Jabr Ra'ed, El Atrouni Wissam, Male Heather J, Hammoud Kassem A
Department of Internal Medicine, The University of Kansas Medical Center, Kansas City, KS 66160, USA.
Division of Infectious Diseases, The University of Kansas Medical Center, Kansas City, KS 66160, USA.
Can J Infect Dis Med Microbiol. 2019 Oct 1;2019:7107326. doi: 10.1155/2019/7107326. eCollection 2019.
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.
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.
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.
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.
组织胞浆菌病是一种具有多种临床表现的地方性真菌病。组织胞浆菌病相关噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见疾病，关于其治疗和预后的数据有限。我们描述了我院5例组织胞浆菌病相关HLH患者的临床特征、治疗及预后情况。本综述还总结了关于这种感染相关HLH实体的表现、治疗和预后的当前文献。
我们检索了我院2006年1月1日至2017年9月30日期间组织胞浆菌病相关HLH患者的电子病历。通过使用HLH - 04标准进行病历审查来确诊HLH。我们还检索了当前文献中的病例报告和病例系列。
我院纳入了5例组织胞浆菌病相关HLH病例。所有5例患者均在2010年后确诊。文献综述又发现了60例组织胞浆菌病相关HLH病例。最常见的基础疾病是HIV，占61%的病例。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具体治疗方法如下：9例患者仅接受类固醇治疗，6例患者仅接受静脉注射免疫球蛋白（IVIG），3例患者接受地塞米松和依托泊苷，2例患者接受依托泊苷、地塞米松和环孢素，2例患者接受类固醇和IVIG，1例患者接受阿那白滞素和IVIG。住院病死率为31%，大多数死亡发生在入院后两周内。
成人组织胞浆菌病相关HLH是一种罕见但严重的并发症，死亡率高。早期使用脂质体两性霉素B进行抗真菌治疗至关重要。在这个疾病实体中，像HLH - 04这样的免疫抑制治疗方案的启动应该个体化。
