血管内大B细胞淋巴瘤2例分析

正血管内大B细胞淋巴瘤(intravascular large B cell lymphoma,IVLBCL)是弥漫性大B细胞淋巴瘤的一个罕见的亚型,本病发病率低,一般在尸检中发现,国内仅报告十余例~([1])。血管内大B细胞淋巴瘤临床表现多样,没有明显特异性,极易导致漏诊或误诊,本病具有高度侵袭性,对化疗不敏感,延误诊断对患者危害极大~([2])。组织病理检查和基因分析对IVLBCL诊断具有     

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。     

原发性皮肤弥漫性大B细胞淋巴瘤并发嗜酸性粒细胞增多

报告1例原发性皮肤弥漫性大B细胞淋巴瘤并发嗜酸性粒细胞增多.患者男,71岁.伞身散在红斑、丘疹及小结节,外周血嗜酸性粒细胞计数增多.第1次行皮损组织病理检查示嗜酸性粒细胞增多,予以精皮质激素治疗后皮损消退,但外周血嗜酸性粒细胞计数持续增多.发病近4个月,结节再次出现,第2次组织病理检查示真皮及皮下组织内弥漫淋巴细胞浸润,多数浸润细胞核大,染色深,核分裂象易见.免疫组化染色:大的肿瘤细胞CD20、CD79a、Bcl-2均(+).诊断:原发性皮肤弥漫性大B细胞淋巴瘤.给予环磷酰胺、长春地辛、呲喃阿霉素、泼尼松(CHOP)方案化疗后皮损改善不明显.     

原发性皮肤弥漫性大B细胞淋巴瘤,腿型

报告1例原发性皮肤弥漫性大B细胞淋巴瘤,腿型.患者女,77岁.因右胫前多发暗红色斑块和结节3个月入院.皮损组织病理检查示:肿瘤细胞在真皮内弥漫性浸润性生长,侵及皮下组织,肿瘤细胞体积增大,形态不规则,细胞核大深染,核分裂象多见.免疫组化染色示:瘤细胞CD20(+),CD79a(+),Bcl-2(+),MuM-1(+).诊断:原发性皮肤弥漫性大B细胞淋巴瘤,腿型.通过CD20单克隆抗体加CHOP化疗方案治疗,病情明显好转.     

恶性血管内皮细胞增生症是一种亲血管性血管内淋巴瘤

最好认为恶性血管内皮细胞增生症(MAE)是一种淋巴结外的血管内淋巴瘤,或者是一种亲血管的大细胞淋巴瘤,该病以小血管内瘤性淋巴细胞增殖为特征,皮肤和中枢神经系统血管最常受累。该文3例患者经常规镜检和免疫组化分析肯定了MAE诊断,并对其中1例患者的皮损进行了分子遗传学分析和电镜研究。 病例1为74岁女性患者,腿部红色皮损10月,检查见双下肢及股部有红色坚实的皮下结节,结节活检示MAE变化;免疫组化证实血管内为B淋巴细胞浸润;分子遗传学研究示B     

皮下脂膜炎样T细胞淋巴瘤九例临床病理及免疫表型研究

目的 分析皮下脂膜炎样T细胞淋巴瘤(SPTL)的临床及组织病理表现、免疫表型、治疗和预后.方法 回顾性分析9例SPTL患者的临床与实验室资料,完善相关病理及免疫组化标记,并随访.结果 9例患者中8例表现为多发皮下结节和肿块,主要累及下肢(8例)和躯干(6例),7例伴发热.3例行全身PET-CT,7例行骨髓穿刺,未见皮肤外内脏系统肿瘤,均不伴噬血细胞综合征.皮肤组织病理示异形单个核细胞核大、深染,主要浸润皮下脂肪组织,围绕单个脂肪细胞呈环状排列,真皮层附属器和血管周围可见肿瘤细胞浸润(5/9例).免疫表型示9例肿瘤细胞表达βF1、CD3、CD8,8例表达粒酶B和T细胞胞内抗原1(TIA-1),均不表达CD4、CD20、CD30、CD56.5例接受化疗(1例儿童和1例产后妇女),1例儿童接受甲泼尼龙冲击治疗,随访8例治疗后均达到临床完全缓解.结论 SPTL来源于α/βT细胞,组织病理和免疫组化检查有助于诊断及鉴别诊断.     

