PDF(Pubmed)
Abstract：
Disseminated histoplasmosis is a progressive granulomatous disease caused by Histoplasma capsulatum, which is an intracellular dimorphic fungus endemic to the Ohio and Mississippi River valleys in the United States. It is usually thought to be due to the failure of the activation of the T-cell-mediated immune response. Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal condition, in which histiocytes and lymphocytes build up in and damage organs and other blood cells. We present a 37-year-old man with a past medical history of systemic lupus erythematosus (SLE) complicated by lupus nephritis on immunosuppressive therapy who presented to the emergency department with hypotension and was admitted for acute kidney injury. Prior to the presentation, he had persistent fever, myalgias, cough, mild shortness of breath, and back pain. Computed tomography (CT) chest shows \"eggshell\" calcification; microbiology evaluation of peripheral blood smear revealed intracellular organism, morphologically consistent with H. capsulatum; and urine histoplasmosis antigen test confirmed the diagnosis of histoplasmosis. HLH diagnosis was made clinically after \"clinical and testing criteria\" were evaluated. Despite further management, he developed coagulopathy and sepsis, which led to his death. At autopsy, we found organomegaly of the liver, spleen, and kidneys. Microscopically, these enlarged organs show old fibrotic granulomas and granulomatous inflammation with suspected fungal organisms. Gomori\'s methenamine silver special stain confirmed these fungal organisms to be consistent with Histoplasma species (3-5 micron budding yeasts). This case highlights that physicians should be aware of the diagnostic challenge that disseminated histoplasmosis with HLH could pose in a patient with SLE, especially in patients on immunosuppression. Failure to recognize the infection promptly could lead to grievous complications and possibly death.
摘要：
播散性组织胞浆菌病是由荚膜组织胞浆菌引起的进行性肉芽肿病，这是一种细胞内二态真菌，在美国俄亥俄州和密西西比河流域特有。通常认为这是由于T细胞介导的免疫应答的激活失败。噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但可能致命的疾病，其中组织细胞和淋巴细胞在器官和其他血细胞中积聚并损害器官和其他血细胞。我们介绍了一名37岁的男性，其既往有系统性红斑狼疮（SLE）合并狼疮肾炎的病史，并接受免疫抑制治疗，他因低血压到急诊科就诊，并因急性肾损伤入院。在介绍之前，他持续发烧，肌痛,咳嗽,轻度呼吸急促,和背部疼痛。计算机断层扫描（CT）胸部显示“蛋壳”钙化；外周血涂片的微生物学评估显示细胞内生物，形态与荚膜H.一致；尿液组织胞浆菌病抗原检测证实了组织胞浆菌病的诊断。HLH诊断是在评估“临床和测试标准”后进行临床诊断。尽管有进一步的管理,他出现了凝血病和败血症,这导致了他的死亡。尸检时,我们发现了肝脏的器官肿大,脾,脾还有肾脏.微观上,这些增大的器官显示陈旧性纤维化肉芽肿和肉芽肿性炎症，怀疑有真菌。Gomori的亚甲基胺银特殊染色证实这些真菌与组织胞浆菌属（3-5微米出芽酵母）一致。该病例突出表明，医生应该意识到HLH播散性组织胞浆菌病可能对SLE患者造成的诊断挑战，尤其是免疫抑制患者。未能及时识别感染可能导致严重的并发症和可能的死亡。
