名词:肿瘤相关性膜性肾病,Membranousnephropathyassociatedwithneoplasms。 膜性肾病是成人肾病综合征的最主要病理形式,也是实体肿瘤相关的最常见副肿瘤性肾小球病(paraneoplasticglomerulopathy)。 膜性肾病(membranousnephropathy,MN)患者中癌的发生率估计为6-22%。 MN伴有的恶性肿瘤常在MN诊断后的12个月内被发现,多数(80%)病例在肾病诊断前或同时发现。 与MN相关的最常见恶性肿瘤为实体瘤,包括:肺癌(肺和支气管)、胃癌;其次为肾癌、前列腺癌和胸腺瘤;乳腺癌、消化道癌(肠癌、胰腺癌、食管癌和肝癌)也有报告,罕见于肉瘤、精原细胞瘤、腮腺腺淋巴瘤、肾上腺节细胞神经瘤、脊柱神经鞘瘤、颈动脉体瘤。 Fig.肺癌合并的MN。 Lightmicroscopyfindingsinapatientwhodevelopedmembranousnephropathyinthesettingofbronchogeniccarcinoma,showinginflammatorycellsinfiltratingtheglomeruli(A)(Massontrichromestain)and,byimmunofluorescence,typicalsubepithelialdeposits(B)(immunofluorescencewithanti-gantibody). Fig.慢性淋巴细胞白血病(CLL)合并的MN Asecondpatientfollowedforchroniclymphocyticleukemia(CLL)developedproliferativeGNwithnonorganizedmonoclonalimmunoglobulindepositsdefinedbyimmunedepositsontheexternalaspectoftheglomerularbasementmembranewithfrequentmesangialhypertrophyonlightmicroscopy(C)(Massontrichromestain);thedepositshaveirregularsize(insetofC),haveaparietalgranulardistributionbyimmunofluorescence(D)(immunofluorescencewithanti-gantibody),andarenonorganizedinthesubepithelialspaceonultrastructuralstudy(E). Fig.另一例慢性淋巴细胞白血病(CLL)合并的MN ThekidneybiopsyspecimenofanotherpatientwithCLLshowsatypicalmembranousnephropathy:notesegmentalmesangialandcellularproliferation(F)(Massontrichromestain).Diffusegranulardepositswerefoundalongthecapillarywallswithpredominantsubepitheliallocation,stainingexclusivelyforIgGandklightchainbyimmunofluorescence(H)(immunofluorescencewithanti-kantibodyontheleftpanel;intheright,notetheabsenceofdepositswithanti-lantibody)andshowingmicrotubularsubstructureonultrastructuralstudies,asdescribedpreviouslyinimmunotactoidglomerulopathy(G). : 1.肿瘤经手术、化疗或其他治疗全部移除后,肾病缓解; 2.肿瘤复发时肾病复发; 3.存在肿瘤和MN的病理生理学联系,包括在上皮下免疫沉积物中检测到肿瘤抗原(如CEA、PSA)和抗肿瘤抗体。 A.原位免疫复合物(insituimmune北京中科白癜风医院哈尔滨治疗白癜风的医院
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