{"id":55538,"date":"2014-11-20T07:55:13","date_gmt":"2014-11-20T12:55:13","guid":{"rendered":"https:\/\/www.erdheim-chester.org\/%e6%b2%bb%e7%99%82\/"},"modified":"2025-05-12T14:41:55","modified_gmt":"2025-05-12T18:41:55","slug":"%e6%b2%bb%e7%99%82","status":"publish","type":"page","link":"https:\/\/www.erdheim-chester.org\/zh-hant\/%e6%b2%bb%e7%99%82\/","title":{"rendered":"\u6cbb\u7642"},"content":{"rendered":"<p>[et_pb_section fb_built=&#8221;1&#8243; _builder_version=&#8221;4.27.4&#8243; _module_preset=&#8221;default&#8221; background_image=&#8221;https:\/\/www.erdheim-chester.org\/wp-content\/uploads\/2024\/12\/Untitled-design-96-1.png&#8221; background_position=&#8221;bottom_center&#8221; background_vertical_offset=&#8221;20%&#8221; custom_padding=&#8221;20px||20px||true|false&#8221; background_last_edited=&#8221;on|desktop&#8221; background_position_tablet=&#8221;bottom_right&#8221; background_position_phone=&#8221;center_right&#8221; global_module=&#8221;28071&#8243; 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_module_preset=&#8221;default&#8221; custom_margin=&#8221;||0px||false|false&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_image][et_pb_button button_url=&#8221;https:\/\/www.erdheim-chester.org\/wp-content\/uploads\/2025\/02\/Treatment-list-graphic-for-website.pdf&#8221; url_new_window=&#8221;on&#8221; button_text=&#8221;\u4e0b\u8f09 PDF&#8221; module_class=&#8221;full-button&#8221; _builder_version=&#8221;4.27.4&#8243; _module_preset=&#8221;default&#8221; custom_padding=&#8221;10px||10px||true|false&#8221; global_colors_info=&#8221;{}&#8221;][\/et_pb_button][\/et_pb_column][et_pb_column type=&#8221;3_5&#8243; _builder_version=&#8221;4.27.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text _builder_version=&#8221;4.27.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<h3>\u6cbb\u7642\u6c7a\u7b56<\/h3>\n<p>\u6c7a\u5b9a ECD \u7684\u9069\u7576\u6cbb\u7642\u65b9\u6cd5\u6d89\u53ca\u60a3\u8005\u548c\u91ab\u751f\u4e4b\u9593\u7684\u5408\u4f5c\u3002\u60a3\u8005\u7684\u6cbb\u7642\u53ef\u80fd\u56e0\u570b\u5bb6\u548c\u5f9e\u696d\u8005\u7684\u7d93\u9a57\u800c\u7570\u3002\u7136\u800c\uff0c\u5927\u591a\u6578\u81e8\u5e8a\u91ab\u751f\u4f9d\u8cf4\u65bc <em>Blood<\/em> \u4e0a\u767c\u8868\u7684\u5171\u8b58\u5efa\u8b70\u6216\u570b\u5bb6\u7d9c\u5408\u764c\u75c7\u7db2\u8def \u00ae \uff08NCCN\uff09 \u00ae \u7684\u5171\u8b58\u5efa\u8b70\u3002\u9019\u4e9b\u5efa\u8b70\u6307\u51fa\uff0c\u6cbb\u7642\u53d6\u6c7a\u65bc\u75be\u75c5\u8ca0\u64d4\u3001\u7a81\u8b8a\u72c0\u614b\u3001\u85e5\u7269\u53ef\u7528\u6027\u548c\u5f9e\u696d\u7d93\u9a57\u3002   <\/p>\n<h3>\u9078\u64c7\u6cbb\u7642<\/h3>\n<p>\u5e78\u904b\u7684\u662f\uff0c ECD \u60a3\u8005\u6709\u6cbb\u7642\u9078\u64c7\uff0c\u5176\u4e2d\u4e00\u4e9b\u5df2\u88ab\u8b49\u660e\u5c0d\u5927\u591a\u6578\u60a3\u8005\u975e\u5e38\u6709\u6548\u3002\u5c11\u6578\u4e0d\u6d89\u53ca\u4e3b\u8981\u5668\u5b98\u7684\u7121\u7665\u72c0\u75be\u75c5\u60a3\u8005\u53ef\u80fd\u6703\u9032\u5165\u89c0\u5bdf\u548c\u7b49\u5f85\u671f\uff0c\u7121\u9700\u6cbb\u7642\u3002\u4f46\u662f\uff0c\u901a\u5e38\u5efa\u8b70\u9032\u884c\u6cbb\u7642\uff0c\u5c24\u5176\u662f\u5c0d\u65bc\u95dc\u9375\u5668\u5b98\u53d7\u7d2f\u7684\u60a3\u8005\uff0c\u4f8b\u5982\u5fc3\u81df\u6216\u4e2d\u6a1e\u795e\u7d93\u7cfb\u7d71\u3002  <\/p>\n<h3>\u6cbb\u7642\u8207\u6cbb\u7652<\/h3>\n<p>\u5118\u7ba1\u6709\u91dd\u5c0d ECD\u7684\u6709\u6548\u6cbb\u7642\u65b9\u6cd5\uff0c\u4f46\u5c1a\u672a\u627e\u5230\u6cbb\u7652\u65b9\u6cd5\u3002\u7576\u4eca\u53ef\u7528\u7684\u6700\u4f73\u6cbb\u7642\u65b9\u6cd5\u7a31\u70ba\u9776\u5411\u6cbb\u7642\uff0c\u901a\u5e38\u53ef\u4ee5\u6210\u529f\u63a7\u5236\u4e26\u7d93\u5e38\u7e2e\u5c0f ECD \u75c5\u8b8a\u3002\u76ee\u524d\uff0c\u9019\u4e9b\u6cbb\u7642\u901a\u5e38\u5fc5\u9808\u7121\u9650\u671f\u5730\u6301\u7e8c\uff0c\u5426\u5247\u75be\u75c5\u5f88\u53ef\u80fd\u6703\u518d\u6b21\u958b\u59cb\u767c\u5c55\u3002\u7136\u800c\uff0c\u901a\u904e\u6210\u529f\u7684\u6cbb\u7642\uff0c ECD \u901a\u5e38\u53ef\u4ee5\u88ab\u63a7\u5236\u70ba\u4e00\u7a2e\u6162\u6027\u75be\u75c5\u3002[\/et_pb_text][\/et_pb_column][\/et_pb_row][et_pb_row _builder_version=&#8221;4.27.4&#8243; _module_preset=&#8221;default&#8221; custom_padding=&#8221;||1px|||&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;4_4&#8243; _builder_version=&#8221;4.27.