以胃肠道症状首发伴有皮肤表现的血管内NK/T细胞淋巴瘤一例

患者男,51岁,上腹部胀痛4个月,颈项、躯干、双大腿结节、斑块1个月。当地多家医院行胃肠道相关检查,并予对症处理,治疗效果不佳。腹部皮肤结节组织病理检查：真皮下层和皮下脂肪小叶内大量增生的小血管腔内填塞中等偏大异形淋巴样细胞;免疫组化标记：血管腔内异形细胞CD2（+++）、CD4（-）、CD8（-）、CD3ε胞质（++）、CD99（++）、CD43（+++）、CD56（+++）、细胞毒颗粒相关蛋白（TIA-1）（++）、穿孔素（++）、EBER（+++）、CD20（-）、CD79a（-）、CD30（-）、细胞角蛋白（-）、S100（-）、CD68（-）、血管内皮细胞CD31（+）、CD34（+）。诊断：以胃肠道症状首发伴有皮肤表现的血管内NK/T细胞淋巴瘤。给予环磷酰胺、长春新碱、柔红霉素、地塞米松 + 依托泊苷方案化疗,病情得到迅速控制,目前仍在随访中。     

皮肤淋巴细胞浸润症一例

患者,女,46岁。鼻根部肿胀性红斑2年,伴双眼睑水肿及双侧耳前皮下肿块20天。查体可见患者鼻背部及左侧下眼睑蚕豆大淡红色浸润性红斑,与周围正常皮肤界限不清,双侧耳前可触及花生大质硬椭圆形肿块。皮肤组织病理示（鼻根部）：表皮轻度角化亢进,真皮层毛囊及皮脂腺周围可见较多淋巴细胞浸润。免疫组化染色显示：CD2（+）,CD3（+）,CD7（+）,CD20（+）,CD30（-）,CD56（-）,CD4（+）,CD8（+）,Ki67（10%+）,EBER（-）,提示浸润细胞以T淋巴细胞为主,伴少量B淋巴细胞。诊断：Jessner皮肤淋巴细胞浸润症。     

黏液炎性纤维母细胞肉瘤

【摘要】 患者男,73岁。左小腿红斑、斑块伴微痒2年,加重为结节、肿块伴疼痛半年余。皮肤科检查：左小腿伸侧、腓侧中下1/3处多个大小不一红斑、斑块、结节、肿块。肿块质地坚韧,基底深在,可活动,边界不清,压痛明显。肿块表面皮肤部分外观正常。组织病理学检查：表皮未见明显异常;真皮中下部及皮下脂肪层可见梭形细胞及大小不一、胞体大、胞质红染、核大、浓染不规则的细胞,呈片状分布,其中可见病理性核分裂象,间质内可见淡蓝染物质,其间杂有淋巴细胞浸润。间质阿新蓝染色阳性。免疫组化染色：梭形细胞及异形大细胞弥漫性强阳性表达波形蛋白（Vim）,30% ~ 40%瘤细胞表达Ki67,CD34和CD68部分细胞阳性,而白细胞共同抗原、人黑素瘤S100蛋白、pCK、CD31、平滑肌肌动蛋白及结蛋白均为阴性。诊断：黏液炎性纤维母细胞肉瘤。     

以皮肤肿块为首发临床表现的伴MYC和BCL2重排弥漫性大B细胞淋巴瘤

报告1例以皮肤肿块为首发临床表现的伴细胞性骨髓细胞瘤病病毒癌(MYC)基因和B细胞淋巴瘤因子2(BCL2)基因重排弥漫性大B细胞淋巴瘤。患者女,87岁。左侧眉弓上方皮肤结节1年。皮肤科检查：左眉上方一肤色圆形肿物,隆起于皮肤表面,肿物表面可见浸润性红斑,伴少许渗出,并见结痂、鳞屑,边界清楚。皮损组织病理检查：表皮形态正常,真皮层内大量母细胞样淋巴细胞结节状浸润。免疫组化：母细胞样淋巴细胞CD45、CD79a、B细胞淋巴瘤因子6(BCL6)、细胞周期蛋白D1(cyclin D1)及MYC均(+);CD3、CD2、CD30、细胞角蛋白(CKP)、间变性淋巴瘤激酶(ALK)及Epstein-Barr病毒编码RNA(EBER)均(-)。荧光原位杂交检测：MYC、BCL2及BCL6均重排。诊断：伴MYC和BCL2重排弥漫性大B细胞淋巴瘤。患者未治疗,2个月后死亡。     

