母细胞性浆细胞样树突细胞肿瘤三例临床及病理分析

回顾性分析2014年1月至2019年1月武汉协和医院收治的母细胞性浆细胞样树突细胞肿瘤（BPDCN）3例临床病例特点，并复习相关文献。3例患者 (男2例，女1例) 均以皮肤表现为首发症状，表现为淡红色丘疹、结节、斑块，组织病理均显示真皮及皮下可见弥漫浸润中等大小淋巴样细胞，可见核分裂象，表皮未受累；免疫组化显示CD4，CD56，CD123均阳性。     

母细胞性浆细胞样树突状细胞瘤

本文报告母细胞性浆细胞样树突状细胞瘤(BPDCN)1例。患者男,70岁。反复皮肤瘙痒伴红斑结节1年。皮损组织病理:瘤细胞在真皮内密集,呈结节、团块状浸润,核深染,形态不规则,可见病理性核分裂象。免疫组化:CD4、CD56、CD123、CD43、CD45RA均阳性。骨髓涂片:骨髓增生明显活跃,异常细胞占72.8%,骨髓流式免疫分型:人白细胞DR抗原(HLA-DR):98.6%,CD56:82.3%,CD38:83%,CD4:92%和CD123:94.6%。该文对BPDCN的诊断、鉴别诊断和治疗进行讨论,揭示该病呈高度侵袭性,预后差,生存期短。     

急性淋巴细胞性皮肤白血病

报告1例急性淋巴细胞性皮肤白血病.患者女,17岁.右胫前肿块2个月余,右大腿出现紫红色结节、躯干出现紫红色浸润性斑块1个月余,偶有疼痛和瘙痒.血常规检查正常.皮损组织病理榆查示真皮浅层有一狭窄无浸润带,在真皮和皮下组织可见大小不一的单一核细胞浸润,部分胞核较大,深染,偶见核丝分裂.免疫组化染色结果示CD3、CD4、CD5、CD8、CD20、CD79、MPO标志均为阴性,CD68阳性,且TdT标记30%～40%单一核细胞为阳性,提示为淋巴细胞前体细胞,结合骨髓细胞学检查诊断为急性淋巴细胞白血病(ALL-L2).给予患者DOCP(吡柔比星、地塞米松、环磷酰胺及长春新碱)方案化疗2个疗程后,胫前肿块缩小变平,身体其他部位皮损均消退.     

母细胞性浆细胞样树突细胞肿瘤

母细胞性浆细胞样树突细胞肿瘤是一种少见的侵袭性淋巴瘤,2008年正式命名.临床上以皮肤受累为首发症状,表现为挫伤样结节或斑块.该肿瘤易侵犯骨髓及淋巴结,病程凶险,预后差.其肿瘤细胞来源于浆细胞样树突细胞前体细胞,具有独特的免疫表型:CD4、CD56阳性,TdT和CD68部分阳性,还表达浆细胞样树突细胞相关表面标志CD123、TCL1和BDCA-2.但目前其发病机制仍未明确,且无标准治疗方案.     

母细胞性浆细胞样树突细胞肿瘤

母细胞性浆细胞样树突细胞肿瘤是一种少见的侵袭性淋巴瘤,2008年正式命名.临床上以皮肤受累为首发症状,表现为挫伤样结节或斑块.该肿瘤易侵犯骨髓及淋巴结,病程凶险,预后差.其肿瘤细胞来源于浆细胞样树突细胞前体细胞,具有独特的免疫表型:CD4、CD56阳性,TdT和CD68部分阳性,还表达浆细胞样树突细胞相关表面标志CD123、TCL1和BDCA-2.但目前其发病机制仍未明确,且无标准治疗方案.     

急性髓细胞性白血病(M4型)

报告1例急性髓细胞性皮肤白血病(M4型).患者女,48岁.全身出现丘疹、红色结节14d,伴剧烈瘙痒.体格检查:全身泛发大小不等的红色丘疹、结节,质韧,无压痛.皮损组织病理检查:真皮内弥漫淋巴样细胞浸润,有明显异形及较多核分裂象.免疫组化组织病理检查:CD68阳性(灶性),MPO阳性(少量).骨髓穿刺:白血病细胞大量增生,免疫标记:CD68、CD11b、MPO及HLA-DR均阳性.诊断:急性髓细胞性白血病(M4型).患者经过2次DA(伊达比星、阿糖胞苷)方案化疗后,再次行骨髓穿刺示缓解,但皮损仍有复发.     

