Mohyuddin Ghulam Rehman, Male Heather J
Kansas University Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA.
Division of Hematologic Malignancies and Cellular Therapeutics (HMCT), Department of Internal Medicine, University of Kansas Cancer Center, 2330 Shawnee Mission Parkway, Mailstop 5003, Westwood, KS 66205, USA.
Case Rep Hematol. 2016;2016:4839146. doi: 10.1155/2016/4839146. Epub 2016 Aug 3.
Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome characterized by excessive activation of the immune system. Bacterial infections are very rare precipitants of this disease. A 19-year-old gentleman presented with headache, fatigue, and malaise. He was found to be hypotensive, tachycardic, and febrile. Broad spectrum antibiotics were initiated, and a lumbar puncture ruled out meningitis. Patient progressively developed shock that required use of vasopressors, as well as renal and respiratory failure. Blood cultures grew Fusobacterium necrophorum. Given continued fevers despite appropriate antimicrobials, a bone marrow biopsy was performed revealing increased histiocytes with hemophagocytosis. Dexamethasone was added with dramatic clinical improvement. Our case highlights Fusobacterium as a rare precipitant of HLH and proves that a high index of clinical suspicion is crucial for early diagnosis of HLH, allowing for prompt initiation of HLH-specific immunosuppressive therapy that can be life-saving.
噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的综合征,其特征为免疫系统过度激活。细菌感染是该疾病非常罕见的诱因。一名19岁男性出现头痛、乏力和不适症状。检查发现他有低血压、心动过速和发热。开始使用广谱抗生素,腰椎穿刺排除了脑膜炎。患者逐渐发展为休克,需要使用血管升压药,同时出现了肾衰竭和呼吸衰竭。血培养结果显示坏死梭杆菌生长。尽管使用了适当的抗菌药物,患者仍持续发热,于是进行了骨髓活检,结果显示组织细胞增多并伴有噬血细胞现象。加用地塞米松后临床症状显著改善。我们的病例突出了坏死梭杆菌作为HLH罕见诱因的情况,并证明高度的临床怀疑对于HLH的早期诊断至关重要,从而能够及时启动可挽救生命的HLH特异性免疫抑制治疗。