套细胞淋巴瘤伴皮肤虫咬样反应一例

【摘要】 患者男,74岁,确诊套细胞淋巴瘤5年,躯干、四肢丘疱疹伴瘙痒10个月就诊。皮疹瘙痒剧烈,给予抗组胺药物对症治疗不能缓解。体检：躯干、四肢皮肤见散在绿豆至黄豆大小红色丘疹及丘疱疹,部分表面见浅表结痂,以双上肢皮损为主,颈部、双侧腹股沟可触及肿大淋巴结,约2 cm × 1 cm。颈部淋巴结病理示,正常淋巴结结构完全破坏,中等大淋巴样细胞呈结节状或弥漫增生浸润,免疫组化示CD20（+++）,CD79α（+++）, Bcl-2（++）,细胞周期蛋白D1（+++）,CD5（++弱）,CD43（++）,Bcl-6（++）, PAX-5（+++）,κ（+++）,λ（±） ,Ki-67（10% ~ 30%+不均）。皮损组织病理及免疫组化：表皮大致正常,真皮浅中层血管、附属器周围见以中等大小淋巴样细胞为主的团灶状浸润,其间散在嗜酸性粒细胞;免疫组化：CD3（部分+）,CD5（弥漫性+）,CD20（部分+）,细胞周期蛋白D1（部分+）,Ki-67（10% +）。根据临床资料、淋巴结及皮损组织病理及免疫组化,诊断为套细胞淋巴瘤伴皮肤虫咬样反应。     

原发皮肤ALK阴性间变性大细胞淋巴瘤1例并文献复习

报告1例原发皮肤间变性大细胞淋巴瘤。患者男,42岁。左小腿红斑5年余,发现左小腿结节10月。皮损组织病理检查:真皮浅层及深层可见淋巴细胞团块状及片状浸润,细胞核大、异型,核分裂象多见。免疫组化示CD4、CD7、CD56、CD45RA、CD45RO、LCA、CD30、VIM阳性,CD8、CD20、Ki67、TDT、P53、ALK阴性,kappa、lamda散在个别(+),CD68散在(+)。诊断为原发皮肤ALK阴性间变性大细胞淋巴瘤。     

伴噬血细胞综合征的皮肤NK/T细胞淋巴瘤鼻型一例

患者男,48岁,反复面颈、躯干、四肢丘疱疹10年,加重10 d,发热4 d。皮损组织病理检查示真皮内大量小到中等异形淋巴样细胞浸润,以血管及附属器周围明显,伴血管结构的破坏,并可累及皮下脂肪小叶间隔。免疫组化标记示真皮及皮下组织浸润的异形细胞CD45RO（+++）、CD3（-）、CD4（-）、CD8（+）、CD56（+）、TIA-1（++）、粒酶B（+）、EBER（++）、CD3ε胞质（++）、CD20（-）、CD79a（-）、CD30（-）。实验室检查示外周血红细胞、白细胞、血小板进行性下降,转氨酶、胆红素持续性升高,高甘油三酯血症及纤维蛋白原降低,腹部B超示肝脾肿大。诊断：皮肤NK/T细胞淋巴瘤鼻型伴噬血细胞综合征。     

