一组肿瘤浆细胞。

EJHaem Pub Date : 2024-10-24 DOI:10.1002/jha2.1010
Radu Chiriac, Sophie Gazzo
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引用次数: 0

摘要

70岁男性,5年前列腺癌病史,目前处于完全缓解期,表现为全血细胞减少和下肋骨疼痛。未见异常循环细胞。正电子发射断层扫描-计算机断层扫描(PET-CT)显示右侧最后两根肋骨骨折,中轴骨骼中F-18氟脱氧葡萄糖(18F-FDG)摄取异常。骨髓抽吸显示异常浆细胞的存在,从小型到中型单核形式到双核、三核、四核甚至五核形式,类似于间变性浆细胞(图1,A组,mayg - grunwald Giemsa染色,x100物镜)。流式细胞术证实了CD38+/CD138+质细胞的lambda受限群体,CD45、CD19、CD56和CD117缺失。血检显示免疫球蛋白G副蛋白。免疫组化排除转移性腺癌。间期荧光原位杂交(FISH)分析显示,95%的细胞(包括二倍体和四倍体克隆)中存在一个涉及TP53的del(17)(p13.1)(图1,B组,TP53/NF1缺失探针),以及一个IgH(14q32)异常(图1,C组,FISH信号减弱,IgH +分离探针)。检测到No (4;14)(p16;q32) FGFR3/IGH易位。这些发现与多发性骨髓瘤表现为间变性特征一致。患者不适合移植,开始使用硼替佐米、环磷酰胺和地塞米松。完成4个周期后,部分反应非常好,患者因呼吸窘迫加重和4级中性粒细胞减少而入院。胸部CT扫描显示两肺散在模糊的磨玻璃影,双肺下叶有较密集的异常。没有其他肺炎的体征或症状。患者接受大剂量甲基强的松龙和无创正压通气治疗硼替佐米引起的肺炎,但未见改善。不幸的是,入院两周后,患者因呼吸机相关性肺炎去世。本病例强调,虽然大多数浆细胞肿瘤可通过经典形态学识别,但一些具有不寻常特征的变异可模仿间变性癌或淋巴瘤。其多形性多核形态可与多核癌甚至发育异常巨核细胞相似,因为其核多叶,核分布异常,使其与转移性癌、骨髓增生异常综合征或浆母细胞淋巴瘤的分化复杂化。此外,患者既往的腺癌病史进一步挑战了本病例的诊断过程。Radu Chiriac和Sophie Gazzo撰写了手稿,进行了细胞学研究,并进行了细胞遗传学研究。所有作者都对定稿做出了贡献。作者声明无利益冲突。作者没有得到这项工作的特别资助。本文尊重CHU Lyon对待人类研究参与者的伦理政策。没有使用患者识别数据。作者没有获得患者的书面知情同意,但患者不反对将其数据用于研究目的(根据CHU Lyon的伦理政策要求)。作者已确认该提交不需要临床试验注册。
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A panoply of neoplastic plasma cells

A 70-year-old man with a 5-year history of prostate adenocarcinoma, currently in complete remission, presented with pancytopenia and pain in his lower ribs. No abnormal circulating cells were observed.

The positron emission tomography-computed tomography (PET-CT) scan revealed fractures of the two last right ribs and abnormal F-18 fluorodeoxyglucose (18F-FDG) uptake in the axial skeleton. A bone marrow aspirate revealed the presence of abnormal plasma cells, varying from small to medium-sized mononucleated forms to bi-, tri-, quadri-, and even pentanucleated forms, resembling anaplastic plasma cells (Figure 1, Panel A, May-Grunwald Giemsa stain, x100 objective). Flow cytometry confirmed a lambda-restricted population of CD38+/CD138+ plasma cells with loss of CD45, CD19, CD56, and CD117. Blood work showed immunoglobulin G lambda paraprotein. Metastatic adenocarcinoma was excluded by immunohistochemistry. Interphase fluorescence in situ hybridization (FISH) analysis revealed a del(17)(p13.1) involving TP53 (Figure 1, Panel B, TP53/NF1 deletion probe) in 95% of cells, including both diploid and tetraploid clones, and an IgH(14q32) abnormality (Figure 1, Panel C, diminished FISH signals, IGH+ break-apart probe). No t(4;14)(p16;q32) FGFR3/IGH translocation was detected. These findings were consistent with multiple myeloma displaying anaplastic features.

The patient, ineligible for a transplant, was started on bortezomib, cyclophosphamide, and dexamethasone. After completing four cycles with a very good partial response, he was admitted to the hospital with worsening respiratory distress and grade 4 neutropenia. A chest CT scan revealed hazy ground-glass opacities scattered throughout both lungs, with denser abnormalities in the lower lobes bilaterally. There were no other signs or symptoms suggestive of pneumonia. The patient was treated with high-dose methylprednisolone and noninvasive positive pressure ventilation for bortezomib-induced pneumonitis but showed no improvement. Unfortunately, two weeks after admission, the patient passed away due to ventilator-associated pneumonia.

This case highlights that while most plasma cell neoplasms are recognizable by classic morphology, some variants with unusual features can mimic anaplastic carcinoma or lymphoma. Their pleomorphic multinucleated morphology can resemble that of multinucleated carcinomas or even dysplastic megakaryocytes due to their multilobed nuclei and, abnormal nuclear distribution, complicating the differentiation from metastatic carcinoma, myelodysplastic syndrome, or plasmablastic lymphoma [1]. Additionally, the patient's prior history of adenocarcinoma further challenged the diagnostic process in this case.

Radu Chiriac and Sophie Gazzo wrote the manuscript, conducted the cytological studies, and performed the cytogenetic studies. All authors contributed to the final manuscript.

The authors declare no conflicts of interest.

The authors received no specific funding for this work.

This manuscript respects the ethics policy of CHU Lyon for the treatment of human research participants.

No patient-identifying data were used. The authors did not obtain written informed consent from the patient but the patient did not object to his data being used for research purposes (as required by the ethics policy of CHU Lyon).

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

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