患儿女,7 岁,因左下肢结节5 月余,全身淋巴结肿大2 个月,皮肤瘀斑3 d 入院。患儿于9个月前无明显诱因左下肢出现无痛性结节,3 个月后眼眶周围、结膜、口腔黏膜、会阴联合、腹股沟等多处出现皮肤出血、瘀斑,双眼呈"熊猫眼征";全身多处浅表淋巴结肿大,肝脾肿大。皮肤结节病理切片示淋巴造血细胞恶性肿瘤,结合临床表现、免疫组化和骨髓流式免疫分析结果(CD4、CD56、CD123 均阳性),诊断为母细胞性浆细胞样树突状肿瘤。确诊后采用高危ALL 方案对患儿进行化疗。目前随访3 个月,患儿瘀斑消退,肿大的淋巴结缩小。骨髓细胞形态学检查未见明显异常细胞。骨髓流式免疫分析示原始前体区细胞占1.5%,表达CD33、CD34、CD123、CD117。
Abstract
A 7-year-old girl was admitted to Xiangya Hospital due to systemic lymphadenectasis for 2 months and skin ecchymosis for 3 days. Nine months ago, the girl experienced painless nodules in the left lower extremity with no apparent causes. Three months later, dermatorrhagia and ecchymosis occurred in many regions such as the periocular areas, conjunctiva, oral mucosa, perineal area, and groin, with a "raccoon sign" in both eyes;superficial lymphadenectasis and hepatosplenomegaly were also observed in many regions. The pathological sections for the skin nodules showed malignant tumors in lymphohematopoietic cells, and in combination with clinical manifestations, immunohistochemistry, and positive results for CD4, CD56, and CD123 by bone marrow flow cytometry, the girl was diagnosed with blastic plasmacytoid dendritic cell neoplasm. Then high-risk ALL regimen was applied as the chemotherapy for this girl. At present, the girl has been followed up for 3 months;ecchymosis has disappeared, and the enlarged lymph nodes have shrunk. No abnormal cells have been found in bone marrow morphological examination, and bone marrow flow cytometry has shown that primitive precursor cells account for 1.5% and express CD33, CD34, CD123, and CD117.
关键词
母细胞性浆细胞样树突状肿瘤 /
皮肤瘀斑 /
儿童
Key words
Blastic plasmacytoid dendritic cell neoplasm /
Skin ecchymosis /
Child
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基金
国家自然科学基金资助项目(81570154)。
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