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text admin_label=&#8221;Text&#8221; _builder_version=&#8221;4.27.4&#8243; background_size=&#8221;initial&#8221; background_position=&#8221;top_left&#8221; background_repeat=&#8221;repeat&#8221; global_colors_info=&#8221;{}&#8221;]<\/p>\n<h3>\u6cbb\u7642\u985e\u578b<\/h3>\n<p>\u5178\u578b\u7684 ECD \u6cbb\u7642\u5206\u70ba\u4ee5\u4e0b\u4e09\u985e\u4e4b\u4e00\uff1a\uff081\uff09 \u9776\u5411\u6cbb\u7642\uff0c\uff082\uff09 \u5316\u7642\uff0c\u6216 \uff083\uff09 \u514d\u75ab\u7642\u6cd5\u3002\u666e\u904d\u8a8d\u70ba\uff0c\u5982\u679c\u60a3\u8005\u6240\u5728\u570b\u5bb6\/\u5730\u5340\u6709\u9776\u5411\u6cbb\u7642\uff0c\u61c9\u5411\u60a3\u6709\u795e\u7d93\u7cfb\u7d71\u6216\u5fc3\u81df\u75c5\u7684\u60a3\u8005\u63d0\u4f9b\u9776\u5411\u6cbb\u7642\u3002\u5728\u5176\u4ed6\u60c5\u6cc1\u4e0b\uff0c\u8003\u616e\u548c\/\u6216\u63a8\u85a6\u4ee5\u4e0b\u6cbb\u7642\u65b9\u6cd5\uff1a  <\/p>\n<ul>\n<li>\u514d\u75ab\u8abf\u7bc0\u6cbb\u7642\n<ul>\n<li>\u5e72\u64fe\u7d20<\/li>\n<li>mTOR \u6291\u88fd\u5291\u6216\u897f\u7f85\u83ab\u53f8<\/li>\n<li>\u7d30\u80de\u56e0\u6578\u6291\u88fd\u5291\uff08\u963f\u90a3\u767d\u6eef\u7d20\uff09<\/li>\n<\/ul>\n<\/li>\n<li>\u5316\u5b78\u7642\u6cd5<\/li>\n<\/ul>\n<p>\u61c9\u6839\u64da\u60a3\u8005\u7684\u7665\u72c0\u548c\u5408\u4f75\u75c7\u8207\u91ab\u751f\u8a0e\u8ad6\u9019\u4e9b\u6cbb\u7642\u65b9\u6cd5\u3002<\/p>\n<h3>\u9776\u5411\u6cbb\u7642<\/h3>\n<p>\u9019\u4e9b\u662f\u53ef\u7528\u65bc ECD\u7684\u6700\u65b0\u4e14\u901a\u5e38\u6700\u6709\u524d\u9014\u7684\u6cbb\u7642\u65b9\u6cd5\u3002\u5b83\u5011\u901a\u5e38\u6703\u963b\u6b62\u5927\u591a\u6578\u5668\u5b98\u4e2d ECD \u7684\u9032\u5c55\uff0c\u4f46\u5fc5\u9808\u975e\u5e38\u4ed4\u7d30\u5730\u89c0\u5bdf\u5927\u8166\u53d7\u7d2f\u60c5\u6cc1\u3002\u5c0d\u65bc\u4e00\u4e9b\u60a3\u8005\u4f86\u8aaa\uff0c\u9776\u5411\u6cbb\u7642\u53ef\u80fd\u96e3\u4ee5\u7a7f\u904e\u8840\u6db2\/\u8166\u5c4f\u969c\uff0c\u5f9e\u800c\u964d\u4f4e\u4e86\u5b83\u5011\u5728\u963b\u6b62\u5927\u8166\u4e2d ECD \u9032\u5c55\u7684\u6709\u6548\u6027\u3002\u9776\u5411\u6cbb\u7642\u5f88\u6602\u8cb4\uff0c\u4e26\u4e14\u5728\u4e16\u754c\u67d0\u4e9b\u5730\u5340\u53ef\u80fd\u96e3\u4ee5\u7372\u5f97\u3002   <\/p>\n<p>ECD \u4e2d\u7684\u9776\u5411\u6cbb\u7642\u963b\u65b7\u7d44\u6210\u578b\u555f\u52d5 MAP \u6fc0\u9176\u901a\u8def\u7d1a\u806f\u53cd\u61c9\uff0c\u89e3\u6c7a ECD \u60a3\u8005\u4e2d\u5b58\u5728\u7684\u5404\u7a2e MAPK \u901a\u8def\u7a81\u8b8a\u3002\u9776\u5411\u85e5\u7269\u7684\u9078\u64c7\u901a\u5e38\u7531\u57fa\u65bc\u57fa\u56e0\u6aa2\u6e2c\u7684 