Shoureshi Pouria, Ruiz Johanna, Abdulzahir Ahmad, Bisch Alexander L, Naddaf Naja, Gisel Justin
Department of Internal Medicine, Orange Park Medical Center, Orange Park, FL, USA.
Medical student, Department of Internal Medicine, Edward Via College of Osteopathic Medicine- Carolinas, Spartanburg, SC, USA.
Oxf Med Case Reports. 2020 Oct 23;2020(10):omaa082. doi: 10.1093/omcr/omaa082. eCollection 2020 Oct.
Haemophagocytic lymphohistiocytosis (HLH) is a rare immunological disorder that is accompanied by a high mortality rate when the underlying aetiology is miliary tuberculosis. We report a case of tuberculosis (TB)-associated HLH in a haemodialysis patient, from a TB-endemic region, who missed two sessions of dialysis before developing the primary symptoms of HLH. The patient presented with non-specific findings including pancytopenia, coagulopathy and transaminitis. Computer-tomography imaging and microbiology from bronchoalveolar lavage evidenced miliary tuberculosis. Further testing revealed the TB-associated-HLH characteristic pattern of thrombocytosis, leukopenia, transaminitis, hyperferritinemia and elevated fibrinogen. The patient initially demonstrated improvement after initiation of anti-TB therapy. However, soon thereafter began to paradoxically deteriorate and then expire from apparent tuberculosis-immune reconstitution inflammatory syndrome. This case highlights the importance of early diagnosis and treatment, and consequently of the utility of diagnostic systems such as the HScore in cases of high clinical suspicion.
噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的免疫紊乱疾病,当潜在病因是粟粒性肺结核时,其死亡率很高。我们报告了一例来自结核病流行地区的血液透析患者发生的与结核病(TB)相关的HLH病例,该患者在出现HLH的主要症状之前错过了两次透析治疗。患者表现出包括全血细胞减少、凝血病和转氨酶升高在内的非特异性症状。计算机断层扫描成像和支气管肺泡灌洗的微生物学检查证实为粟粒性肺结核。进一步检测发现了与TB相关的HLH的特征性模式,即血小板增多、白细胞减少、转氨酶升高、高铁蛋白血症和纤维蛋白原升高。患者在开始抗结核治疗后最初表现出病情改善。然而,此后不久病情反而开始恶化,随后因明显的结核免疫重建炎症综合征而死亡。该病例强调了早期诊断和治疗的重要性,以及因此在临床高度怀疑的病例中使用HScore等诊断系统的实用性。