原发性皮肤CD30+间变性大细胞淋巴瘤1例及文献复习

报告1例原发性皮肤CD30+间变性大细胞淋巴瘤。患者男,73岁。右上肢红色丘疹伴溃疡1年余。皮肤科检查：右上肢邻近腋窝外侧可见数个圆形或类圆形红色丘疹,边界清晰,部分融合成片状,皮损中央破溃、结痂,其间可见色素沉着及瘢痕形成,质稍硬。皮损组织病理检查：真皮全层大量异形淋巴细胞弥漫浸润,核大深染,可见核分裂象。免疫组化：CD30强阳性,CD2、CD4、CD43、MUM-1及TIA-1均阳性,Granzyme B（部分细胞阳性）,增殖核抗原（Ki-67）（约80%阳性）,CD20、CD79a、CD56、BCL-2、BCL-6及CD10均阴性。EB病毒编码小RNA(EBER)原位杂交阴性。诊断：原发性皮肤CD30+间变性大细胞淋巴瘤。     

血管免疫母细胞性T细胞淋巴瘤1例

患者,男,64岁,躯干部丘疹1个月,皮疹逐渐增多,瘙痒明显,淋巴结肿大伴高热。淋巴结病理检查可见病理性核分裂象。免疫组化示：Ki-67标记指数约90%、CD21及CD23（示FDC网结构紊乱）、CD3及CD5（+）、CD34（血管+）、CD30（免疫母细胞+）、BCL-2（+）。 诊断：血管免疫母细胞性T细胞淋巴瘤。     

表现为神经淋巴瘤病的外周T细胞淋巴瘤一例

31岁男性患者,全身反复出现红斑、结节伴肢端麻木感8年。体格检查:全身皮肤散在直径1～5 cm淡红至紫红色斑片和结节。双侧颈部及腹股沟可触及数个直径1 cm的淋巴结。双手尺侧及双足深浅感觉减退,双下肢腱反射减弱。四肢肌电图检查示:获得性多灶性感觉运动神经病。PET-CT提示淋巴瘤。右肘尺神经组织病理示:弥漫增生的淋巴样细胞;免疫组化染色示:肿瘤细胞CD3、CD5、TIA-1均阳性,Ki-67约40%～70%阳性。TCR克隆性基因重排阳性。左小腿皮肤结节组织病理:表皮和真皮浅层、皮肤附属器及部分血管周围较多异形淋巴样细胞浸润;免疫组化染色示:CD3、CD5、GrB、TIA-1均阳性,Ki-67约40%阳性。诊断为:(1)非霍奇金淋巴瘤IVB期;(2)外周T细胞淋巴瘤,非特指型;(3)神经淋巴瘤病。治疗:先后采取吉西他滨+培门冬酶、DICE、CHOP、EPOCH+来那度胺、BECOP+西达苯胺等化疗方案,目前口服西达苯胺治疗,原皮损逐渐消退,但双小腿仍有新发结节,总体病程缓慢进展。     

间变性淋巴瘤激酶阴性的间变性大细胞淋巴瘤泛发性皮肤侵犯一例

患者男,37岁,入院前7个月无明显诱因右大腿出现一鹅蛋大小肿物,无明显不适,肿物逐渐增大,右大腿、臀部出现弥漫性、非凹陷性肿胀,入院前2个月全身皮肤出现暗红色丘疹、结节、斑块,部分斑块渐出现大小不一的糜烂、溃疡。实验室检查：白蛋白降低,乳酸脱氢酶显著升高。B超示浅表淋巴结肿大、融合,彩色多普勒示淋巴结内部较丰富的树枝样血流信号。CT显示右大腿及会阴部广泛淋巴结肿大伴软组织水肿,上腹部广泛淋巴结肿大,纵膈内淋巴结肿大。皮损组织病理：真皮全层致密分布单一核细胞,部分有异形性及不典型核分裂;免疫组化：CD3、CD8、CD30（阳性细胞占80%）、CD4、CD45RＯ、粒酶B 阳性,CD56、间变性淋巴瘤激酶（ＡＬＫ）、T细胞胞质内抗原1阴性。淋巴结病理：淋巴结结构完全破坏,肿瘤弥漫成片生长,肿瘤细胞比一般的大细胞淋巴瘤瘤细胞大,胞质丰富,嗜碱性或嗜双色性,细胞核偏位,呈马蹄形、肾形或分叶状,核染色质稀疏,可见单个或多个嗜碱性小核仁;免疫组化：CD2、CD4、CD3、粒酶B、上皮膜抗原（EMA）、Ki-67、CD30阳性,CD8、CD56、T细胞胞质内抗原（TIA）-1、ＡＬＫ均为阴性。诊断：间变性淋巴瘤激酶阴性的原发系统型间变性大细胞淋巴瘤泛发性皮肤侵犯。     

