浆膜腔积液中的淋巴造血组织肿瘤细胞病理诊断分析

目的 探讨用细胞病理方法诊断浆膜腔积液中的淋巴造血组织肿瘤的可行性和准确性,避免漏诊和误诊,评估用细胞块切片进行免疫细胞化学染色的作用.方法 收集通过组织活检证实的伴发浆膜腔积液的淋巴造血组织肿瘤33例,对其临床特点、细胞形态、免疫表型特征等进行分析,另外1例结外鼻型NK/T细胞淋巴瘤进行EBER原位杂交检测,3例T淋巴母细胞白血病/淋巴瘤、2例弥漫性大B细胞淋巴瘤(DLBCL)和2例伯基特淋巴瘤进行了基因重排分析.结果 累及浆膜腔的33例淋巴造血组织肿瘤包括T淋巴母细胞白血病/淋巴瘤12例;成熟B细胞肿瘤16例,其中DLBCL 9例,伯基特淋巴瘤2例,浆细胞骨髓瘤2例,慢性淋巴细胞白血病/小B细胞淋巴瘤2例,套细胞淋巴瘤1例;成熟T细胞和NK细胞肿瘤3例,其中血管免疫母细胞性T细胞淋巴瘤、结外鼻型NK/T细胞淋巴瘤、T细胞幼淋巴细胞性白血病各1例;粒细胞肉瘤和肥大细胞肉瘤各1例.8例DLBCL、2例浆细胞骨髓瘤、慢性淋巴细胞白血病/小B细胞淋巴瘤、1例套细胞淋巴瘤、T淋巴母细胞白血病/淋巴瘤、血管免疫母细胞性T细胞淋巴瘤、肥大细胞肉瘤共16例为复发病例,其余17例均以浆膜腔积液为首发表现并由细胞病理初诊.所有病例细胞病理学诊断均与组织病理学诊断结果相符.结论 结合临床特点、细胞形态、免疫表型、原位杂交和基因重排等检测,用细胞病理方法可以对浆膜腔积液中的淋巴造血组织肿瘤,特别是复发病例进行明确诊断和鉴别诊断.     

小细胞性非特指外周T细胞淋巴瘤病理形态和免疫表型分析

目的 探讨小细胞性非特指外周T细胞淋巴瘤(PTCL,NOS)的临床病理与免疫表型及其病理诊断和鉴别诊断.方法 对5例小细胞性PTCL,NOS进行临床病理回顾性研究和随访,免疫表型检测(SP和EnVision法),以及EBER原位杂交和T细胞受体(TCR)基因重排分析.结果 5例均为男性,平均年龄52.6岁.中位病程1个月.5例中3例为临床Ⅳ期,2例为临床Ⅲ期.4例有全身浅表淋巴结及脾脏肿大,1例有肝肿大.2例有浆膜腔积液.行骨髓检查的4例中,3例有肿瘤累及.1例有外周血自细胞总数和淋巴细胞分类计数升高.主要病理改变为淋巴结结构的破坏和单一形态的小淋巴细胞弥漫性浸润,4例可见少数大的异形细胞散在分布,2例见小血管增生现象.5例之肿瘤细胞均表达两种以上T细胞分化抗原和CD43,表达CD99(3/4),均不表达CD20、末端脱氧核苷酸转移酶、CD56和粒酶B.Ki-67指数为5%-15%.4例行TCR基因重排分析,均存在TCRy基因克隆性重排,1例检出TCRβ基因克隆性重排.EBER原位杂交检测均为阴性.获得3例随访资料,且患者均死亡,平均生存时间21.7个月.结论 小细胞性PTCL,NOS少见,呈高临床分期,预后差,组织形态表现为惰性淋巴瘤.     

