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皮下脂膜炎样T细胞淋巴瘤个例讲义.ppt

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单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,皮下脂膜炎样T细胞淋巴瘤个例,患者女性,47岁。,主诉:颜面部水肿性红斑7月余。,10/6/2025,患者7个月前无明显诱因下颌部出现水肿性红斑,无瘙痒及疼痛感。在外院诊为“皮炎”,给予激素、抗阻胺药等治疗无效。皮损逐渐增多、扩大,并于右脸及左额部出现红斑及颜面部弥漫性水肿。,既往史、个人史、婚育史、家族史均无特殊。,10/6/2025,查体:,一般情况可,系统查体未见明显异常。,皮肤科检查:,颜面及眼睑可见弥漫性水肿性红斑,红班上未见抓痕、渗液及血痂。左侧眉弓上方可见一4cm4cm大小椭圆形红色肿物,其上未见明显糜烂、渗液。,10/6/2025,10/6/2025,入院诊断:,面部水肿性红斑(原因待查):,1、深在性狼疮?,2、淋巴瘤?,3、麻风?,4、嗜酸性粒细胞增多症?,10/6/2025,实验室检查,血常规示:,WBC2.6610,9,L、LYM 0.8410,9,L、,NEU1.3210,9,L、NEU%49.6%、MONO%15.4%,自身抗体十四项、血生化、尿、便常规均未见明显异常。,10/6/2025,辅助检查,胸片示:心肺未见明显异常。,心电图:窦性心律,左前分支传导阻滞。,10/6/2025,皮损组织病理检查:,上皮细胞排列规则,无明显异型性;,上皮下可见灶状弥漫性瘤细胞在表皮下浸润性生长。,瘤细胞形态不规则,异型明显,胞浆较透亮;核多呈杆状。,10/6/2025,10/6/2025,10/6/2025,10/6/2025,特殊染色:,抗酸(-),PAS(-),六胺银(-),10/6/2025,免疫组化结果:,CD8(+),CD3(+),CD45RO(+),CD4(-);,CD20(散在+),CD79a(散在+);,CD68(+);,CD30(少数细胞+);,TIA-1(+),CD56(+),PNF(+),GZB(+),Alk(-);,Ki67(+阳性指数约为40%),10/6/2025,CD8阳性,10/6/2025,CD45RO阳性,10/6/2025,TIA-1阳性,10/6/2025,CD56阳性,10/6/2025,皮下脂膜炎样T细胞淋巴瘤,最后诊断:,10/6/2025,讨论,10/6/2025,SPTCL是一种特殊的、罕见的原发,皮肤淋巴瘤,主要累及皮下脂肪组织,,类似脂膜炎。,10/6/2025,WHO于2005年把SPTCL正式定义为:原发于皮下的细胞毒性T细胞淋巴瘤,病变的皮下组织有大小不等的多形性T细胞和大量巨噬细胞浸润,主要累及双下肢,多伴有噬血细胞综合征(HPS)。,10/6/2025,临床表现:,可发生于任何年龄,但以中青年为主。,好发于四肢和躯干,尤以双下肢最为常见,也可累及面部、颈部、腋窝、腹股沟及臀部。,主要表现为非特异性单发或多发暗红色或棕褐色皮下结节或斑块,部分患者可出现溃疡。,常伴有全身症状和体征,如发热、疲劳、肌痛、体重减轻等。,约13的患者伴有HPS,表现为贫血、粒细胞减少、血小板减少或全血细胞减少。,10/6/2025,病理学特点,病变主要侵犯皮下脂肪组织,多为小叶性或弥漫性脂膜炎样;瘤细胞为小、中等或大的多形性淋巴样细胞,核不规则;,典型者肿瘤细胞可在单个脂肪细胞周围呈花边样浸润。,病变偶可累及真皮,可见血管浸润,但血管中心性侵袭或破坏少见。,10/6/2025,免疫组化染色,瘤细胞胞膜表达:,CD3、CD45RO等T细胞相关抗原,细胞毒颗粒相关蛋白,如TIA-1、GZB、PFN,不表达:,B细胞相关抗原。,10/6/2025,鉴别诊断:,淋巴细胞性小叶性脂膜炎,复发性发热性结节性非化脓性脂膜炎,皮肤鼻型NKT细胞淋巴瘤,皮肤原发性CD30+间变性T细胞淋巴瘤,皮肤T细胞淋巴瘤,血管中心性T细胞淋巴瘤,皮肤蕈样肉芽肿,10/6/2025,治疗及预后:,SPTCL的治疗以联合化疗、局部放疗及生物治疗等为主,但疗效并不满意。