血管内大B细胞淋巴瘤

报告1例血管内大B细胞淋巴瘤。患者女,63岁。双下肢多发红斑、皮下结节半年余。组织病理显示真皮及皮下脂肪小到中等大血管内可见大量的形态大小较为一致的肿瘤细胞,瘤细胞体积较大。免疫组化结果显示血管内肿瘤细胞CD20、CD79a、B细胞淋巴瘤-2基因(BCL-2)、BCL-6、多发性骨髓瘤癌基因1(MUM1)阳性,CD2、CD3、CD4、CD8、CD30、CD56、穿孔素、颗粒酶、T细胞内抗原(TIA)阴性,Ki-67增殖指数100%,EBV原位杂交阴性。诊断为血管内大B细胞淋巴瘤。     

血管内大B细胞淋巴瘤一例

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。     

血管内大B细胞淋巴瘤4例临床病理分析

目的:分析4例血管内大B细胞淋巴瘤（IVLBCL）的临床病理特点。方法:回顾北京协和医院皮肤科2020年1-11月行皮肤组织病理确诊的4例IVLBCL患者的临床及病理资料。结果:4例患者年龄57~76岁,男2例,女2例。4例出现神经系统症状,3例发热,3例运动耐量下降、憋气,3例躯体凹陷性水肿。例1背部出现0.2 cm...     

血管内大B细胞淋巴瘤一例

患者女,60岁。反复双下肢结节、溃疡、坏死1年,加重6个月。患者1年前,出现左下肢结节、溃疡、坏死,皮肤瘢痕、硬化,皮损沿血管走行,伴有间断发热。2012年8月16日皮损组织病理检查提示皮肤血管炎,给予甲泼尼龙80 mg/d治疗15 d,皮损好转,但糖皮质激素减量后病情出现反复。既往有慢性肾功能不全病史7年,贫血病史2年及甲状腺结节病史1年……     

原发性皮肤弥漫大B细胞淋巴瘤1例

报告1例原发性皮肤弥漫大B细胞淋巴瘤。患者男,72岁,因四肢、躯干红斑、结节、肿块2年,加重伴溃烂3月,于2009年3月9日就诊。皮损组织病理检查报告见皮肤真皮内大量淋巴样细胞弥漫浸润,未侵及表皮,免疫组化染色结果示瘤细胞L26（＋）,Bcl-6（＋）,Mum-1（＋）,CD10弱（＋）,K i-67（＋）约90%。诊断为原发性皮肤弥漫性大B细胞淋巴瘤。     

皮肤血管内T细胞淋巴瘤：l例报道及文献复习

对l例血管内淋巴瘤进行免疫表型分析及原位杂交检测与EB病毒的关系,并复习相关文献。患者男,32岁,发热2个月,左大腿斑块1月。全身体检及辅助检查血小板90×109,血沉24fml／h,C反应蛋白28．9mg／L,LDH448U／L。病理学检查肿瘤细胞位于真皮层的小m管内,瘤细胞体积较大、核仁明显、胞浆嗜碱性,核分裂相可见。瘤细胞免疫表型CD3,CD45（LCA）呈阳性表达。,原位杂交显示E2BERs阴性。患者经CHOP联合化疗一个疗程后获得缓解。血管内T细胞淋巴瘤是一种罕见淋巴瘤,此瘤的诊断必须依赖病理学及免疫组织化学检查,住不明原因发热伴发皮肤斑块时,应及时活检,积极联合化疗。     

皮肤血管内T细胞淋巴瘤:1例报道及文献复习

对1例血管内淋巴瘤进行免疫表型分析及原位杂交检测与 EB病毒的关系,并复习相关文献患者男,32岁,发热2个月,左大腿斑块1月.全身体检及辅助检查血小板90× 109,血沉24mm/h,C反应蛋白28.9 mg/L,LDH 448 U/L.病理学检查肿瘤细胞位于真皮层的小血管内,瘤细胞体积较大、核仁明显、胞浆嗜碱性,核分裂相可见.瘤细胞免疫表型CD3,CD45( LCA)呈阳性表达.原位杂交显示E2BERs阴性.患者经CHOP联合化疗一个疗程后获得缓解.血管内T细胞淋巴瘤是一种罕见淋巴瘤,此瘤的诊断必须依赖病理学及免疫组织化学检查,在不明原因发热伴发皮肤斑块时,应及时活检,积极联合化疗.     

皮肤血管内T细胞淋巴瘤一例

患者女,64岁,6个月前全身出现多发淡红色斑丘疹,质硬,直径1～3 cm,以左侧颜面部及双下肢明显.曾就诊于某医院,诊断为血管炎.1个月前出现头痛、头晕伴步态不稳,同时伴双手及左足麻木、视物模糊,行头颅CT检查提示"右侧额辐射区腔隙性脑梗塞",1个月来步态不稳逐渐加重,再次就诊于当地医院,行头颅MRI检查提示"双侧丘脑、右侧基底节、右侧额叶及右侧小脑半球梗塞,左侧蝶窦旁异常信号灶".患者为进一步诊治于2010年1月至本院治疗,门诊拟诊脑梗死收住入院.皮疹予复方倍他米松注射液、冰黄肤乐软膏等治疗后稍见好转.     