硬化性淋巴细胞性小叶性乳腺炎4例临床病理分析

目的探讨硬化性淋巴细胞性小叶性乳腺炎(sclerosing lymphocytic lobuartitis of breast,SLL)的临床病理特征、免疫表型、鉴别诊断及预后。方法回顾性分析4例SLL的临床病理资料、免疫表型、治疗及预后,并复习相关文献。结果SLL组织学表现为乳腺小叶萎缩伴小叶内大量淋巴细胞浸润,小叶间间质纤维化,有时小叶结构消失伴有淋巴滤泡形成,为假性淋巴瘤。免疫表型:小叶内CK导管上皮阳性,淋巴细胞以CD20阳性B细胞为主,伴有少量CD3阳性T细胞,后期淋巴滤泡免疫组化标记可见完整的CD21阳性FDC网,Ki-67增殖指数2%～3%,CD30阴性。结论 SLL是一种少见的乳腺炎症性病变,常伴发于I型糖尿病的女性患者,镜下以小叶为单位的炎症性病变及间质纤维化为特征,需与其它良恶性病变鉴别,治疗以手术切除为主,患者预后良好,罕见复发。     

肛管直肠恶性黑色素瘤7例临床病理学分析

目的探讨肛管直肠恶性黑色素瘤的临床病理特征。方法收集7例肛管直肠恶性黑色素瘤的临床病理资料并随访,分析其临床和病理组织学特征和免疫表型。结果本例中包含女性5例,男性2例,年龄50~68岁,平均61岁。肿瘤位于直肠下端1例,齿线附近2例,肛管肛缘4例。该肿瘤组织结构和细胞形态有多样性。组织结构上主要为弥漫片状、巢状、腺泡状。细胞形态主要为上皮样、梭型细胞样、淋巴细胞样。免疫组织化学检测显示肿瘤细胞HMB45、S-100和vimentin强阳性,LCA、CK和EMA均阴性,其中2例CEA灶性阳性,Ki-67 LI为20%~50%。结论肛管直肠恶性黑色素瘤是少见的恶性肿瘤,其特征性的组织形态、免疫表型有助于诊断和鉴别诊断。     

组织细胞肉瘤的临床病理观察

目的 探讨组织细胞肉瘤(histiocytic sarcoma,HS)的病理形态学、免疫表型、鉴别诊断和预后.方法 分析6例HS患者临床病理资料,对标本行光镜观察、免疫组织化学EliVision法染色,并对患者进行6～36个月的随访.结果 6例HS中男女各3例,年龄12～81岁(平均54.6岁).发生部位为淋巴结(2例)、皮肤及软组织(4例).组织形态改变:瘤细胞弥漫性浸润,呈中至大的多角形上皮样细胞,胞质丰富,嗜酸性,核圆形或不规则,空泡状,有1至多个明显核仁,核分裂象多见.细胞境界较清.6例均见双核瘤细胞,2例多形性明显,可见多核瘤巨细胞,3例可见灶状泡沫状胞质的瘤细胞,1例可见灶状肉瘤样梭性细胞区,2例可见噬血细胞现象.6例均见到多少不一的炎细胞背景.均弥漫表达白细胞共同抗原、CD4、CD68、CD163,溶菌酶染色的5例中4例阳性.4例得到随访资料,3例确诊后6～11个月内死亡,1例局限于皮肤及皮下软组织者已存活3年.结论 明确HS诊断需结合形态改变与免疫表型.该病就诊时多处于进展期,对化疗反应差,但少数病变局限的病例不表现为侵袭性过程,预后较好.     

原发皮肤NK/T细胞淋巴瘤2例临床病理分析

目的探讨原发皮肤NK/T细胞淋巴瘤的临床病理特征、诊断与鉴别诊断。方法对2例下肢皮肤NK/T细胞淋巴瘤行HE及免疫组化EnVision法染色,并复习相关文献。结果 2例肿瘤侵犯至皮下组织,肿瘤细胞为小至中等大小的淋巴细胞,胞质丰富、透明,异型性明显,核不规则,围绕血管生长,核分裂象活跃。免疫表型:瘤细胞表达CD2、CD3、CD56、TIA-1、perforin等,原位杂交检测EBER阳性,Ki-67增殖指数为60%～70%,初诊无其他脏器查见肿瘤,提示本组病例为原发皮肤的NK/T细胞淋巴瘤。结论原发皮肤NK/T细胞淋巴瘤临床罕见,侵袭性高,预后差,其组织形态复杂多样,需与其他T、B细胞淋巴瘤、鳞状细胞癌等鉴别。     