血管内筋膜炎2例

报告2例发生在掌跖部位的血管内筋膜炎.例1.女,65岁.右跖皮下结节1个月.例2.男,76岁.右掌蓝色皮下结节5年.2例患者皮损组织病理检查均显示扩张的血管内大量梭形细胞增生:免疫组化染色结果示增生的梭形细胞波形蛋白、平滑肌肌动蛋白阳性,CD31和CD34阴性.结合临床及组织病理特点诊断为血管内筋膜炎.     

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...     

皮肤Merkel细胞癌一例

患者，女，77岁。因左侧面部肿物3个月就诊。皮损组织病理检查:肿瘤位于真皮内，累及皮下脂肪层，瘤细胞排列呈巢团状、弥漫片状，核大深染，异型、核分裂相易见，切缘可见肿瘤细胞。诊断为Merkel细胞癌。     

原发皮肤结外NK/T细胞淋巴瘤-鼻型一例

74岁女性患者,双下肢反复多发肿块伴溃疡6个月。皮损组织病理示:大、中、小3种肿瘤细胞混合浸润,有明显的血管中心性和血管破坏,伴广泛的凝固性坏死和溃疡。免疫表型:CD3ε、CD43、CD56、TIA-1、EB病毒潜伏膜蛋白(EBV LMP-1)、粒酶B(Granzyme B,Gr B)均阳性,Ki-67阳性(≥85%),CD8+CD4+。全身检查未发现皮肤以外系统受累证据,诊断为原发皮肤结外NK/T细胞淋巴瘤-鼻型。该病恶性程度高,需尽早进行组织病理检查和免疫组化染色以帮助诊断。     

Intravascular large B-cell lymphoma with cutaneous manifestations: a clinicopathologic, immunophenotypic and molecular study of three cases

Background:  Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare type of malignant lymphoma characterized by exclusive or predominant growth of neoplastic cells within the lumen of blood vessels. Cases in the literature predominantly involve the skin and central nervous system, with special emphasis on the 'cutaneous variant'.
Methods:  Three cases of IVLBCL with cutaneous manifestations, including two systemic IVLBCL and one cutaneous variant, were described in this study. In all cases, clinical presentation and follow-up data were meticulously evaluated and immunophenotypic and molecular studies were performed.
Results:  All three cases displayed the B-cell phenotype and showed monoclonality with immunoglobulin heavy chain gene rearrangement. Bcl2 was expressed in the two systemic IVLBCL cases with fatal outcomes. The third patient with the 'cutaneous variant' achieved complete remission and a longer survival time of 15 months after chemotherapy.
Conclusions:  Skin manifestations and neurological findings, although to different degrees, are important clues to the diagnosis of IVLBCL. As most IVLBCL are grouped into the post-germinal center B-cell subtype of diffuse large B-cell lymphoma, Bcl2 expression may be correlated with a worse prognosis in IVLBCL. The cutaneous variant of IVLBCL has a significantly better outcome than that of systemic IVLBCL.     

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.     

Subcutaneous T-cell lymphoma

A 70-year-old woman presents with a 2-year history of intermittent, subcutaneous nodules. The patient was otherwise asymptomatic. A biopsy specimen was consistent with a subcutaneous T-cell lymphoma, a rare subset of peripheral T-cell lymphoma; when accompanied by the hemocphagocytic syndrome, it can be rapidly fatal. The histopathologic characteristics and nature of the disease are discussed.     

脂膜炎样T细胞淋巴瘤1例

报告1例皮下脂膜炎样T细胞淋巴瘤,患者女,44岁,左侧臀部出现红肿、结节,伴疼痛和发热半个月,皮损组织病理检查：皮下脂肪层有淋巴细胞、组织细胞浸润,免疫组化证明浸润淋巴细胞为T细胞来源,组织病理改变符合脂膜炎样T细胞淋巴瘤。     

Lymphomatoid Papulosis Followed by Ki-1 Positive Anaplastic Large Cell Lymphoma: Proliferation of a Common T-Cell Clone