A case of intravascular large B-cell lymphoma mimicking erythema nodosum: the importance of multiple skin biopsies

BACKGROUND: Intravascular lymphoma is a rare disease characterized by the proliferation of neoplastic monuclear cells within the lumens of small blood vessels. The neoplastic cells are usually of B-cell origin, and rarely of T-cell or histiocytic origin. Although this clinicopathological entity of lymphoma has not been listed in general pathological classifications such as REAL classification or the Working Formulation, it is recently in the WHO classification scheme, which is essentially an updated REAL scheme, and the EORTC classification scheme. METHODS: In this report, a 62-year-old woman with intravascular large B-cell lymphoma was observed by clinical, histopathological, immunohistochemical and molecular methods. RESULTS: A 62-year-old woman presented with large erythematous macules on the bilateral thighs and lower legs. The lesions were accompanied with hard, tender, intradermal or subcutaneous nodules mimicking erythema nodosum. Histopathological examination in the first biopsy revealed non-specific panniculitis compatible with erythema nodosum. The second biopsy revealed emboli of atypical lymphocytes within many of the dilated and proliferated vessels in the deep dermis and subcutaneous tissue. These cells were positive for L-26 and kappa light chain, and negative for lambda light chain, factor VIII-related antigen, CD30, CD34, CD68 and UCHL-1. These findings confirmed the diagnosis of intravascular large B-cell lymphoma. A laboratory examination showed a high level of LDH and abnormal cells in the bone marrow. An MRI of the brain and computed tomographic (CT) scans of the chest and abdomen revealed no evidence of malignancy. Before the treatment, the size of the nodules decreased spontaneously by about 50% in one month and significantly in two months. Although combination chemotherapy, which consisted of CHOP, brought her partial remission, she experienced neurological symptoms 6 months after the initial treatment and died of brain metastasis 9 months after the treatment. CONCLUSIONS: This is a unique case for two following reasons: 1) the first biopsy revealed non-specific findings compatible with erythema nodosum; and 2) before the treatment, the nodules regressed spontaneously. Dermatologists should take multiple skin biopsies for EN lesions with the non-specific histopathological findings not to refute the existence of this disease.     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤.     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤.     

Anaplastic large-cell T-cell lymphoma

A 64-year-old woman presented with 2 years of pruritic and ulcerated nodules and tumors on the trunk and arms. Histopathologic examination showed a diffuse infiltrate that consisted of predominantly small lymphocytes and scattered large atypical multinucleated cells positive for CD30. These findings were consistent with a diagnosis of anaplastic large-cell T-cell lymphoma, which is a CD30+ cutaneous lymphoma. This case highlights the importance of considering both histopathologic and clinical criteria in diagnosing a patient with a CD30+ cutaneous lymphoma.     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤一例

报告1例原发性皮肤CD4+多形性小/中T细胞淋巴瘤.患者男,19岁.全身多发暗红色斑块和小结节2年.皮损组织病理检查示,真皮内血管及附属器周围有致密小到中等大,胞质空亮,核扭曲的淋巴样细胞浸润,可见核分裂像.未见亲表皮性,但有浸润毛囊现象.免疫组化检查显示,CD3阳性、CD4阳性、CD8阴性、CD30阴性和CD20阴性,Ki-67阳性率约为25%.诊断:原发性皮肤CD4+多形性小/中T细胞淋巴瘤.     