胃孤立性朗格汉斯细胞组织细胞增生症1例

目的探讨胃孤立性朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的组织形态和免疫表型及鉴别诊断。方法胃体大弯黏膜隆起性病灶活检组织石蜡包埋HE切片及免疫组化EnV ision两步法染色,进行形态学和免疫表型分析。结果胃体大弯黏膜隆起性病灶,胃壁固有层和黏膜下层可见单一、无特别组织结构的细胞聚集。细胞体积比较大,胞质淡红色;核椭圆形,高倍镜下核呈咖啡豆样,有一条纵向核沟,免疫组化标记CD1a、S-100均阳性;背景炎症反应明显,主要为中性粒细胞、小淋巴细胞、浆细胞及一些嗜伊红细胞。结论 LCH的诊断主要依赖形态学及免疫组化标记,胃孤立性LCH预后显著好于系统性病变。     

淋巴细胞丰富型经典型霍奇金淋巴瘤的病理诊断与鉴别诊断

目的 探讨淋巴细胞丰富型经典型霍奇金淋巴瘤(lymphocyte-rich classical hodgkin lymphoma,LRCHL)病理形态学特点、免疫表型、诊断与鉴别诊断.方法 观察和分析3例LRCHL的临床、组织病理学和免疫表型特征,并对病变淋巴结行EBV原位杂交检测.结果 3例患者中男性2例,女性1例,平均年龄49岁.临床表现以淋巴结肿大为主,均无B症状.镜检、免疫表型和EBV检测:淋巴结呈不规则小结节状增生,可见残留滤泡和增宽的套区,套区内见R-S样细胞,2例上皮样组织细胞浸润明显,均未见嗜酸性粒细胞和中性粒细胞.R-S样细胞CD30均阳性,2例CD15阳性,1例CD20异质性表达,1/2例EBV阳性.结论 LRCHL是经典型霍奇金淋巴瘤(classical hodgkin lymphoma,CHL)少见的一种亚型,具有独特的临床特征和预后,诊断上需与结节性淋巴细胞为主型霍奇金淋巴瘤(nodular lymphocyte predominant hodgkin lymphoma,NLPHL)、富于T/组织细胞的弥漫大B细胞淋巴瘤(T cell/histiocyte-rich large B-cell lymphoma,THRLBCL)、血管免疫母细胞性T细胞淋巴瘤(angioimmunoblastic T-cell lymphoma,AILT)及CHL的其它亚型相鉴别.     

浆细胞肿瘤病理形态的多样性

目的 观察浆细胞肿瘤的病理形态学特点,探讨其组织结构和细胞形态的类型及其鉴别诊断.方法应用HE、免疫组织化学(EliVision法),对46例浆细胞肿瘤的组织形态学及免疫表型进行研究.结果 46例浆细胞肿瘤中,有40例组织结构以弥漫分布为主,3例呈巢状结构而似神经内分泌肿瘤,3例出现硬化的纤维性背景.淀粉样物质沉积、钙化骨化及"血湖"样结构在部分病例中有可能会非常突出而掩盖了肿瘤性浆细胞的特点.细胞形态上,30例由较成熟和欠成熟的浆样细胞组成而较易辨认.6例由类似免疫母细胞的浆母细胞组成.4例肿瘤细胞较小,似小淋巴细胞.2例瘤细胞胞质透亮似透明细胞或印戒细胞.另各有1例分别由异型性明显的间变型细胞、组织细胞样细胞及梭形细胞构成.最后1例细胞形态多样,可出现分叶核、单核及多核型细胞.免疫表型上93.1%(27/29)的病例表达CD79a而仅有5.1%(2/39)的病例表达CD20,87.1%(27/31)的病例表达CD38和83.3%(25/30)表达CD138,96.8%(30/31)的病例表达MUM-1.38例呈免疫球蛋白轻链限制性表达,其中表达λ链27例,表达κ链11例.结论浆细胞肿瘤除了常见的组织形态外,还可出现不典型或少见的组织结构和细胞形态,诊断时应注意与其他类型淋巴瘤如小淋巴细胞淋巴瘤及间变性大细胞淋巴瘤、低分化癌、透明细胞或印戒细胞癌、间叶性肉瘤等进行鉴别,免疫组织化学是必不可少的.     

