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一例形态学具有欺骗性的儿童B淋巴细胞白血病的罕见病例,伴有颗粒状“泡状”原始细胞及弥漫性大B细胞淋巴瘤样活检结果。

A Curious Case of Morphologically Deceptive Pediatric B-Lymphoblastic Leukemia with Granular "Blebbed" Blasts and DLBCL-Like Biopsy Findings.

作者信息

Sharma Sunita, Gaur Kavita, Perme Tatton, Singh Amrita

机构信息

Department of Pathology, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, Shaheed Bhagat Singh Marg, New Delhi, Delhi, India.

出版信息

Fetal Pediatr Pathol. 2023 Feb;42(1):98-102. doi: 10.1080/15513815.2022.2050325. Epub 2022 Mar 14.

Abstract

B-cell acute lymphoblastic leukemia is known for heterogeneous blast morphology. Cytoplasmic granules and blebs have both been described, but not together, in lymphoblasts. : A 7-year-old boy presented with fever, abdominal distension for 3 weeks, with hepatosplenomegaly. Investigations revealed pancytopenia, peripheral smear showing 21% blasts of varying size, displaying cytoplasmic blebbing and granulation. Marrow aspirate was hemodilute, 10% cells showed dim to moderate expression of CD45, CD10, CD19, HLA-DR confirming B-ALL. Megakaryocytic markers (CD41, CD61) were negative. Marrow biopsy showed a focus of large atypical cells displaying increased nuclear-cytoplasmic ratio, vesicular nuclear chromatin and macronucleoli, reminiscent of diffuse large B-cell lymphoma, cells strongly expressing PAX5, CD19, CD20, and bcl-2.The child was put on standard induction therapy. No blasts were detected in subsequent peripheral smears. To the best of our knowledge, this is the first case of pediatric B-ALL displaying granular "blebbed" blasts.

摘要

B 细胞急性淋巴细胞白血病以原始细胞形态异质性为特征。在淋巴母细胞中,胞质颗粒和泡状突起均有描述,但未同时出现过。一名 7 岁男孩出现发热、腹胀 3 周,伴有肝脾肿大。检查发现全血细胞减少,外周血涂片显示 21%大小各异的原始细胞,可见胞质泡状突起和颗粒形成。骨髓穿刺液呈血液稀释状,10%的细胞显示 CD45、CD10、CD19、HLA - DR 呈弱阳性至中度表达,确诊为 B 细胞急性淋巴细胞白血病。巨核细胞标志物(CD41、CD61)为阴性。骨髓活检显示有一大片非典型细胞灶,核质比增加,核染色质呈泡状,有大核仁,类似弥漫性大 B 细胞淋巴瘤,细胞强烈表达 PAX5、CD19、CD20 和 bcl - 2。该患儿接受了标准诱导治疗。后续外周血涂片中未检测到原始细胞。据我们所知,这是首例表现为颗粒状“泡状”原始细胞的儿童 B 细胞急性淋巴细胞白血病病例。

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