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诊断范可尼贫血:来自印度农村地区的罕见病例报告。

Diagnosing Fanconi Anemia: A Rare Case Report From Rural India.

作者信息

Malik Aashita

机构信息

Pediatrics, Datta Meghe Institute of Higher Education and Research, Wardha, IND.

出版信息

Cureus. 2024 Jun 28;16(6):e63381. doi: 10.7759/cureus.63381. eCollection 2024 Jun.

Abstract

Fanconi anemia is a rare but most prevalent form of inherited aplastic anemia, predominantly transmitted in an autosomal recessive manner, except for one X-linked variant. It arises from mutations in the genes across 16 different complementation groups that are crucial for DNA stability. It is marked by a wide range of congenital malformations, progressive pancytopenia, and an increased risk of both hematological malignancies and solid tumors. The congenital abnormalities associated with it can affect various organ systems, including the skeletal system, with significant variability among patients. One similar case has been reported here, which had the typical clinical features of FA. Due to varied phenotypic presentation, diagnosing FA can be challenging. A Chromosomal Breakage Study using mitomycin C (MMC) or diepoxybutane (DEB) is a distinctive cellular marker that aids in the diagnosis.

摘要

范可尼贫血是一种罕见但最常见的遗传性再生障碍性贫血,除一种X连锁变异型外,主要以常染色体隐性方式遗传。它源于16个不同互补组中的基因突变,这些基因对DNA稳定性至关重要。其特征是广泛的先天性畸形、进行性全血细胞减少,以及血液系统恶性肿瘤和实体瘤的风险增加。与之相关的先天性异常可影响包括骨骼系统在内的各种器官系统,患者之间存在显著差异。本文报告了一例具有典型范可尼贫血临床特征的类似病例。由于表型表现多样,诊断范可尼贫血具有挑战性。使用丝裂霉素C(MMC)或环氧丁烷(DEB)进行染色体断裂研究是一种有助于诊断的独特细胞标志物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bab4/11283913/f3966117c0f8/cureus-0016-00000063381-i01.jpg

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