每日一题_2023.11.30

A 53-year-old woman presented with a 3-month history of worsening vascular skin lesions and a 1-month history of fever. On physical examination, diffuse telangiectasis, hyperpigmented plaques, and several ulcerated nodules (arrows) were observed on the skin across the chest and abdomen (left) and the legs. No palpable lymphadenopathy or hepatosplenomegaly was noted. Laboratory studies were notable for a lactate dehydrogenase level of 35664 U per liter (reference range, 120 to 250). A deep skin biopsy specimen from the abdomen showed intravascular aggregation of round, atypical lymphocytes (right, hematoxylin and eosin staining). Subsequent immunohistochemical staining was positive for CD20, PAX-5, and MUM-1 in the neoplastic cells. Which of the following is the most likely diagnosis?

患者女，53岁，因血管性皮损加重3个月、发热1个月就诊。体格检查时，在胸腹部(左)和腿部的皮肤上观察到弥漫性毛细血管扩张、色素沉着斑块和几个溃疡性结节(箭头所示)。未见明显淋巴结肿大或肝脾肿大。值得注意的是，实验室检查显示乳酸脱氢酶水平为35664 U / l(参考范围，120 ~ 250 U / l)。腹部深层皮肤活检标本显示血管内圆形异型淋巴细胞聚集(右，苏木精-伊红染色)。免疫组织化学染色示肿瘤细胞CD20、PAX-5、MUM-1阳性。下列哪一项是最可能的诊断?

A. Chronic lymphocytic leukemia 慢性淋巴细胞白血病

B. Cutaneous small vessel vasculitis 皮肤小血管炎

C. Idiopathic multicentric Castleman 特发性多中心卡斯尔曼病

D. Intralymphatic histiocytosis 淋巴管内组织细胞增生症

E. Intravascular lymphoma 血管内淋巴瘤

答案解析：

诊断为血管内大B细胞淋巴瘤。血管内大B细胞淋巴瘤是一种罕见的、常具有侵袭性的结外淋巴瘤，其特征是小血管腔内的恶性侵犯。由于其非特异性症状和体征(如发热、疲劳和无淋巴结肿大)和异质性表现，该病的诊断具有挑战性。

正确答案：E