髓细胞性白血病皮肤浸润的临床及病理特征

目的 探讨髓细胞性白血病皮肤浸润的临床表现、皮损组织病理及免疫组化特点.方法 分析6例髓细胞性白血病皮肤浸润患者的临床表现、皮损组织病理、免疫组化、实验室检查、治疗及转归.结果 6例中1例以皮肤白血病为首发表现,5例皮肤浸润出现于确诊白血病后的4 ～ 20个月.4例皮疹表现为泛发的红色浸润性丘疹、斑丘疹或结节,2例表现为局部肿物.组织病理示,真皮及皮下脂肪大量肿瘤细胞浸润,免疫组化髓过氧化物酶、CD43、CD45阳性率较高,其他出现阳性的标志物包括CD15、颗粒酶B、白细胞共同抗原、CD68.5例急性髓细胞性白血病均于出现皮肤浸润后60 d至1年内死亡.结论 髓细胞性白血病皮肤浸润一般发生于外周血象与骨髓象改变之后,偶尔也发生于白血病血象和骨髓象改变之前.皮疹常表现为结节或肿物,可局限可泛发,组织病理及免疫组化具有特征性.出现皮肤浸润者生存期短、预后差.     

原发性皮肤CD4+多形性小/中T细胞淋巴瘤1例临床病理观察并文献复习

报告1例原发性皮肤CD4 多形性小/中T细胞淋巴瘤.患者女,45岁.右膝右上方反复红斑、结节15年.组织病理检查示真皮全层及皮下脂肪层弥漫性结节性致密小到中等大淋巴样细胞浸润.细胞有异形,其间混杂少量炎性细胞,无亲表皮现象.免疫组化检查示全T抗原缺失的Th表型.诊断:原发性皮肤CD4 多形性小/中T细胞淋巴瘤.     

皮肤Rosai-Dorfman病临床表现及组织病理学特征分析

目的 探讨皮肤Rosai?Dorfman病（CRDD）的临床表现、皮损形态特征和组织病理学特点。方法 收集20例CRDD患者的基本情况及临床资料,分析其皮损特征并进行分型,同时对皮损进行组织病理学检查和免疫组化染色。结果 20例CRDD患者中,多发皮损11例,单发皮损9例;按累及的解剖部位分单处16例,多处4例,共计24处。皮损表现为丘疹结节型10处（41.67%）、浸润斑块型12处（50.00％）和肿瘤样型2处（8.33％）。20例中6例皮损表现为混合型（丘疹结节型/浸润斑块型5例,浸润斑块型/肿瘤样型1例）。24处组织标本病理表现大致相同,即真皮和（或）皮下脂肪层可见数量不一、体积较大的组织细胞散在或片状分布,伴大量以淋巴细胞和浆细胞为主的炎症细胞浸润,组织细胞胞质内吞噬有数量不一的淋巴细胞、中性粒细胞等。免疫表型均为组织细胞S100阳性、CD68阳性、CD1a阴性。17处皮损病变累及真皮全层,其中13处病变侵入脂肪层;6处病变仅累及真皮浅中层;1处病变仅累及真皮深层及脂肪层。不同形态类型皮损之间的病变累及范围和炎症浸润模式均无明显差异。结论 CRDD临床皮损以丘疹结节型和浸润斑块型为主,肿瘤样型少见。不同形态皮损组织病理均可见数量不等具有伸入运动的组织细胞。S100蛋白、CD68等免疫组化染色有助于CRDD的诊断与鉴别诊断。     

母细胞性浆细胞样树突细胞肿瘤一例

患者男,51岁,全身皮肤散在暗红色斑丘疹、结节1年余。皮损逐渐增多,初发时无明显不适,逐渐出现触痛。体检：头面、躯干、四肢可见散在分布的暗红色斑丘疹、浸润性斑块、皮下结节,边界尚清,部分有触痛,右腹股沟可触及一樱桃大小淋巴结,余浅表淋巴结未触及。骨髓穿刺结果显示,淋巴系统占32.5%,其中幼稚淋巴细胞占10%,此类细胞大小不等,形态多不规则,细胞质量中等,色蓝,核形不规则,核染色质呈颗粒状。皮损组织病理检查：真皮浅层及脂肪组织大量中等大小异型肿瘤细胞弥漫性浸润,肿瘤细胞染色质细颗粒状,稀疏,核仁不明显,核分裂相易见。免疫组化：CD4、CD56、CD43强阳性;CD68及TdT少量细胞阳性;L26、CD3、CD38、颗粒酶B、MPO均阴性。根据临床资料、实验室检查、皮肤组织病理及免疫组化结果,诊断为母细胞性浆细胞样树突细胞肿瘤。     

Induction of skewed Th1/Th2 T-cell differentiation via subcutaneous immunization with Freund's adjuvant

