一名24岁的厄瓜多尔女性,先前在怀孕期间诊断为急性脂肪肝(AFL),出现了宪法症状,黄疸,以及随后怀孕时的腹痛,提示提示AFL复发的调查。她接受了选择性堕胎,这导致了她腹痛的缓解,还有肝脏活检,表现为肉芽肿性炎症和淋巴细胞浸润。她后来出现腹胀,生产性咳嗽,持续的全身症状和黄疸。广泛的实验室和影像学研究表明败血症,急性肝损伤,和弥散性血管内凝血病。她的血清EB病毒(EBV)水平升高。她先前的肝活检的特殊染色显示EBV阳性自然杀伤(NK)细胞。骨髓活检也显示EBV阳性NK细胞。她被诊断为有或没有慢性活动性EBV(CAEBV)的侵袭性NK细胞白血病(ANKL)。治疗包括地塞米松,atovaquone,硼替佐米,还有更昔洛韦,有干细胞移植的计划.然而,她的病程因感染和多器官衰竭而变得复杂,导致她过世。这个案例凸显了管理与EBV相关的ANKL的稀有性和挑战,强调需要早期发现和改进治疗方案,干细胞移植提供最好的预后。
A 24-year-old Ecuadorian female, previously diagnosed with acute fatty liver (AFL) during pregnancy, developed constitutional symptoms, jaundice, and abdominal pain in a subsequent pregnancy, prompting investigations that suggested a recurrence of AFL. She underwent an elective abortion, which resulted in the resolution of her abdominal pain, and a liver biopsy, which showed granulomatous inflammation and lymphocytic infiltration. She later presented with abdominal distention, productive cough, and persistent constitutional symptoms and jaundice. Extensive laboratory and imaging studies indicated sepsis, acute liver injury, and disseminated intravascular coagulopathy. Her serum Epstein-Barr virus (EBV) level was elevated. Special staining of her previous liver biopsy revealed EBV-positive natural killer (NK) cells. A bone marrow biopsy also revealed EBV-positive NK cells. She was diagnosed with aggressive NK cell leukemia (ANKL) with or without chronic active EBV (CAEBV). Treatment included dexamethasone, atovaquone, bortezomib, and ganciclovir, with plans for a stem cell transplant. However, her course was complicated by infections and multi-organ failure, resulting in her passing. This case highlights the rarity and challenges in managing EBV-associated ANKL, emphasizing the need for early detection and improved treatment options, with stem cell transplantation offering the best prognosis.