ECD \u7d44\u7e54\u4e2d\u5b58\u5728\u7684\u7a81\u8b8a\u6c7a\u5b9a\u3002 <\/p>\n<ul>\n<li><strong>BRAF V600E \u6b63 ECD<\/strong><br \/>\u5927\u7d04 50% \u7684\u60a3\u8005 BRAF V600E \u7a81\u8b8a\u6aa2\u6e2c\u5448\u967d\u6027\u3002\u9019\u4e9b\u60a3\u8005\u7684\u6cbb\u7642\u9078\u64c7\u901a\u5e38\u5305\u62ec BRAF \u6291\u88fd\u5291\u3001MEK \u6291\u88fd\u5291\u6216\u5169\u8005\u7684\u7d44\u5408\uff08\u5728\u6975\u5c11\u6578\u60c5\u6cc1\u4e0b\uff09\u3002\u5e38\u7528\u7684 BRAF \u6291\u88fd\u5291\u5305\u62ec vemurafenib\u3001dabrafenib \u548c encorafenib\u3002\u5e38\u7528\u7684MEK\u6291\u88fd\u5291\u5305\u62eccobimetinib\u3001trametinib\u3001binimetinib\u6216 selumetinib\u3002\u5728\u7f8e\u570b\uff0cvemurafenib \u548ccobimetinib\u662f FDA \u6279\u51c6\u7684 ECD\u6cbb\u7642\u65b9\u6cd5\u3002    <\/li>\n<li><strong>BRAF V600E \u8ca0 ECD<\/strong><br \/>\u5927\u591a\u6578 BRAF V600E \u7a81\u8b8a\u6aa2\u6e2c\u5448\u9670\u6027\u7684\u60a3\u8005\u5728\u6240\u8b02\u7684 MAPK \u901a\u8def\uff08\u4e5f\u7a31\u70ba Ras-Raf-MEK-ERK \u901a\u8def\uff09\u4e2d\u6703\u51fa\u73fe\u4e0d\u540c\u7684\u7a81\u8b8a\u3002\u8a72\u901a\u8def\u662f\u7d30\u80de\u4e2d\u7684\u4e00\u689d\u86cb\u767d\u8cea\u93c8\uff0c\u5b83\u5c07\u4fe1\u865f\u5f9e\u7d30\u80de\u8868\u9762\u7684\u53d7\u9ad4\u50b3\u905e\u5230\u7d30\u80de\u6838\u4e2d\u7684DNA\u3002\u9019\u4e9b\u4fe1\u865f\u63a7\u5236\u7d30\u80de\u7684\u589e\u6b96\u3001\u5206\u5316\u3001\u767c\u80b2\u3001\u708e\u75c7\u53cd\u61c9\u548c\u7a0b\u5e8f\u6027\u7d30\u80de\u6b7b\u4ea1\u3002MEK \u6291\u88fd\u5291\u901a\u5e38\u8003\u616e\u7528\u65bc\u6240\u6709 BRAF V600E \u6aa2\u6e2c\u672a\u5448\u967d\u6027\u7684\u60a3\u8005\uff0c\u5305\u62ec\u4efb\u4f55\u6e2c\u5e8f\u5747\u672a\u767c\u73fe\u7a81\u8b8a\u7684\u60a3\u8005\u3002\u5e38\u7528\u7684MEK\u6291\u88fd\u5291\u5305\u62eccobimetinib\u3001trametinib\u3001binimetinib\u6216 selumetinib\u3002\u5728\u7f8e\u570b\uff0ccobimetinib \u662f FDA \u6279\u51c6\u7684 ECD \u6cbb\u7642\u65b9\u6cd5\u3002     <\/li>\n<li><strong>\u7f55\u898b\u75c5\u7684\u7f55\u898b\u5be6\u4f8b<\/strong><br \/>\u6975\u5c11\u6578\u60c5\u6cc1\u4e0b\uff0c ECD \u60a3\u8005\u53ef\u80fd\u6709\u5176\u4ed6\u907a\u50b3\u7570\u5e38\uff0c\u4e26\u4e14\u5c0dMEK\u6291\u88fd\u5291\u6c92\u6709\u53cd\u61c9\u3002\u76ee\u524d\uff0c\u5728\u6975\u5c0f\u6bd4\u4f8b\u7684 