Abstract
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.
Highlights
Haemophagocytic Lymphohistiocytosis (HLH), during HIV infection is a rare complication with a poor prognosis
The most frequent opportunistic infection in HIV patients is disseminated histoplasmosis, and it has long been the first cause of AIDSrelated death (Nacher et al, 2011)
In Latin America, it has been described as a neglected disease (Nacher et al, 2013), and the burden of disseminated histoplasmosis among persons with HIV is estimated to be on par with that of tuberculosis (Nacher et al, 2016; Adenis et al, 2018)
Summary
Haemophagocytic Lymphohistiocytosis (HLH), during HIV infection is a rare complication with a poor prognosis. The most frequent opportunistic infection in HIV patients is disseminated histoplasmosis, and it has long been the first cause of AIDSrelated death (Nacher et al, 2011). In Latin America, it has been described as a neglected disease (Nacher et al, 2013), and the burden of disseminated histoplasmosis among persons with HIV is estimated to be on par with that of tuberculosis (Nacher et al, 2016; Adenis et al, 2018). HLH has been previously reported among HIV patients with and without disseminated infections. Our initial hypothesis was that disseminated histoplasmosis was the main cause of HLH in HIV-infected persons and that antifungals targeting this pathogen would have a beneficial role on prognosis.
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