中华皮肤科杂志 ›› 2009, Vol. 42 ›› Issue (4): 241-243.

• 论著 • 上一篇    下一篇

血管内大B细胞淋巴瘤一例

沈燕芸1,徐金华1,孔蕴毅1,罗燕1,陈连军1   

  1. 1. 复旦大学附属华山医院皮肤科
  • 收稿日期:2008-04-16 修回日期:2008-06-13 出版日期:2009-04-15 发布日期:2009-04-14
  • 通讯作者: 沈燕芸

Intravascular large B-cell lymphoma: a case report

  • Received:2008-04-16 Revised:2008-06-13 Online:2009-04-15 Published:2009-04-14

摘要:

患者,女,82岁。大腿、腹部皮疹2月余,伴间歇性发热。体检:浅表淋巴结未扪及肿大,肝脾肋下未触及。神经系统检查未见异常。双股及下腹部可见大小不一的暗红色斑块,质地坚实,有触痛,伴有明显的非凹陷性水肿,皮肤表面高低不平,局部呈橘皮样外观。血常规示三系减低。乳酸脱氢酶显著升高。第1次大腿部肿块穿刺和病理活检均未明确诊断。在不同医院诊断过慢性淋巴管炎、皮肤变应性血管炎和发热待查,经抗生素治疗无效,皮疹从大腿渐扩展至下腹部。第2次皮肤组织病理:皮下脂肪组织间隔血管腔内可见异形淋巴样细胞。免疫组化:异形淋巴样细胞抗淋巴细胞毒抗体(LCA)、CD20、CD79α、bcl-2阳性,bcl-6、CD10、CD3、CD45RO、CD30、EMA、AE1/3、CK均阴性,血管内皮细胞示CD34阳性。基于其组织形态学表现,结合免疫组化标记结果,符合血管内大B细胞淋巴瘤的诊断。患者2个月后死亡。

关键词: 弥漫性血管内大B细胞淋巴瘤;诊断

Abstract:

An 82-year-old Chinese woman presented with skin eruptions on the thigh and abdomen accompanied by intermittent fever of unknown origin for more than 2 months. No hepatosplenomegaly, lymphadenopathy or neurological abnormality was found with physical examination. There were irregular, tender, indurated, dark-erythematous plaques on bilateral thigh and lower abdomen, along with nonpitting edema and peau d′orange appearance. A significant decrease was observed in the count of white blood cells, red blood cells and platelets, but the serum level of lactate dehydrogenase was elevated. Tumor aspiration and the first pathology yielded no confirmed diagnosis, and the patient had ever been diagnosed with chronic lymphangitis, allergic cutaneous vasculitis and fever of unknown origin in other hospitals. Antibiotic therapy leaded to no improvement, and the lesions gradually spread from the thigh to lower abdomen. The second histopathology revealed the presence of atypical lymphoid cells with hyperchromatic nuclei and irregular morphology in the lumens of small blood vessels in subcutaneous tissue. Immunohistochemically, the atypical lymphoid cells were positive for lymphocytotoxic antibody (LCA), CD20, CD79α and bcl-2, but negative for bcl-6, CD10, CD3, CD45RO, CD30, EMA, AE1/3 or CK, and vascular endothelial cells were positive for CD34. The diagnosis of intravascular large B-cell lymphoma was made based on the histopathological features and immunohistochemical findings. The patient died in two months.