We report a case of lymphomatoid papulosis (LyP) followed by Ki-1 positive anaplastic large cell lymphoma (LCL). A 33-year-old man developed subcutaneous nodules in the left inguinal region and the left thigh after a seven-year-history of self-healing papulonecrotic lesions of LyP. Histological and immunohistochemical examination of the subcutaneous nodules revealed LCL. DNA was isolated from a nodule of the initial stage of LyP in 1988, a subcutaneous nodule of LCL in 1993, and a papule of LyP in 1993 which appeared after chemotherapy for LCL. T cell receptor gene rearrangement analysis demonstrated an identical rearranged pattern in all the three specimens, indicating that a common T cell clone proliferated throughout the course in both the LyP and LCL lesions.     

Subkutanes pannikulitisches T-Zell-Lymphom

Subcutaneous panniculitic T-cell lymphoma is categorized as a subtype of peripheral T-cell lymphoma. Patients typically present with nontender subcutaneous nodules. The characteristic histologic features include the presence of atypical lymphocytes and benign macrophages that infiltrate between the adipocytes of the subcutis mimicking panniculitis. We report a 75-year old patient with a three week history of asymptomatic subcutaneous nodules. The diagnosis of subcutaneous T-cell lymphoma was confirmed by immunohistological and molecular biological studies. Chemotherapy had to be interrupted due to a rapid worsening of the patient's general condition. She died few weeks after confirmation of diagnosis.     

Subcutaneous panniculitis-like T-cell lymphoma in a 26-month-old child with a review of the literature

Subcutaneous panniculitis-like T-cell lymphoma is a rare cytotoxic T-cell lymphoma of the skin that preferentially infiltrates the subcutaneous tissue. We report here this lymphoma occurring in a 26-month-old Korean girl. She presented with multiple erythematous subcutaneous nodules on both extremities and her back along with systemic symptoms. She had a protracted course of multiple erythematous subcutaneous nodules for 1 month and a spiking fever was often noted. The histopathologic findings for the subcutaneous nodules were lobular panniculitis-like material that was composed of atypical lymphocytes and histiocytes. The atypical lymphocytes characteristically rimmed individual fat cells in a lace-like pattern and some of the histiocytes showed phagocytosed white blood cells occasionally. Immunophenotypic studies showed CD3(+), CD45RO(+), CD20(-), CD4(-), CD8(+), and CD56(-). She is currently being treated with combination chemotherapy of cyclophosphamide, doxorubicin, vincristine, and prednisolone.     

CD30 expression in a case of intravascular large B‐cell lymphoma,cutaneous variant

Intravascular large B‐cell lymphoma (IVLBCL) is one of the rarest B‐cell non‐Hodgkin lymphomas (NHL), with an aggressive clinical behavior and a poor prognosis; in fact, its treatment is still an unmet clinical need, with a 3‐year overall survival (OS) rate of 60% to 81%, and a central nervous system relapse rate of 25%. It usually presents as a widespread disease at diagnosis, with multi‐organ involvement. Previously considered as a diffuse large B‐cell lymphoma variant, it now represents a different extranodal large B‐cell lymphoma entity in the last WHO Classification of tumors of hematopoietic and lymphoid tissues. We hereby describe the case of an 84‐year‐old Italian woman with an IVLBCL, cutaneous variant, who suffered from early relapse after R‐COMP chemotherapy regimen, and was therefore treated with a palliative metronomic chemotherapy. Interestingly, neoplastic cells showed CD30 expression at relapse. CD30 positivity has never been reported in this disease so far, and its expression is known to be involved in NF‐kB activation. CD30 expression may be further studied as for prognostic and therapeutic significance; in fact, new therapeutic strategies, such as antibody‐drug conjugate targeting CD30, are now available.     

原发性皮肤CD30~+间变性大细胞淋巴瘤一例

患者,男,50岁。背部、前胸多发结节伴轻压痛半年余。皮损组织病理检查示:表皮角化过度、棘层肥厚;真皮全层及皮下组织间变性弥漫肿瘤细胞浸润、瘤细胞体积大,可见核分裂相。免疫组化结果:85%CD30强阳性,Ki-67约40%(+),CD3(++),LCA(+)。诊断:原发性皮肤CD30~+间变性大细胞淋巴瘤,T细胞型。