Early diagnosis of recurrent diffuse large B-cell lymphoma showing intravascular lymphoma by random skin biopsy

A 66-year-old man was admitted to our hospital presenting 2 weeks' history of fever of unknown origin with elevated levels of lactate dehydrogenase and C-reactive protein. Six years before this episode, he had developed diffuse large B-cell lymphoma, which had been successfully treated with chemoradiation. While recurrence of diffuse large B-cell lymphoma was suspected, there was neither lymphadenopathy nor tumor formation by the imaging study. Random biopsy from normal-appearing abdominal skin showed extensive infiltration of CD20(+), CD79a(+), CD3(-) atypical lymphoid cells in the lumen of vessels in subcutaneous tissues. These findings led us to the diagnosis of intravascular B-cell lymphoma. Following rituximab plus cyclophosphamide, adriamycin, vincristine and prednisolone therapy, high fever subsided, and lactate dehydrogenase and C-reactive protein levels returned to the normal range. In conclusion, random skin biopsy is useful for the early diagnosis of intravascular B-cell lymphoma.     

Intravascular large B-cell lymphoma of the cutaneous variant in Korea

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma. It is characterized by proliferation of malignant lymphoid cells within the small vessels of various organs. The skin and central nervous system are commonly involved although it occurs in other organs such as the kidneys, adrenals, lung and liver. In Western patients, there is a cutaneous variant of IVLBCL, which involves only the skin. However, the Asian variant, which rarely involves the skin, is associated with hemophagocytosis. It was reported in the majority of Japanese patients. Here, we report a case of the cutaneous variant of IVLBCL in a Korean individual.     

Concurrent mycosis fungoides and intravascular large B-cell lymphoma in a single patient

Mycosis fungoides (MF) is an indolent, uncommon, non-Hodgkin T-cell lymphoma of the skin. It classically presents with patches, plaques, and tumors and may rarely show spread to internal organs or bone marrow. Up to 7.5% of MF patients may be diagnosed with a second malignancy. Intravascular large B-cell lymphoma (IVLBCL) is an exceedingly rare non-Hodgkin B-cell lymphoma characterized by predominant growth of large neoplastic cells in the lumina of blood vessels. This case presents with an unusual confluence of two rare diagnoses, MF and IVLBCL, made more remarkable by the presence of both diagnoses on a single skin biopsy sample.     

Primary subcutaneous B-cell lymphoma: case report and literature review

Primary cutaneous B-cell lymphomas are defined as malignant B-cell proliferations presenting with cutaneous involvement alone and no evidence of extracutaneous manifestations when complete staging has been performed. It has been shown that the infiltrate in some cases could involve the underlying subcutaneous tissues, but primary localization in this compartment has been rarely reported. We describe here the case of a 53-year-old woman who noticed a nodular lesion on the left shoulder that rapidly enlarged in a few months. The histological and immunophenotypical features were compatible with a subcutaneous B-cell lymphoma. The tumoural mass was confined predominantly to the subcutaneous compartment, as confirmed by computed tomography. No other tumour localizations were found. Thus, primary B-cell lymphoma of the subcutis was diagnosed. We report a review of the literature indicating that B-cell lymphomas that are primarily localized to the subcutaneous tissues represent a very rare modality of presentation with a biological behaviour different from conventional cutaneous B-cell lymphoma.     

弥漫性大B细胞淋巴瘤皮肤受累一例

患者,女,76岁,全身皮下多发结节伴右下肢皮肤溃烂2个月。皮损病理活检示（腹壁肿物）：非霍奇金淋巴结,B细胞型,免疫表型符合DLBCL,生发中心来源。免疫组化：CD20弥漫连片（+）,CD3散在（+）,CD10（+）,CD21显示FDC网消失,Ki67（80%）。胸腹部增强CT示胃窦十二指肠占位性病变,胃镜检查取胃窦部病变活检示：（胃窦小弯、大弯）非霍奇金B细胞淋巴瘤,考虑DLBCL或其他高侵袭性类型;免疫组化：粘膜腺体间弥漫的淋巴样细胞侵润性生长,CD20连片阳性,Ki67（90%）。我院血液科诊断为非霍奇金淋巴瘤（弥漫大B细胞型）IV期IPI,评分5分 继发皮肤受累。患者家属拒绝治疗,患者出院1周后死亡。     

A case report and literature review of cutaneous intravascular large B-cell lymphoma presenting clinically as panniculitis: a difficult diagnosis,but a good prognosis

Intravascular large B-cell lymphoma is a rare, non-mass-forming, extranodal large B-cell lymphoma subtype characterized by the presence of tumor cells in the lumens of vessels. It is divided into two major types: classical and Asian. Patients presenting only with skin involvement are mostly female, at a younger age than classical intravascular large B-cell lymphoma patients, and have a better prognosis. Since the diagnosis of cases with isolated skin involvement is difficult, keeping this entity in mind, performing a careful microscopic examination, and applying new, effective treatment regimens will make it possible to achieve better clinical outcomes in these cases.     

Angiotropic B-cell lymphoma with telangiectasia, accompanied by panniculitic formation

A 67-year-old female presented with diffuse edema, generalized telangiectasia, and indurated skin plaques. Histopathological findings of the skin lesion included CD45+/CD79+/CD20+ large lymphoma cells that filled the vascular lumina and infiltrated the subcutaneous tissues with panniculitic formations. Three cycles of a CHOP (cyclophosphamide, adriamycin, vincristine, and prednisolone) chemotherapy regimen resolved the skin manifestations. After five cycles of CHOP, however, she developed dementia-like symptoms. Three cycles of additional intrathecal chemotherapy (methotrexate, cytarabine and prednisolone) did not improve the neurological symptoms.