Infection and Drug Resistance (Dec 2019)
Talaromycosis-Associated Secondary Hemophagocytic Lymphohistiocytosis in Nine Human Immunodeficiency Virus-Negative Patients: A Multicenter Retrospective Study
Abstract
Mianluan Pan, Ye Qiu, Wen Zeng, Shudan Tang, Xin Feng, Jingmin Deng, Xuan Wei, Zhiyi He, Jianquan Zhang Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning, People’s Republic of ChinaCorrespondence: Jianquan ZhangDepartment of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi, People’s Republic of ChinaTel +86 139 7812 3845Fax +86 0771 535 0031Email [email protected]: Talaromyces marneffei (T.M) is an intracellular opportunistic fungus that causes invasive mycosis in patients with or without human immunodeficiency virus (HIV) infection. Hemophagocytic lymphohistiocytosis (HLH) caused by T.M infection is extremely rare. Here, we analyzed the clinical features, immune mechanisms, treatment, and prognosis related to this comorbidity.Patients and Methods: This retrospective study was conducted between August 2012 and February 2019 at multiple research centers. Patients who presented with culture and/or histopathological proof of talaromycosis-associated HLH were included.Results: HIV-negative patients (n = 126) were enrolled. Of nine patients with T.M infection combined with secondary HLH, six were preschool children (five boys and one girl), and three were adults (two men and one woman). Seven of these nine had underlying diseases or recurrent infections. The most common symptoms were fever, anemia, hypoproteinemia, cough, weight loss, oral thrush, lymphadenopathy, hepatomegaly, splenomegaly, digestive symptoms, joint pain, and dyspnea. All patients showed reduced hemoglobin concentrations and platelet numbers. Liver dysfunction, hyperferritinemia, elevated lactate dehydrogenase, and low natural killer cell numbers were observed. Eight of nine patients received antifungal therapy, one patient did not receive therapy, and two of nine patients received anti-HLH therapy. Four died during treatment.Conclusion: T.M fungemia associated with HLH was related to high mortality. Once diagnosed, timely and effective antifungal treatments and supportive care are essential.Keywords: Talaromyces marneffei, hemophagocytic lymphohistiocytosis, human immunodeficiency virus, prognosis
