A rare case report of idiopathic CD4 lymphocytopenia in an Indian male with nasal Rhizopus fungal polyp with drug-induced acute kidney injury

Rajesh Kumar, Yasir Rizvi, C. Kumar, Pal Singh Athwal
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Abstract

Idiopathic CD4 lymphocytopenia (ICL) is a rare entity of immune deficit of CD4+ T cells (below 300/mm3) which was first defined in 1992, unrelated to human immunodeficiency virus (HIV) syndrome without predefined clinical presentation and natural history. The etiology, pathogenesis, and management of ICL remains poorly understood and inadequately defined. The clinical presentation can range from serious opportunistic infections to incidentally diagnosed asymptomatic individuals. Cryptococcal and nontuberculous mycobacterial infections and progressive multifocal leukoencephalopathy are the most significant presenting infections, although the spectrum of opportunistic diseases can be similar to that in patients with lymphopenia and HIV infection. The prognosis is influenced by the accompanying opportunistic infections and response to the treatment. This rare disease invites uncountable opportunistic infections sometimes leading to lethal outcome. We report a case of ICL in an immunocompetent 18-year-old male with a history of intermittent epistaxis, fungal nasal polyp diagnosed to have Rhizopus with drug-induced acute kidney injury during the course of treatment, a rarest diagnosis as underlying main disease entity. To the best of our knowledge, ICL presenting with opportunistic infection of Rhizopus is the first case to be reported here from a tertiary care center of India, a developing country where a major population lives with poor hygiene and low socioeconomic status.
一个罕见的病例报告特发性CD4淋巴细胞减少症在印度男性鼻根霉真菌息肉与药物引起的急性肾损伤
特发性CD4淋巴细胞减少症(ICL)是一种罕见的CD4+ T细胞免疫缺陷(低于300/mm3),首次定义于1992年,与人类免疫缺陷病毒(HIV)综合征无关,没有预定义的临床表现和自然病史。ICL的病因、发病机制和治疗仍然知之甚少,定义不充分。临床表现可以从严重的机会性感染到偶然诊断的无症状个体。隐球菌和非结核分枝杆菌感染和进行性多灶性脑白质病是最显著的感染,尽管机会性疾病的频谱可能与淋巴细胞减少症和HIV感染的患者相似。预后受伴随的机会性感染和对治疗的反应的影响。这种罕见的疾病引起无数的机会性感染,有时导致致命的后果。我们报告一例免疫功能正常的18岁男性ICL,有间歇性鼻出血史,真菌鼻息肉在治疗过程中诊断为根霉菌伴药物性急性肾损伤,这是一种罕见的诊断,是潜在的主要疾病实体。据我们所知,以根霉机会性感染为表现的ICL是印度三级保健中心报告的首例病例,印度是一个主要人口生活卫生条件差、社会经济地位低的发展中国家。
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