滤泡性淋巴瘤转分化为组织细胞肉瘤1例

EJHaem Pub Date : 2025-02-08 DOI:10.1002/jha2.1107
Dina Osman, Reem Ahmed
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引用次数: 0

摘要

患者55岁,既往健康,有几个月的全身性淋巴结病和盗汗史。PET CT扫描显示横膈膜上下淋巴结肿大,左侧髂外淋巴结最大SUV为17.4。淋巴结活检显示局灶性浸润,大肿瘤细胞呈实性弥漫性浸润,细胞质丰富,细胞核多形性(上图,左侧H&;E ×20),有丝分裂率高,Ki-67指数高(图1)。这些细胞弥漫性CD68(上图,中间)、CD163(上图,右侧)、CD4(下图,左侧)、S-100和溶菌酶阳性,OCT2和cyclin D1阳性。这些特征强烈提示高级别组织细胞肉瘤(HS)。低级别滤泡性淋巴瘤(FL)的小局灶性淋巴浸润在未受HS影响的区域,CD10+, CD20+和强BCL2+(下图,中),Ki-67指数低。分子分析显示HS和FL浸润共享t(14;18),提示FL向HS的转分化。骨髓(BM)形态学显示HS的大量浸润(右下,×100)。流式细胞术检测骨髓组织中有少量kappa+ CD10+ FL浸润。患者接受CHOP方案治疗,但不幸的是,由于严重的细胞因子释放综合征,肿瘤溶解和多器官衰竭而去世。HS是一种非常罕见且极具侵袭性的疾病,如果在早期诊断,预后要好一些。正如本病例所证明的那样,它可以从头开始或通过b细胞恶性肿瘤[2]的反式分化产生。迪娜·奥斯曼和里姆·艾哈迈德撰写了手稿。迪娜·奥斯曼拍摄了这些照片。作者声明无利益冲突。作者没有什么可报告的,已获得已故亲属的口头同意发表。作者已确认该提交不需要临床试验注册
本文章由计算机程序翻译,如有差异,请以英文原文为准。

A Rare Case of Trans-differentiation of Follicular Lymphoma into Histiocytic Sarcoma

A Rare Case of Trans-differentiation of Follicular Lymphoma into Histiocytic Sarcoma

A 55-year-old previously healthy gentleman presented with a few months’ history of generalised lymphadenopathy and drenching night sweats. PET CT scan showed enlarged lymph nodes above and below the diaphragm with an SUV max of 17.4 at the left external iliac lymph node.

The lymph node biopsy showed focal effacement by a solid appearing diffuse infiltrate of large neoplastic cells with abundant cytoplasm and pleomorphic nuclei (upper panel, left H&E ×20) with high mitotic rate, reflected by high Ki-67 index (Figure 1). These cells were diffusely positive for CD68 (upper panel, middle), CD163 (upper panel, right), CD4 (lower panel, left), S-100 and lysozyme, with variable OCT2 and cyclin D1 positivity. These features were strongly suggestive of high-grade histiocytic sarcoma (HS). A small focal lymphoid infiltrate of low-grade follicular lymphoma (FL) was noted in areas unaffected by the HS that was CD10+, CD20+ and strongly BCL2+ (lower panel, middle) with low Ki-67 index. Molecular analysis revealed that the HS and FL infiltrates shared t(14;18), suggesting trans-differentiation of FL into HS.

The bone marrow (BM) morphology showed heavy infiltration by the HS (lower panel, right ×100). A small kappa+ CD10+ FL infiltrate was detected by flow cytometry analysis of the BM.

The patient was treated with CHOP regimen but, unfortunately, passed away due to severe cytokine release syndrome, tumour lysis and multi-organ failure.

HS is a very rare and extremely aggressive disorder with somewhat better prognosis if diagnosed at an early stage [1]. It can arise de novo or via trans-differentiation from B-cell malignancies [2], as was demonstrated by this case.

Dina Osman and Reem Ahmed wrote the manuscript. Dina Osman took the photographs.

The authors declare no conflicts of interest.

The authors have nothing to report

Verbal consent for publication was obtained from the Late's next of kin.

The authors have confirmed clinical trial registration is not needed for this submission

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