CD4+ T cells differentiate into at least two distinct subsets, Th1 and Th2, that are characterized by their cytokine-producing profiles. In this study, we attempted to delineate whether and how CD4+ T-cell responses could be skewed in one direction or another. BALB/c mice were immunized with chicken ovalbumin (OVA) emulsified with either incomplete or complete Freund's adjuvant (IFA or CFA). When lymph node cells were assessed on day 7, antigen specific proliferation was similarly observed both in the mice immunized with IFA and CFA. In contrast, on day 28 there was a less significant response in the mice primed with IFA than in those primed with CFA. ELISA analyses revealed more Th1 predominant cytokine production by T cells immunized with OVA+CFA rather than in IFA, which resulted in balanced IFN-gamma and IL-4 production. Flow cytometric analyses of intracellular cytokines confirmed that T cells from mice primed with CFA produced Th1 cytokines more predominantly. When lymph node dendritic cells (DC) were compared for their co-stimulatory molecule expression, priming with CFA and IFA similarly upregulated CD80 and CD86 expression by lymph node DC, and no significant differences were observed in CD40, 54, 80 and 86 expression between the DC harvested from IFA and CFA immunized mice. In addition, both priming with IFA and CFA similarly induced IL-12 production by DC. Thus, although the reason(s) for the preferential induction of a Th1/Th2 response remains unknown, these results indicate that a relatively Th1/Th2 skewed response is differentially induced by different types of adjuvants, and induction of a Th1 skewed response may be responsible for long lasting cellular immunity.     

Characterization of human skin-derived CD1a-positive lymph cells

Abstract The phenotype and function of CD1a⁺ lymph cells is of considerable interest. By means of microsurgical lymph cannulation human lymph derived from normal skin was sampled. Cells were isolated and processed for immunocytochemistry, electron microscopy, flow cytometry and functional assays. The majority of the cells, (62%), were T cells. The other cells comprised CD1a⁺ cells (7%), monocytes/macrophages (8%), and B cells (1%); the remainder were erythrocytes or uncharacterized cells. The CD1a⁺ cells reacted with antibodies against protein S-100, HLA-DR, the Lag antigen, CD4, CD11a, CD11b, CD18, CD25, CD40, CD54, CD80 and CD86. Interestingly, a small prolow portion the of CD1a⁺ cells (about 5%) reacted with an antibody to CD14. The CD1a⁺ cells did not react with an antibody against human follicular dendritic cells nor were they CD19-, CD23-, E-cadherin- or factor XIIIa-positive. Both allogenic and antigen-specific T cell proliferation stimulated by antigen-presenting lymph cells were strongly inhibited by adding anti-CD80 and anti-CD86 antibodies. By electron microscopy Birbeck granules were detected in only 22% of the CD1a⁺ lymph cells and these cells exhibited an extensive ruffling of the surface. These findings demonstrate that CD1a⁺ lymph cells, which do not express the dermal dendritic cell marker factor XIIIa, resemble dendritic cells formerly designated as ‘veiled’ as well as lymphoid dendritic cells, suggesting that after migration to the regional lymphoid organs, Langerhans cells form a more differentiated population of dendritic cells specialized in sensitizing T lymphocytes. Our results add further support to the view that resident Langerhans cells may be precursors of lymphoid dendritic cells acquiring the final phenotype in the microenvironment of the lymph node. Received: 30 July 1998 / Received after revision: 30 September 1998 / Accepted: 19 October 1998     

Characterization of vibrissa germinative cells: transition of cell types

Germinative cells, small cell masses attached to the stalks of dermal papillae that are able to differentiate into the hair shaft and inner root sheath, form follicular bulb-like structures when co-cultured with dermal papilla cells. We studied the growth characteristics of germinative cells to determine the cell types in the vibrissa germinative tissue. Germinative tissues, attaching to dermal papillae, were cultured on 3T3 feeder layers. The cultured keratinocytes were harvested and transferred, equally and for two passages, onto lined dermal papilla cells (LDPC) and/or 3T3 feeder layers. The resulting germinative cells were classified into three types in the present experimental condition. Type 1 cells grow very well on either feeder layer, whereas Type 3 cells scarcely grow on either feeder layer. Type 2 cells are very conspicuous and are reversible. They grow well on 3T3 but growth is suppressed on LDPC feeder layers. The Type 2 cells that grow well on 3T3 feeder layers, however, are suppressed when transferred onto LDPC and the Type 2 cells that are suppressed on LDPC begin to grow again on 3T3. The transition of one cell type to another in vitro and the cell types that these germinative cell types correspond to in vivo is discussed. It was concluded that stem cells or their close progenitors reside in the germinative tissues of the vibrissa bulb except at late anagen-early catagen.     

毛乳头细胞与毛发上皮细胞相互作用的研究

目的探讨人毛乳头细胞和毛发上皮细胞间的相互作用,了解毛发生长周期的调控,寻找体内外毛囊重建的途径。方法人毛乳头细胞和毛发上皮细胞分室和共同培养于 DMEM培养基中,在不同时相计数两种细胞,并观察细胞的生长方式。结果毛乳头细胞和毛发上皮细胞的生长能相互促进,毛发上皮细胞能诱导培养的毛乳头细胞形成毛乳头样结构。结论毛囊真－表皮间的作用是双向的,它们共同控制着毛囊的生长和发育。     

干细胞治疗斑秃的研究进展

【摘要】 目前认为，斑秃是由于毛囊免疫赦免被破坏导致。干细胞具有免疫调节功能，可分泌多种细胞因子，促进毛囊免疫赦免。干细胞治疗，尤其是脐带和脂肪来源的干细胞相关疗法已应用于多种脱发的临床前和临床研究，为难治性斑秃提供了治疗新途径。