Introduction: Haemophagocytic Lymphohistiocytosis (HLH), during HIV infection is a rare complication with a poor prognosis. There are few data on HLH within the Amazon region. The objective was to describe epidemiological, clinical and therapeutic features of HIV-related HLH in French Guiana.

Methods: A retrospective analysis of adult HIV patients at Cayenne hospital with HLH between 2012 and 2015. A diagnosis of HLH was given if the patient presented at least 3 of 8 criteria of the HLH-2004 classification.

Results: Fourteen cases of HLH were tallied during the study period. The mean age was 46 years with a sex ratio of 1.8. The most frequent etiology of HLH was an associated infection (12/14). Confirmed disseminated histoplasmosis, was found in 10 of 14 cases, and it was suspected in 2 other cases. The CD4 count was below 200/mm³ in 13/14 cases. An HIV viral load >100,000 copies/ml was observed in 13/14 cases. An early treatment with liposomal amphotericin B was initiated in 12/14 cases. The outcome was favorable in 12/14 of all cases and in 10/12 cases involving histoplasmosis. Case fatality was 2/14 among all cases (14.3%) et 1/10 among confirmed disseminated histoplasmosis with HLH (10%). During the study period 1 in 5 cases of known HIV-associated disseminated histoplasmosis in French Guiana was HLH.

Conclusion: Histoplasmosis was the most frequent etiology associated with HLH in HIV-infected patients in French Guiana. The prognosis of HLH remains severe. However, a probabilistic empirical first line treatment with liposomal amphotericin B seemed to have a favorable impact on patient survival.

介绍：HIV 感染期间的噬血细胞性淋巴组织细胞增多症 (HLH) 是一种罕见的并发症，预后不良。亚马逊地区有关 HLH 的数据很少。目的是描述法属圭亚那 HIV 相关 HLH 的流行病学、临床和治疗特征。

方法：对 2012 年至 2015 年间卡宴医院患有 HLH 的成年 HIV 患者进行回顾性分析。如果患者符合 HLH-2004 分类的 8 个标准中的至少 3 个，则诊断为 HLH。

结果：研究期间统计了 14 例 HLH 病例。平均年龄为46岁，性别比为1.8。HLH 最常见的病因是相关感染 (12/14)。14 例中有 10 例确诊为播散性组织胞浆菌病，另外 2 例疑似为播散性组织胞浆菌病。13/14 例中CD4 计数低于 200/mm ³。在 13/14 例病例中观察到 HIV 病毒载量 >100,000 拷贝/ml。12/14 例开始使用脂质体两性霉素 B 进行早期治疗。12/14 的所有病例和 10/12 涉及组织胞浆菌病的病例结果良好。所有病例的病死率为 2/14 (14.3%)，确诊的播散性组织胞浆菌病伴 HLH 的病死率为 1/10 (10%)。研究期间，法属圭亚那已知的 HIV 相关播散性组织胞浆菌病病例中，五分之一为 HLH。

结论：组织胞浆菌病是法属圭亚那 HIV 感染者中与 HLH 相关的最常见病因。HLH 的预后仍然很严重。然而，脂质体两性霉素 B 的概率经验一线治疗似乎对患者生存产生有利影响。