富于细胞性神经鞘瘤2例临床病理分析

目的 探讨富于细胞性神经鞘瘤临床病理学特点及其诊断与鉴别诊断.方法 对2例富于细胞性神经鞘瘤进行临床资料分析、组织学形态和免疫表型观察.结果 富于细胞性神经鞘瘤好发于腹膜后,临床上表现为缓慢生长的无痛性肿块.大体肿瘤呈圆形、椭圆形,包膜完整,切面实性,灰白、灰黄色.镜下肿瘤具有完整的包膜,在包膜外或包膜下可见淋巴细胞聚集灶,形成袖套样结构.免疫组化肿瘤细胞S-100、GFAP和CD57均弥漫强阳性,不表达CK(AE1/AE3)、desmin、SMA、CD34、CD117和DOG1.结论 富于细胞性神经鞘瘤是一种少见的假肉瘤性病变,大体表现、组织学特点是其诊断及鉴别诊断的主要依据,免疫组化标记有助于诊断和鉴别诊断.     

儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病的临床病理观察

目的 探讨儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病(CSEBV+T-LPD)的临床病理特征、诊断及鉴别诊断要点.方法 经过HE染色观察并采用免疫组织化学、原位杂交技术,结合临床资料,对30例CSEBV+T-LPD病例进行回顾性研究,并进行随访.结果 (1)CSEBV+T-LPD,男19例,女11例,中位年龄9岁;起病至确诊的时间平均14个月;主要临床表现有发热(96.7%),淋巴结增大(83.3%),肝脾肿大(66.7%),常见皮肤损害包括蚊虫叮咬超敏反应(13.3%)和皮疹(20.0%);20例随访患者中6例死亡,4例发展为淋巴瘤.(2)组织病理特点:淋巴结表现为T区扩大,淋巴滤泡缩小、减少甚至消失;病变主要为中小淋巴细胞以及多少不等的组织细胞,大细胞散在其中,轻至中度异型;21例淋巴结的间质及小血管增多,11例包膜增厚;另外,肝、脾和皮肤主要表现为轻至中度异形淋巴细胞浸润;3例可见噬血细胞现象.(3)免疫表型特点:病变中的中小淋巴细胞表达CD3、粒酶B和T细胞胞质内抗原(TIA-1);8例检测病例中7例表达CD8,1例主要表达CIM,也表达CD8;15例检测了CD56,2例阳性并已进展为NK/T细胞淋巴瘤,其余13例阴性.(4)30例均检测出EB病毒编码的小RNA(EBER)阳性细胞,数量5～500个/HPF;EBER在各种淋巴细胞中均阳性,9例以中至大淋巴细胞阳性为主,其余主要是中小淋巴细胞阳性;主要分布在T区,也见于套区、初级滤泡和生发中心内.结论 中国存在CSEBV+T-LPD;主要发生于儿童和青少年,病程为亚急性或慢性伴反复发热;此病为系统性病变,主要累及淋巴结和肝脾,也可累及皮肤,淋巴结以T区扩大和轻中度异形淋巴细胞浸润为主要形式;EB病毒感染的细胞毒性T细胞构成病变主体;是一种危及生命的疾病,患者往往死于严重并发症或进展为恶性淋巴瘤;应综合考虑临床、病理、免疫表形和EB病毒感染4方面信息以获得准确诊断.     

儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病的临床病理观察

目的 探讨儿童系统性EB病毒阳性T细胞淋巴组织增殖性疾病(CSEBV+T-LPD)的临床病理特征、诊断及鉴别诊断要点.方法 经过HE染色观察并采用免疫组织化学、原位杂交技术,结合临床资料,对30例CSEBV+T-LPD病例进行回顾性研究,并进行随访.结果 (1)CSEBV+T-LPD,男19例,女11例,中位年龄9岁;起病至确诊的时间平均14个月;主要临床表现有发热(96.7%),淋巴结增大(83.3%),肝脾肿大(66.7%),常见皮肤损害包括蚊虫叮咬超敏反应(13.3%)和皮疹(20.0%);20例随访患者中6例死亡,4例发展为淋巴瘤.(2)组织病理特点:淋巴结表现为T区扩大,淋巴滤泡缩小、减少甚至消失;病变主要为中小淋巴细胞以及多少不等的组织细胞,大细胞散在其中,轻至中度异型;21例淋巴结的间质及小血管增多,11例包膜增厚;另外,肝、脾和皮肤主要表现为轻至中度异形淋巴细胞浸润;3例可见噬血细胞现象.(3)免疫表型特点:病变中的中小淋巴细胞表达CD3、粒酶B和T细胞胞质内抗原(TIA-1);8例检测病例中7例表达CD8,1例主要表达CIM,也表达CD8;15例检测了CD56,2例阳性并已进展为NK/T细胞淋巴瘤,其余13例阴性.(4)30例均检测出EB病毒编码的小RNA(EBER)阳性细胞,数量5～500个/HPF;EBER在各种淋巴细胞中均阳性,9例以中至大淋巴细胞阳性为主,其余主要是中小淋巴细胞阳性;主要分布在T区,也见于套区、初级滤泡和生发中心内.结论 中国存在CSEBV+T-LPD;主要发生于儿童和青少年,病程为亚急性或慢性伴反复发热;此病为系统性病变,主要累及淋巴结和肝脾,也可累及皮肤,淋巴结以T区扩大和轻中度异形淋巴细胞浸润为主要形式;EB病毒感染的细胞毒性T细胞构成病变主体;是一种危及生命的疾病,患者往往死于严重并发症或进展为恶性淋巴瘤;应综合考虑临床、病理、免疫表形和EB病毒感染4方面信息以获得准确诊断.     

Primary gastric T-cell lymphoma with manifold histologic appearances.

A rare case of malignant T-cell lymphoma with manifold histologic appearances was described. The lymphoma occurred in the stomach of a 50-year-old Japanese male. Grossly, the lymphoma exhibited a deeply ulcerated mass. Histologically, in addition to diffuse infiltrate of large lymphoid cells with deeply indented nuclei, there were many epithelioid cell granulomas, remarkable tissue eosinophilia and stromal fibrosis, mimicking inflammatory disease. Immunohistochemical studies and a gene analysis demonstrated the T-cell phenotype.     

Primary Gastric T-cell Lymphoma with Manifold Histologic Appearances

A rare case of malignant T-cell lymphoma with manifold histologic appearances was described. The lymphoma occurred in the stomach of a 50-year-old Japanese male. Grossly, the lymphoma exhibited a deeply ulcerated mass. Histologically, in addition to diffuse infiltrate of large lymphoid cells with deeply indented nuclei, there were many epithelioid cell granulomas, remarkable tissue eosinophilia and stromal fibrosis, mimicking inflammatory disease. Immunohistochemical studies and a gene analysis demonstrated the T-cell phenotype.     

Primary Hodgkin lymphoma of the stomach--a case report

A case of a 69-year-old male with a one-month history or recurrent attacks of GI bleeding, secondary anemic syndrome and epigastric pain is reported. Endoscopic examination revealed a tumorous lesion suspicious of malignancy. Neither lymphadenopathy nor any other tumorous lesion was identified by extensive clinical examination. In the biopsy specimens there was ulcerated gastric mucosa with an infiltration by lymphoid cells, predominantly of mature T-cell type. Atypical large cells with large nuclei with prominent nucleoli were dispersed among the mature lymphocytes. These atypical cells were both mono- and binucleated (Hodgkin and RS cells). Both cell types revealed immunohistochemically membranous and dot-like perinuclear positivity of CD30 and CD15 antigens. Based on morphologic features and immunohistochemical findings, a diagnosis of primary gastric Hodgkin lymphoma was established. Diagnostic approach, as well as differential diagnosis of primary Hodgkin lymphoma in this extremely rare location, are discussed.     