Cutaneous intravascular CD30+ T‐cell pseudolymphoma occurring in a regressing keratoacanthoma

Cutaneous intravascular CD30+ pseudolymphoma is an uncommon incidental finding that may mimic intravascular or angiotropic lymphoma. We describe a 78‐year‐old female with a traumatized regressing keratoacanthoma on her left cheek. A shave biopsy revealed intravascular staining of atypical lymphocytes positive for CD45, CD3 and CD30. Clinical exam revealed no other evidence of lymphoma, the patient denied constitutional symptoms, and routine blood work was normal. The patient is healthy and doing well 28 months after her first visit. CD30+ pseudolymphoma should be distinguished from malignant intravascular lymphoproliferative disorders.     

累及皮肤的套细胞淋巴瘤一例

患者男,53岁,躯干四肢紫红色结节、斑块半月,眶周肿胀4 d。体检：全身多处浅表淋巴结肿大,脾肿大。血钙3.12 mmol/L,LDH 853 U/L,血免疫固定电泳示单克隆免疫球蛋白IgM阳性（κ链型）,正电子发射断层显像/X线计算机体层成像检查示全身多处淋巴结肿大,咽后壁肿块,脾脏大。颈部皮肤结节组织病理检查示皮下脂肪组织内大量异形淋巴样细胞弥漫浸润,体积中等偏大,核圆形,核仁较明显,核分裂易见。免疫组化标记示异形细胞L26、CD79a、Bcl-2、细胞周期蛋白 D1均阳性,多发性骨髓瘤原癌基因１部分阳性,Ki-67阳性率 > 80%, CD5、CD21、CD23、CD38、CD3、CD10、Bcl-6、CD45RO、末端脱氧核苷酸转移酶（TdT）、髓过氧化物酶（MPO）、CD30、间变性淋巴瘤激酶（ALK）、CD117、CD34均阴性。荧光原位杂交检测示t（11;14）CCND1/IGH融合基因阳性。诊断：伴皮肤和眶周受累的套细胞淋巴瘤（母细胞变异型）合并高钙血症。治疗：给予利妥昔单抗注射液联合环磷酰胺、长春新碱、多柔比星、地塞米松,交替以大剂量甲氨蝶呤及阿糖胞苷静脉注射,第3天血钙降至正常,第6天病情得到迅速控制,皮损及眶周肿胀明显消退,但随访治疗１个月后,患者最终死于严重肺部感染。     

泛发型多核细胞血管组织细胞瘤一例

患者,男,38岁.躯干、四肢起丘疹、结节1年.组织病理示:真皮浅中层血管增生,血管周围及胶原间可见少许多核巨细胞及淋巴细胞、组织细胞、浆细胞.免疫组织化学染色:CD68、CD3、CD4、CD8、CD20、CD79、CD30阳性,Ki-67<10％.诊断:泛发型多核细胞血管组织细胞瘤.     

Intravascular B-cell lymphoma: the role of skin biopsy

Intravascular B-cell lymphoma is a rare aggressive systemic neoplasm with cutaneous and neurological presentations, which commonly eludes the diagnosis ante mortem. First reported in 1959 as "angioendotheliomatosis proliferans" by Pfleger and Tappeiner, it is a subtype of extranodal diffuse large-B-cell lymphoma defined by an intravascular proliferation of clonal lymphocytes. We describe a case of intravascular lymphoma in a 68-year-old female who presented with altered mental status and indurated, erythematous, ecchymotic plaques with overlying telangiectasia and ulceration. The diagnosis was made by skin biopsy of an abdominal plaque revealing large hyperchromatic cells filling the lumina of several small blood vessels within the dermis and subcutis. CD20 and CD79a immunostains were strongly positive, confirming the diagnosis of intravascular large-B-cell lymphoma. On review of a previous biopsy from another institution, which was reported to be nondiagnostic, we were able to find tumor cells in the blood vessels but only very focally. The presence of a brisk, perivascular, nonneoplastic lymphocytic infiltrate may have obscured the identification of tumor cells. This case illustrates an unusual subtype of extranodal diffuse large-B-cell lymphoma, which demonstrates protean clinical presentations, requires microscopic examination for diagnosis, but can be easily overlooked on skin biopsy.