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ALK 阳性组织细胞增生症伴外周血组织细胞增多:一例报告。

ALK-Positive Histiocytosis with Peripheral Blood Histiocytes: A Case Report.

机构信息

Princess Alexandra Hospital, Cancer Services, Brisbane, Queensland, Australia,

Pathology Queensland, Haematology Department, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia.

出版信息

Acta Haematol. 2021;144(2):218-221. doi: 10.1159/000508524. Epub 2020 Jul 28.

Abstract

Histiocytoses are a diverse group of rare, clinically heterogeneous disorders characterised by tissue infiltration of histiocytes, which may result in organ dysfunction and failure. Over 100 different subtypes of histiocytoses have been recognised, including rare cases of ALK-positive histiocytosis. We report a case of histiocytosis in a neonate who presented with refractory thrombocytopenia, anaemia, and intermittent neutropenia. Histiocytes were present in both peripheral blood smears and bone marrow; ALK positivity was demonstrated by immunohistochemistry. Given the scarce reports of this condition, the variable organ involvement, and the different approaches to management in the cases described, we seek to expand the literature by providing a report of our patient whose condition improved without chemotherapy. The presence of histiocytes in peripheral blood smears of patients with this condition has not previously been reported, and it underscores the importance of routine careful evaluation of blood smears.

摘要

组织细胞增生症是一组罕见的、临床表现多样的疾病,其特征为组织中存在组织细胞浸润,可能导致器官功能障碍和衰竭。已经识别出超过 100 种不同类型的组织细胞增生症,包括罕见的 ALK 阳性组织细胞增生症。我们报告了一例新生儿组织细胞增生症病例,该患儿表现为难治性血小板减少症、贫血和间歇性中性粒细胞减少症。外周血涂片和骨髓中均存在组织细胞;免疫组织化学显示 ALK 阳性。鉴于这种情况的报道很少,受累器官的变化,以及描述的病例中管理方法的不同,我们通过报告我们的患者的情况来扩展文献,该患者在没有化疗的情况下病情得到改善。以前没有报道过这种情况下外周血涂片存在组织细胞,这强调了常规仔细评估血涂片的重要性。

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