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朗格汉斯细胞组织细胞增多症:罕见的骨髓浸润——厄瓜多尔2例报告

Langerhans cell histiocytosis: unusual bone marrow infiltration-report of 2 cases in Ecuador.

作者信息

Santana Paulina, Arias-Intriago Marlon, Izquierdo-Condoy Juan S

机构信息

Department of Pathology, Medical Science Faculty, Universidad Central del Ecuador, Quito, Ecuador.

Department of Anatomic Pathology, Hospital de Especialidades Carlos Andrade Marín, Quito, Ecuador.

出版信息

Front Med (Lausanne). 2024 Jul 16;11:1433463. doi: 10.3389/fmed.2024.1433463. eCollection 2024.

Abstract

Langerhans cell histiocytosis (LCH) is a histiocytic neoplasm characterized by the abnormal proliferation of Langerhans cells. Bone marrow (BM) involvement is associated with high-risk disease and poor survival. Although BM involvement is particularly uncommon, no reported cases of LCH with BM infiltration have been documented in Latin America until now. The aim of this report is to highlight the clinical, hematological, and BM findings of two cases of LCH with BM infiltration, providing insights that may aid in detecting suspected patients. We present two cases of LCH with BM infiltration. One case involved a 23-month-old male patient, and the other a 16-month-old female patient. Common clinical findings in both cases included hepatosplenomegaly and fever. Hematological findings revealed anemia in both cases. The key diagnostic tool was the BM biopsy, which revealed histiocyte nests with characteristic morphology, CD1a-positive cells, increased eosinophils, and reactive paratrabecular lymphocytes. This report underscores the significance of clinical profiles in predicting BM infiltration in LCH. The presence of histiocyte nests displaying the characteristic morphology of Langerhans cells, accompanied by an elevation in eosinophils, indicates bone marrow involvement. Furthermore, the demonstration of CD1a-positive cells through immunohistochemistry serves as a crucial diagnostic tool.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种组织细胞肿瘤,其特征为朗格汉斯细胞异常增殖。骨髓受累与高危疾病及不良生存相关。尽管骨髓受累极为罕见,但截至目前拉丁美洲尚无LCH伴骨髓浸润的报道病例。本报告旨在突出两例LCH伴骨髓浸润患者的临床、血液学及骨髓检查结果,提供有助于检测疑似患者的见解。我们呈现两例LCH伴骨髓浸润病例。一例为23个月大的男性患者,另一例为16个月大的女性患者。两例患者的常见临床发现均包括肝脾肿大和发热。血液学检查结果显示两例均有贫血。关键诊断工具为骨髓活检,其显示具有特征性形态的组织细胞巢、CD1a阳性细胞、嗜酸性粒细胞增多以及反应性小梁旁淋巴细胞。本报告强调了临床特征在预测LCH骨髓浸润中的重要性。出现显示朗格汉斯细胞特征性形态的组织细胞巢,同时伴有嗜酸性粒细胞增多,提示骨髓受累。此外,通过免疫组织化学证明CD1a阳性细胞是关键的诊断工具。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73c4/11286394/d766c4821372/fmed-11-1433463-g001.jpg

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