会导致急性肝衰竭(ALF)。EBV 相关的 ALF(EBV-ALF)占所有 ALF 病例的 0.2%。EBV-ALF 的预后不佳,可影响免疫功能正常和免疫功能低下的个体。我们对一名 30 岁免疫功能正常的传染性单核细胞增多症和 ALF 患者进行了部分尸检。尸检显示 EBV-ALF 的特征组织形态学表现为中央融合性肝细胞坏死、门脉混合炎症浸润、窦状隙淋巴细胞增多、大量噬血细胞现象和组织 EBV 编码 RNA 原位杂交(EBER-ISH)阳性。肝脏、脾脏、淋巴结和骨髓中广泛存在噬血细胞现象。其他特征包括淋巴结和脾脏的 T 区扩张、肺部间质性肺炎模式和出疹性皮肤改变。从尸检后的血液样本中进行的 EBV-DNA 聚合酶链反应得出 70200 拷贝/µL。该病例突出了 EBV-ALF 的罕见发生、其组织形态学特征及其潜在发病机制。