ECD \u60a3\u8005\u4e2d\u767c\u73fe\u4e86\u4ee5\u4e0b\u7a81\u8b8a\uff0c\u800c\u6240\u5217\u76f8\u61c9\u6cbb\u7642\u7684\u5831\u544a\u6709\u9650\u3002   \n<table id=\"tablepress-2\" class=\"tablepress tablepress-id-2\">\n<thead>\n<tr class=\"row-1\">\n\t<th class=\"column-1\"><strong>Discovered Mutation in the ECD Lesion<\/strong><\/th><th class=\"column-2\"><strong>Possible Therapy<\/strong><\/th>\n<\/tr>\n<\/thead>\n<tbody class=\"row-striping row-hover\">\n<tr class=\"row-2\">\n\t<td class=\"column-1\">ALK fusion<\/td><td class=\"column-2\">Crizotinib<\/td>\n<\/tr>\n<tr class=\"row-3\">\n\t<td class=\"column-1\">CSF1R mutation<\/td><td class=\"column-2\">Pexidartinib<\/td>\n<\/tr>\n<tr class=\"row-4\">\n\t<td class=\"column-1\">NTRK gene fusion<\/td><td class=\"column-2\">Larotrectinib<\/td>\n<\/tr>\n<tr class=\"row-5\">\n\t<td class=\"column-1\">NTRK gene fusion<\/td><td class=\"column-2\">Entrectinib<\/td>\n<\/tr>\n<tr class=\"row-6\">\n\t<td class=\"column-1\">PIK3CA mutation<\/td><td class=\"column-2\">Sirolimus<\/td>\n<\/tr>\n<tr class=\"row-7\">\n\t<td class=\"column-1\">RET fusion<\/td><td class=\"column-2\">Selpercatinib<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<!-- #tablepress-2 from cache --><\/li>\n<li><strong>\u5176\u4ed6\u9776\u5411\u6cbb\u7642<\/strong><br \/>\u5728\u77ad\u89e3 ECD \u4e2d\u89c0\u5bdf\u5230\u7684\u57fa\u56e0\u7a81\u8b8a\u4e4b\u524d\uff0c\u4f7f\u7528\u4e86\u5176\u4ed6\u9776\u5411\u6cbb\u7642\uff0c\u4f46\u6c92\u6709\u4e0a\u8ff0\u6cbb\u7642\u4e2d\u5e38\u898b\u7684\u986f\u8457\u826f\u597d\u6548\u679c\u3002\u4e00\u4e9b\u65e9\u671f\u7528\u65bc ECD \u7684\u9776\u5411\u6cbb\u7642\u5305\u62ec\u4f0a\u99ac\u66ff\u5c3c\uff08\u916a\u6c28\u9178\u6291\u88fd\u5291\uff09\u548c\u7d22\u62c9\u975e\u5c3c\uff08\u6fc0\u9176\u6291\u88fd\u5291\uff09\u3002 <\/li>\n<li><strong>\u9776\u5411\u6cbb\u7642\u7684\u5291\u91cf<br \/><\/strong>\u9700\u8981\u6ce8\u610f\u7684\u662f\uff0c\u5927\u591a\u6578 ECD \u60a3\u8005\u901a\u5e38\u53ea\u80fd\u8010\u53d7\u4f4e\u5291\u91cf\u7684\u9776\u5411\u6cbb\u7642\u85e5\u7269\u3002\u56e0\u6b64\uff0c\u70ba ECD \u60a3\u8005\u958b\u5177\u7684\u7d66\u85e5\u5291\u91cf\u901a\u5e38\u4f4e\u65bc\u7528\u65bc\u6cbb\u7642\u5176\u4ed6\u75be\u75c5\u7684\u5291\u91cf\u3002\u76ee\u524d\uff0c\u5118\u7ba1\u9019\u4e9b\u6cbb\u7642\u901a\u5e38\u5fc5\u9808\u7121\u9650\u671f\u5730\u6301\u7e8c\uff0c\u4f46\u4e00\u65e6\u60a3\u8005\u7684\u75be\u75c5\u5728\u6cbb\u7642\u4e2d\u5b8c\u5168\u7a69\u5b9a\u4e0b\u4f86\uff0c\u901a\u5e38\u53ef\u4ee5\u9032\u4e00\u6b65\u6e1b\u5c11\u5291\u91cf\u3002\u5728\u505a\u51fa\u6539\u8b8a\u4e4b\u524d\uff0c\u60a3\u8005\u61c9\u59cb\u7d42\u8207\u91ab\u751f\u8a0e\u8ad6\u4efb\u4f55\u5efa\u8b70\u7684\u6cbb\u7642\u5291\u91cf\u66f4\u6539\u3002   <\/li>\n<li><strong>\u6cbb\u7642\u5f62\u5f0f<\/strong><br \/>\u5728\u5927\u591a\u6578\u60c5\u6cc1\u4e0b\uff0c\u6240\u6709\u9776\u5411\u6cbb\u7642\u90fd\u4ee5\u85e5\u4e38\u5f62\u5f0f\u7d66\u846f\uff0c\u6bcf\u5929\u4e00\u6b21\u6216\u5169\u6b21\u3002<\/li>\n<li><strong>\u526f\u4f5c\u7528<\/strong><br \/>\u9776\u5411\u6cbb\u7642\u7684\u526f\u4f5c\u7528\u5fc5\u9808\u901a\u904e\u60a3\u8005\u8207\u5176\u91ab\u7642\u5718\u968a\u4e4b\u9593\u7684\u5408\u4f5c\u9032\u884c\u7ba1\u7406\u3002\u60a3\u8005\u5728\u958b\u59cb\u4efb\u4f55\u6cbb\u7642\u6642\u61c9\u59cb\u7d42\u5411\u4ed6\u5011\u7684\u91ab\u7642\u5718\u968a\u8a62\u554f\u53ef\u80fd\u7684\u526f\u4f5c\u7528\u3002BRAF \u6291\u88fd\u5291\u7684\u5e38\u898b\u526f\u4f5c\u7528\u5305\u62ec\u76ae\u75b9\u3001\u95dc\u7bc0\u75db\u548c\u76ae\u819a\u5c0d\u967d\u5149\u654f\u611f\u3002MEK \u6291\u88fd\u5291\u7684\u5e38\u898b\u526f\u4f5c\u7528\u5305\u62ec\u76ae\u75b9\u3001\u8179\u7009\u548c\u817f\u90e8\u816b\u8139\u3002BRAF \u6291\u88fd\u5291\u7684\u7f55\u898b\u526f\u4f5c\u7528\u5305\u62ec\u76ae\u819a\u764c\u6216\u5fc3\u5f8b\u554f\u984c\uff0c\u800cMEK\u6291\u88fd\u5291\u7684\u526f\u4f5c\u7528\u5305\u62ec\u8996\u529b\u969c\u7919\u548c\u5fc3\u529b\u8870\u7aed\u3002    <\/li>\n<li><strong>\u6cbb\u7642\u8cbb\u7528<\/strong><br \/>\u9776\u5411\u6cbb\u7642\u7684\u6210\u672c\u6709\u6642\u53ef\u80fd\u975e\u5e38\u9ad8\u3002\u5728\u4e00\u4e9b\u570b\u5bb6\uff0c\u4fdd\u96aa\u548c\u653f\u5e9c\u4ed8\u6b3e\u4eba\u5c07\u627f\u64d4\u5927\u90e8\u5206\u6cbb\u7642\u8cbb\u7528\u3002\u5728\u67d0\u4e9b\u5730\u65b9\uff0c\u53ef\u80fd\u6709\u4e00\u4e9b\u7d44\u7e54\u53ef\u4ee5\u8aac\u660e\u652f\u4ed8\u5269\u9918\u7684\u6cbb\u7642\u8cbb\u7528\uff0c\u5177\u9ad4\u53d6\u6c7a\u65bc\u60a3\u8005\u7684\u6536\u5165\u3002\u7136\u800c\uff0c\u5728\u5176\u4ed6\u5730\u65b9\uff0c\u7372\u5f97\u9776\u5411\u6cbb\u7642\u7684\u6a5f\u6703\u6975\u5176\u6709\u9650\u3002\u60a3\u8005\u61c9\u8207\u4ed6\u5011\u7684\u91ab\u7642\u5718\u968a\u8a0e\u8ad6\u9019\u4e9b\u6cbb\u7642\u7684\u8cbb\u7528\u4e26\u8a0e\u8ad6\u4ed8\u6b3e\u65b9\u5f0f\u3002 