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Blastoid mantle cell lymphoma involving skin and orbit with hypercalcemia: A case report and literature review

机译:涉及皮肤和眼眶高钙血症的类胚层套细胞淋巴瘤1例并文献复习

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Mantel cell lymphoma is one of the small B-cell non-Hodgkin's lymphomas and usually involves lymph nodes, bone marrow, spleen, liver, gastrointestinal tract, and Waldeyer's ring, but rarely skin and orbit. A 53-year-old man presented skin nodules and plaques on the head, trunk, and lower extremities for half a month, and the left periorbital region swelled 4 days ago. Serum calcium and lactate dehydrogenase were increased to 3.12mmol/L (normal 2.03–2.65mmol/L) and 853U/L (normal 71–231U/L), respectively. Histopathologic examination of the skin nodule revealed tumor cells infiltrated nodular distribution in the dermis and subcutaneous tissue. Immunophenotyping of the abnormal lymphocytes indicated positive reactions for L26, CD79a, Bcl-2, Cyclin D1, and Ki-67 (>80%), but negative for CD5, CD21, CD23, CD38, CD3, CD10, UCHL-1, TdT, MPO, CD30, ALK, CD117, and CD34. Fluorescence in situ hybridization analysis with the CCND1/IGH probe revealed a fusion signal on the abnormal lymphocytes. The final diagnosis was a rare case of blastoid mantle cell lymphoma involving skin and orbit with hypercalcemia. Following the R-Hyper-CVAD treatment plan, the patient achieved a quick and excellent recovery on the 6th day. Unfortunately, the patient eventually died of pneumonia one month later.
机译:Mantel细胞淋巴瘤是一种小B细胞非霍奇金淋巴瘤,通常涉及淋巴结,骨髓,脾脏,肝脏,胃肠道和Waldeyer环,但很少涉及皮肤和眼眶。一名53岁的男子在头部,躯干和下肢出现了皮肤结节和斑块,持续了半个月,而四周前左眼眶周围肿胀。血清钙和乳酸脱氢酶分别升高至3.12mmol / L(正常2.03–2.65mmol / L)和853U / L(正常71–231U / L)。皮肤结节的组织病理学检查显示,肿瘤细胞浸润了真皮和皮下组织中的结节状分布。异常淋巴细胞的免疫分型表明L26,CD79a,Bcl-2,Cyclin D1和Ki-67阳性反应(> 80%),但CD5,CD21,CD23,CD38,CD3,CD10,UCHL-1,TdT阴性,MPO,CD30,ALK,CD117和CD34。用CCND1 / IGH探针进行的荧光原位杂交分析揭示了异常淋巴细胞上的融合信号。最终诊断为少见的囊样淋巴瘤淋巴瘤,涉及皮肤和眼眶高钙血症。遵循R-Hyper-CVAD治疗计划,患者在第6天获得了快速而卓越的康复。不幸的是,患者最终在一个月后死于肺炎。

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