DisseminatedTalaromyces marneffeiInfection With STAT3-Hyper-IgE Syndrome: A Case Series and Literature Review

Abstract

Background Little is known about the clinical characteristics of talaromycosis with hyper–immunoglobulin E syndrome (HIES). Methods We conducted a multicenter retrospective study, which included 7 hospitals from 2016 to 2022. Five consecutive cases of human immunodeficiency virus (HIV)–negative patients with systemic Talaromyces marneffei infections due to STAT3-HIES were identified. A systematic literature review of original articles published in English identified an additional 7 cases. Clinical characteristics and laboratory parameters were collected. Results Forty-two percent (5/12) of patients were young adults. The main symptoms of 10 patients were similar: fever (75%), cough (75%) and dyspnea (33%), but two patients mainly had gastrointestinal symptoms. Most patients had a history of infections since infancy. T marneffei was cultured from the bronchoalveolar lavage fluid (50%) and 25% of patients were next-generation sequencing positive. Eight patients had significantly elevated serum immunoglobulin E, increased B cells and decreased natural killer cells. There were ten different STAT3 mutations, three of which were reported for the first time in this study. Chest computed tomography examinations showed multiple exudations with cavities in the lungs. Voriconazole combined with thymosin was effective. Despite given antifungal agents, most had poor outcomes and the case fatality rate was as high as 25%. Conclusions STAT3-HIES is most likely a susceptibility factor for T marneffei infections among HIV-negative patients, which has a high case fatality rate. Increased awareness among clinicians is necessary to help in early diagnosis.

Case report: Diagnosis of Talaromyces marneffei infection in an HIV-negative patient with septic shock and high-titer anti-interferon gamma autoantibodies by metagenomic next-generation sequencing

“…( Chen et al., 2020 ). From the literature review, we can see that the immunoglobulin profile was not the same, some patients showed high IgM, some showed low IgM ( You et al., 2021 ; Li et al., 2023 ). The reported trend of IgA, IgE and IgM in HIV-Negative patients with disseminated Talaromyces marneffei infection with high-titer anti-interferon gamma autoantibodies is not completely consistent, indicating that patients are heterogeneous.…”

Ultrasound characterization of superficial lymph nodes in HIV patients with Talaromyces marneffei infection

“…The LN L/S ratio less than 2 was known as malignant enlargement of LNs ( 28 ), so this relationship between CD4 + T cell and LN L/S ratios may be explained as the worse immune status resulting in a heavier infection. Another subset of blood lymphocytes, NK cells, was reported to be decreased in T.marneffei -infected HIV-negative patients ( 29 , 30 ). Apart from their decrease in HIV patients, lower NK cell counts and NK cell ratios were observed in patients who had retroperitoneal lymphadenectasis.…”

HIV-Negative Case of Talaromyces marneffei Pulmonary Infection with Liver Cirrhosis in China: A Case Report and Literature Review

“…STAT3-HIES is most likely a susceptibility factor for TM infections among HIV-negative patients and has a high case fatality rate. 30 Either hyperactivation or inactivation of STAT3 results in human disease. 31 STAT3 hyper-IgE syndrome comprises a heterogeneous group of immune-sharing manifestations, including increased infection susceptibility.…”