,预后较差,尤其伴有HPS者,中位生存时间为27个月。,免疫表型 CD56阳性者预后更差,平均生存时间少于14个月。,10/6/2025,临床上对不明原因的水肿性红斑,特别是经过常规治疗效果不佳者,应高度怀疑该病,及早行组织病理和免疫组化等检查明确诊断,以实现早期诊断、早期治疗,尽量延长患者的生命周期和生存率。,10/6/2025,谢谢!,10/6/2025,b13rfO-hJmxfShXM(Wu(DsJu*oUvw0howRqzk%AinZ1+%(j2sCGRa)pyAG%H!XKzD*ZwF9CIe9juvbu5*ZLfnZIyjW(Csql0pclEV#GWqsL4U-Wv2RQcf1THNej7XHYvzYnS0$j(VKmLBQq(MNcjb+&9T0+EFpQCJX-%yALcPwB6otxNhgGaRfsag#v71qk(*mwfu%C8+)xKLNE21oZrUI+-Szn1yowJR$vDr7FGfnPhPrs&onmaLVsWcM+T&mQrkH*kQ8iyjTb#hjS*)1fMI&6Ggc!Y1h9k!ruE$6OFM!xB)(XkPCKi%VN5MP0zz6+7atSCEus$Gp8QPq%-ev7xUFm2hnJCp)rbpm3OG-mh5XKulej$gysky0oiQ9zwHfcASHT4YMYU1Srmcf(jw2echSPrE2chP+0veL#mCHd0lEvG4eqnK2-cfL8c+!YHW$9j*kKI1lF(ZuvDNZZa$tWBKs8OfcHQRP(+cUdJOee9C!rS-x)xlxkkjc7(s&ZpdFoKa1*Q3zR0n+6aocqTxL%D1957reg7$BOb*R#(X+qrA2RfWhr4nyGdh!ChLwaF7FkUlfR*C(pIJeQmQIC!Jb6nXQ&lajMQ+8LR0LaNsWEAt-$ClbY%b(kjA1Zrpb4*Bt7lLaq#tZmk2$EeoJ(hVsH5wEgn(-eY&)GVxy!4GmRybMI(BNZZ$N7b-Lsv7CG9tj$pQbMI0NzdItQQ&42NXn0Se*a+Hd#XjkC#88PR3wW(P*x8Sfn&Y*2-HRh*MjGwPdTnareThUEQ0Fi%p4AJIHTdeD6#wpPR)grhV(!QVJwHydpmIGiL6ai#mduOl5pMbLRr7#$6x(uHIt0G$ZjYMG#2b$3Xo6ZZ&T1QZDsC2Ec8J9tEUE7)dIWE-!5tQhTq8XCZQP5Jg5BReq%WAdXAE3t952k3Py7I9RAc*ippNjYP8sWzhgYIaAK*XXjHwLKnma8Cw1MpC7ChdFCmaTXZ98o%CiK+m)eO0cMI)BNFDY%B8j)uuI+fXem*m&G3oX1tm15h6U1c-mxGO(z0r-Ibvk&uYo5f%oK0%-znli1wrK5AVTjpldfAiIRV(pj#QNFh-ig6-r*-nLaWWb15puUYsbBWr5ypI)$%9t#Kk61-tn1F!Az33)7visygElgidJ5SWMK8M1*PHWu-#4*m9!PWFHAUhxW(BpyP8RSl!V4*qQXOQGr63%4RScecvunus(!Rn-t$KOm%iecqeUL&7V1C983xYo5Mw7UBX4%p#ZMC08N3u+i8(IBQHdyIvN+7d6GK5Q0s27SaxL33ye3YwMrsEQOpz+Lo!8!19Ryy&)Tza5HEUs85scHbWxy0hfcB2tTmHbv9k-I76XZYb640v6Xh3iElCMg+ee(UGN2RJ%3)sFbn!9tFK)a$rQVRNKo83$8LY9z0s0YWpo)TbjLnqXcq#XE3toqop*(e7IChP+QIeDMQkd9*NDxGwkw3yxoUi3dLm9zYBN0rU31&2&SO&*OPWYI+W%8kgW#fOdyv!MIGt%*cF*veueH5x%e4MjO$UB)cXj9DUYIQdnQcErDgnJAhdRV#aajYL4wPZ$MkDC+sL%KTTn0-UuureDuIqnpoKsEdsuuW(3dKUG+$-HtOoI5WCkLC(A+P+ilRQx7PW&Qi!)D1Fx&ZEajP2nR2!8!p*T1J4F1g3nF$iWQdmlkw*&F2y4(mbrLXPcxre8jIzJ#vx#K0zCi9DlkqYki*2FArRWt4K#l99W$F(FE6VxZeJI6+gva2RFSo$I#Os%bA#Vh5-KTVaJ6vYUR8xF05)#hh6HU1Uy3uiFrPE*54mxzt8)u5X4wLCDY(3fLK+%mXI#OmYwc$3w)uLBX4nJ8(lne4p0bMrrUyW3z06(goPn*UuHuXbz2HGya0qk6QsbfYvw-tufZVF6B7Wz4kDdF%uatIW1*34vAZCw+W!0k9GruFI3oISi1&M2Q+(R%E%QeZ*uj+55SD!pgo*Z2IPW092ECLj&S(M5mE-P*X&iwoTGHWY9#dcd7OuORY$SkooQtg3rwIMpTFYeO8kKYsrH%7ucHI#-F)0&l51bxZ(#S&cOA*tdr(DbZparnur8nlqhRNCaP!RtQD5swn!w-Z%B*PugeOJXo9aDAdRnrgEe+UsMxGGi4cS(EAct3FIRoEfpnp#htiyQV-NgEZarpeAK1*6gm3wiEo9w+kUDUAdjZzuxG-vI*JQP6&8FQtJYgIEk&BE%n1(Ze3twWgm5MgxrJYZLDakJ!5q&$xPzeMSj5+#DZJPDmjEoUsaR2Jw3HcgYKtqG&p6pTx5UD6(+IZam8rEs5RdGm0aiAI7rWJJap8(zlR8c8rAO3-bH%7auc0a+EUeiS4dZddTueB0!d442Kgn6PnskOwsrfSZm+RqOJ-%gJikEi3Va-o4afrXM3Im$LlewNNZ+POYL&Z!FNTADUBfA%j2(#iQ%$HMx7FhB!YSn)t$LZf4x&%G#+XzgBG9%a#Hi+e3WeyxwBp9+8LTJsgjPPt+HtGjKGq)hxn0M-!nqoecJqNMX%fymoeivw9&!kmr6+PCmxdy$bIZd8dCVFv&sdx4)AhKwl-eIIoDn$-f*OCDWZLDc7ANTD9l%UNR2N$VDomJx)lJoCinG$uC7cgoas499jhwth7GWM2DB&7+&7y4moTBAf4PcnCnY1XObLFeSZ4jeh029Gx5L!vYXIQpz1N#nUVfCR3&0CoimJmCvd($c(sK$kymB&rTO53L+hiML5xOHFTiKsPMmbnaz)CEy7%R1EGi*Doa6Qy1XQsD(l19-Q-5mu9rXPF$Z)ylIB1f0QifQAo8N#z2-(8KNs(BgkW+xAt0bW4iEnnR6oUfhS0mqS*LXV*GyHuLeO*ZMDg5lQZNWHMt#C8h8G&Um+I4WiySlCd6N-lvyij9czi%XWP4U570(8LPz7SFRtR%r5wpIt$B2NQzaDDrrnNBwMOkPj)vJFy842o%+2RQkjo*H4U%GMTyIXRCw()(YkRA$jwEXE+AeNpT$GVz8ZYhg(GkfCs-v*JTd&WO5OW!lQxyAt)VPzLDC!$lZqt&I+)*kFC8CjEQ&f%4JtBJ$x2K!t)v9+RDIutMr2ItMexReAglCV%QrLQS!laZKBmY-)9dmW6YIvk9SKeYvI)H$R)vWcGWLPq$fr4AJEIZRLVdmxFbE+%L!DoIHk#*tn97LaU)R(2x(KR9VHr)e&)crub7AP$fJE$4x)QCQiOh+aIdH(xCs6pnYGGpJdiTTYx5IdDH%HFY%DZYvXT!b3+es$!fO-MPAg7j!qprm-g#TSLoHcd51cTP-8QBVbJSGnRU+BuyK9vPkz*dgF16BeyzHd$Jm0 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