Adult-Onset Immunodeficiency in Thailand and Taiwan

Abstract

Background Autoantibodies against interferon-γ are associated with severe disseminated opportunistic infection, but their importance and prevalence are unknown. Methods We enrolled 203 persons from sites in Thailand and Taiwan in five groups: 52 patients with disseminated, rapidly or slowly growing, nontuberculous mycobacterial infection (group 1); 45 patients with another opportunistic infection, with or without nontuberculous mycobacterial infection (group 2); 9 patients with disseminated tuberculosis (group 3); 49 patients with pulmonary tuberculosis (group 4); and 48 healthy controls (group 5). Clinical histories were recorded, and blood specimens were obtained. Results Patients in groups 1 and 2 had CD4+ T-lymphocyte counts that were similar to those in patients in groups 4 and 5, and they were not infected with the human immunodeficiency virus (HIV). Washed cells obtained from patients in groups 1 and 2 had intact cytokine production and a response to cytokine stimulation. In contrast, plasma obtained from these patients inhibited the activity of interferon-γ in normal cells. High-titer anti–interferon-γ autoantibodies were detected in 81% of patients in group 1, 96% of patients in group 2, 11% of patients in group 3, 2% of patients in group 4, and 2% of controls (group 5). Forty other anti-cytokine autoantibodies were assayed. One patient with cryptococcal meningitis had autoantibodies only against granulocyte–macrophage colony-stimulating factor. No other anti-cytokine autoantibodies or genetic defects correlated with infections. There was no familial clustering. Conclusions Neutralizing anti–interferon-γ autoantibodies were detected in 88% of Asian adults with multiple opportunistic infections and were associated with an adult-onset immunodeficiency akin to that of advanced HIV infection.

Two Unusual Cases of Human Immunodeficiency Virus–Negative Patients with Talaromyces marneffei Infection

“…Systemic intravenous amphotericin B and itraconazole are the main effective treatment options. [1][2][3][4][5] However, in case 1, after 10 months of regular and adequate antifungal treatments, but without treatment of LCH, the patient ultimately died. This outcome suggested that it is essential to treat not only the secondary infection, but also the underlying disease such as connective tissue diseases, etc.…”

“…[2][3][4][5]7 However, T. marneffei complicated by LCH has not been reported. LCH is a proliferation of Langerhans cells mixed with inflammatory cells, particularly eosinophils, characterized by the expression of CD1a and S100 protein, and the presence of Birbeck granules on ultrastructural examination.…”

“…2 Browne and others reported that antiinterferon-γ autoantibodies were correlated with disseminated opportunistic infections. 3,7 Thus, humoral immunologic defect involving anti-interferon-γ autoantibodies or heterozygous missense genetic mutations may provide plausible explanations.…”

“…However, it is increasingly observed in HIV-negative subjects without any obvious risk factors or immunocompromised condition. [1][2][3][4][5] The infection can disseminate hematogenously to the bone, bone marrow, joints, lymph nodes, pericardium, liver, trachea, spleen, etc. [6][7][8] Herein, we report the first case of T. marneffei complicated by Langerhans cell histiocytosis (LCH) and a case of pleural nodules and pleural effusion caused by T. marneffei using thoracoscopic pleural biopsy for differential diagnosis.…”

Non, la maladie de Chagas n’est pas le nouveau sida des Amériques !

“…Au moment même où un autre syndrome d'immunodé-pression, proche du sida dans sa traduction clinique, mais différent par son mécanisme physiopathologique comme dans son épidémiologie, est en train d'être individualisé et caractérisé en Asie [5], l'heure n'est pas aux amalgames et aux comparaisons contestables et douteuses qui ne peuvent générer, dans le grand public, qu'inquiétudes et confusions.…”