Bahmad Hisham F, Gogola Samantha, Elajami Mohamad K, Brathwaite Carole, Castellano-Sánchez Amilcar A, Sriganeshan Vathany, Omarzai Yumna
Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA.
Herbert Wertheim College of Medicine, Florida International University, Miami Beach, FL 33199, USA.
Diseases. 2022 Aug 19;10(3):54. doi: 10.3390/diseases10030054.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyper-inflammatory disorder that occurs due to immunologic dysregulation. HLH can be primary (hereditary) or secondary to infections, autoimmune diseases, immune deficiencies, metabolic diseases, drugs, or malignancies. Lymphoid neoplasms mostly accompany malignancy-associated HLH. We present a case of a 12-year-old boy with a history of precursor B lymphoblastic leukemia (B-ALL), who subsequently developed chemotherapy-induced acute myeloid leukemia (t-AML). The patient was admitted for febrile neutropenia and initial laboratory tests revealed hemophagocytic lymphohistiocytosis (HLH). The hospital course was complicated by multiple infections and septic shock. The patient received several broad-spectrum antimicrobials, dexamethasone, as well as a pericardial drain to drain the hemorrhagic pericardial effusion. Despite intervention, the patient expired, and an autopsy was performed. We provide a synopsis of the main autopsy findings.
噬血细胞性淋巴组织细胞增生症（HLH）是一种因免疫调节异常而引发的危及生命的高度炎症性疾病。HLH 可为原发性（遗传性），或继发于感染、自身免疫性疾病、免疫缺陷、代谢性疾病、药物或恶性肿瘤。淋巴系统肿瘤大多伴随恶性肿瘤相关的 HLH。我们报告一例 12 岁男孩，有前驱 B 淋巴细胞白血病（B-ALL）病史，随后发生化疗诱导的急性髓系白血病（t-AML）。该患者因发热性中性粒细胞减少症入院，初始实验室检查显示噬血细胞性淋巴组织细胞增生症（HLH）。住院期间病情复杂，出现多种感染及感染性休克。患者接受了多种广谱抗菌药物、地塞米松治疗，以及心包穿刺引流出血性心包积液。尽管进行了干预，患者仍死亡，并进行了尸检。我们提供主要尸检结果的概述。