Aggressive natural killer-like T-cell malignancy with leukemic presentation following solid organ transplantation

NK-like T-cell malignancies are part of a spectrum of lymphoproliferative diseases that complicate immunosuppression associated with solid organ transplantation. We describe 2 patients with long-standing immunosuppression following solid organ transplantation. Both patients had systemic symptoms that included fever, myalgia, and weight loss. Organ involvement and lymphadenopathy were not initially observed. Unique to these 2 cases are the initial leukemic symptoms, which led to further characterization and identification of NK-like T-cell malignancies. Both patients exhibited an anomalous T/NK phenotype, CD56 positivity, and atypical blastic architecture of the large granular lymphocytes. Clonal rearrangement of T-cell receptor genes was detected in both patients. In 1 patient, a cytogenetic abnormality involving 8q24 was demonstrated. The disease course in both patients was aggressive, with involvement of multiple sites and rapid demise. This study emphasizes the importance of including NK-like T-cell malignancies in the differential diagnosis of lymphoproliferative disorders associated with immunosuppression and recognizing that an aggressive clinical course may follow leukemic presentation of disease.     

B-and T-cell non-Hodgkin''s lymphomas with large multilobated cells: morphological, phenotypic and clinical heterogeneity

Ten cases of non-Hodgkin's lymphomas, mainly composed of large multilobated cells, have been studied. Our results are consistent with the view that they represent a somewhat heterogeneous group of lymphoid tumours displaying different morphological, clinical and immunophenotypic features. In B-cell type the large multilobated cells were histologically characterized by prominent nucleoli and distinctly basophilic cytoplasm whereas in the T-cell type they had indistinct or small nucleoli and ill-defined weakly eosinophilic cytoplasm. These differential features between B- and T-cell type were confirmed by electron microscopy. From a clinical standpoint B-cell type was characterized by a constant involvement of lymphoid tissues (lymph nodes and/or Waldeyer's ring); T-cell type showed, on the contrary, a more frequent involvement of extra-lymphoid sites (mainly bone and subcutaneous tissues). Our study provides some morphological features that may be helpful for a correct differential diagnosis in this heterogeneous group of non-Hodgkin's lymphomas.     

Myxoid variant of follicular dendritic cell sarcoma arising in the breast

Follicular dendritic cell sarcoma is a malignant tumor of the follicular dendritic cell which can arise in extranodal sites. We present here a case arising as a mass in the breast of a 41-year-old woman. The tumor was composed of mildly pleomorphic spindly cells with pale ovoid nuclei and cell processes intimately admixed with mature lymphocytes. In much of the lesion the cells were dispersed in cords in a myxoid stroma, and elsewhere there were solid sheets. The neoplastic cells were immunoreactive for CD21, CD35, EMA, and S100 protein, but not for other lymphoid markers or cytokeratin. Electron microscopy showed interdigitating cytoplasmic processes with junctions but no external lamina. The differential diagnosis includes carcinoma, lymphomas, and a variety of myxoid sarcomas. The tumor recurred within a few months and displayed increased nuclear pleomorphism and lymphatic invasion but the patient appears free of disease 3 years after the further excision. This case extends the spectrum of follicular dendritic cell sarcoma in soft tissue sites.     

Kimura's disease of the lymph node. Histology, immunohistochemistry, and differential diagnosis

Kimura's disease, a chronic inflammatory condition of unknown cause, occurs predominantly in young Asian adult men. The disease involves subcutaneous tissues and lymph nodes in the head and neck region. We report a new case occurring in a young woman. The diagnosis was made late on a retroauricular lymph node examination. Immunohistochemical study showed that B lymphoid area remained, located in the germinal center showing some fibrosis involvement, and in the subcortex. T lymphoid zones were observed in the paracortex. Strong IgE positivity was noted in germinal centers. Vessels were positive with CD31, CD34, factor VIII and smooth muscle actin antibodies. Warthin-Finkeldey cells were negative for lymphoid and macrophagic markers used in this study. Differential diagnosis is discussed.