Background. Histoplasmosis is an endemic fungal disease with diverse clinical presentations. Histoplasmosis-associated hemophagocytic lymphohistiocytosis (HLH) is a rare disorder with limited data regarding treatment and outcome. We described the clinical features, treatment, and outcomes of five patients in our institution with histoplasmosis-associated HLH. This review also summarizes the current literature about presentation, treatment, and outcome of this infection-related HLH entity. Methods. We searched the electronic medical records for patients with histoplasmosis-associated HLH at our institution from 1/1/2006 to 9/30/2017. Diagnosis of HLH was confirmed by chart review using the HLH-04 criteria. We also searched the current literature for case reports and case series. Results. Five cases of histoplasmosis-associated HLH were included from our institution. All five patients were diagnosed after 2010. The literature review yielded 60 additional cases of histoplasmosis-associated HLH. The most common underlying condition was HIV in 61% of cases. The majority of histoplasmosis patients (81%) were treated with amphotericin B formulations. Documented specific treatments for HLH were as follows: nine patients received steroids only, six patients received intravenous immunoglobulin (IVIG) only, three patients received dexamethasone and etoposide, two patients received etoposide, dexamethasone, and cyclosporine, two patients received steroids and IVIG, and one patient received Anakinra and IVIG. The inpatient case fatality rate was 31% with most of the deaths occurring within two weeks of hospital admission. Conclusions. Histoplasmosis-associated HLH among adults is an uncommon but serious complication with high associated mortality. Early antifungal therapy with a lipid formulation amphotericin B is critical. The initiation of immunosuppressive therapy with regimens like HLH-04 in this disease entity should be individualized.

中文翻译：

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组织胞浆菌病相关的噬血细胞性淋巴组织细胞增多症：文献综述。

背景。组织胞浆菌病是一种具有多种临床表现的地方性真菌病。组织胞浆菌病相关的噬血细胞性淋巴组织细胞增多症 (HLH) 是一种罕见的疾病，有关治疗和结果的数据有限。我们描述了我们机构中 5 名组织胞浆菌病相关 HLH 患者的临床特征、治疗和结果。本综述还总结了当前有关这种感染相关 HLH 实体的表现、治疗和结果的文献。方法。我们在 2006 年 1 月 1 日至 2017 年 9 月 30 日期间检索了我们机构组织胞浆菌病相关 HLH 患者的电子病历。HLH 的诊断通过使用 HLH-04 标准的图表审查来确认。我们还检索了当前文献中的病例报告和病例系列。结果. 我们的机构包括 5 例组织胞浆菌病相关的 HLH。所有 5 名患者均在 2010 年之后确诊。文献回顾发现另外 60 例组织胞浆菌病相关 HLH 病例。在 61% 的病例中，最常见的潜在疾病是 HIV。大多数组织胞浆菌病患者 (81%) 接受了两性霉素 B 制剂治疗。记录的 HLH 特定治疗如下：9 名患者仅接受类固醇，6 名患者仅接受静脉注射免疫球蛋白 (IVIG)，3 名患者接受地塞米松和依托泊苷，2 名患者接受依托泊苷、地塞米松和环孢素，2 名患者接受类固醇和 IVIG，以及一名患者接受了 Anakinra 和 IVIG。住院病死率为31%，大部分死亡发生在入院两周内。结论。成人组织胞浆菌病相关 HLH 是一种罕见但严重的并发症，相关死亡率很高。使用脂质制剂两性霉素 B 进行早期抗真菌治疗至关重要。在这种疾病实体中使用 HLH-04 等方案开始免疫抑制治疗应个体化。