Kay Carl L, Rendo Matthew J, Gonzales Paul, Beganovic Sead G, Czader Magdalena
Brooke Army Medical Center, Ft. Sam Houston, TX, USA.
Hematology-Oncology, Indiana University School of Medicine, Indianapolis, IN, USA.
Case Rep Oncol Med. 2019 Sep 8;2019:9781065. doi: 10.1155/2019/9781065. eCollection 2019.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome characterized by clinical signs and symptoms of extreme inflammation. In adults, HLH is typically a complication of infections, autoimmune diseases, and malignancies. While the disease is often fatal, classic management of HLH revolves around early diagnosis and initiation of protocolized therapy. We present a case of a previously healthy 56-year-old female who developed distributive shock requiring intubation, vasopressors, and continuous venovenous hemofiltration. In the setting of multiple infectious syndromes, severe cytopenias, and rising direct hyperbilirubinemia, her diagnosis of HLH was confirmed. Therapy was initiated with dexamethasone and two doses of reduced-intensity etoposide based on the patient's clinical course. Over the next few weeks, she continued to improve on dexamethasone monotherapy and has maintained remission up to the present with complete resolution of her cytopenias and return of baseline renal function. Our case highlights the variability in the management of probable infection-associated HLH (IHLH) with a good patient outcome. We demonstrate the potential to treat IHLH with partial protocols and minimal chemotherapeutics.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的、以极度炎症的临床体征和症状为特征的高炎症综合征。在成人中，HLH通常是感染、自身免疫性疾病和恶性肿瘤的并发症。虽然该疾病往往是致命的，但HLH的经典治疗围绕早期诊断和启动标准化治疗展开。我们报告一例病例，一名56岁的既往健康女性出现分布性休克，需要插管、使用血管升压药和持续静静脉血液滤过。在多种感染综合征、严重血细胞减少和直接胆红素血症升高的情况下，她的HLH诊断得到证实。根据患者的临床病程，开始使用地塞米松和两剂低强度依托泊苷进行治疗。在接下来的几周里，她继续接受地塞米松单药治疗并持续改善，目前维持缓解状态，血细胞减少完全缓解，肾功能恢复至基线水平。我们的病例突出了可能的感染相关性HLH（IHLH）治疗的变异性以及良好的患者预后。我们证明了使用部分方案和最小剂量化疗药物治疗IHLH的潜力。