ECDGA \u53ef\u8207\u60a3\u8005\u53ca\u5176\u91ab\u7642\u5718\u968a\u5408\u4f5c\uff0c\u5728\u53ef\u80fd\u7684\u60c5\u6cc1\u4e0b\u5e6b\u52a9\u5c0b\u627e\u4ed8\u6b3e\u65b9\u5f0f\u3002     <\/li>\n<\/ul>\n<h3>\u5316\u5b78\u7642\u6cd5<\/h3>\n<p>\u5316\u7642\u4f7f\u7528\u7834\u58de\u764c\u7d30\u80de\u6216\u5176\u4ed6\u5feb\u901f\u5206\u88c2\u7d30\u80de\u7684\u85e5\u7269\u3002\u591a\u7a2e\u5316\u7642\u65b9\u6848\u5df2\u7528\u65bc\u6cbb\u7642 ECD\u3002\u9019\u4e9b\u85e5\u7269\u5305\u62ec\u514b\u62c9\u5c48\u6ff1\u548c\u7532\u6c28\u8776\u5464\u3002\u5c0f\u578b\u7814\u7a76\u8868\u660e\uff0c\u4e00\u4e9b\u60a3\u8005\u5c0d\u5316\u7642\u53cd\u61c9\u826f\u597d\uff0c\u5118\u7ba1\u53cd\u61c9\u7387\u9060\u4f4e\u65bc\u9776\u5411\u6cbb\u7642\u3002\u9019\u4e9b\u6cbb\u7642\u7684\u4e00\u500b\u512a\u9ede\u662f\uff0c\u4e00\u4e9b\u60a3\u8005\u5728\u6709\u9650\u7684\u6cbb\u7642\u6301\u7e8c\u6642\u9593\u5167\u7d93\u6b77\u4e86\u9577\u6642\u9593\u7684\u53cd\u61c9\u3002    <\/p>\n<ul>\n<li><strong>\u514b\u62c9\u5c48\u6ff1<\/strong><br \/>\u5728\u9776\u5411\u6cbb\u7642\u53ef\u7528\u4e4b\u524d\uff0c\u514b\u62c9\u5c48\u6ff1\u88ab\u4f7f\u7528\u4e26\u7814\u7a76\u4f5c\u70ba ECD \u7684\u6cbb\u7642\u65b9\u6cd5\u3002\u5b83\u5c0d ECD \u60a3\u8005\u986f\u793a\u51fa\u4e2d\u7b49\u7642\u6548\u3002\u901a\u5e38\uff0c\u60a3\u8005\u901a\u904e\u975c\u8108\u6ce8\u5c04\u63a5\u53d7\u514b\u62c9\u5c48\u6ff1 5 \u5929\uff0c\u6bcf 28 \u5929\u4e00\u6b21\uff0c\u6301\u7e8c 2-3 \u500b\u6708\u3002  <\/li>\n<li><strong>\u7532\u6c28\u8776\u5464<\/strong><br \/>\u4f7f\u7528\u7532\u6c28\u8776\u5464\u6cbb\u7642 ECD \u7684\u53cd\u61c9\u7387\u8f03\u4f4e\u3002\u7136\u800c\uff0c\u9019\u7a2e\u6cbb\u7642\u901a\u5e38\u8010\u53d7\u6027\u826f\u597d\uff0c\u56e0\u6b64\u53ef\u80fd\u5c0d\u5b83\u6709\u6548\u7684\u4eba\u6709\u76ca\u3002\u7532\u6c28\u8776\u5464\u53ef\u4ee5\u8f38\u6ce8\u3001\u6ce8\u5c04\u6216\u7247\u5291\u7684\u5f62\u5f0f\u7d66\u846f\u3002\u5c0d\u65bc ECD \u60a3\u8005\uff0c\u5b83\u901a\u5e38\u6bcf\u5468\u6ce8\u5c04\u5230\u808c\u8089\u4e2d\u3002   <\/li>\n<\/ul>\n<p><strong>\u514d\u75ab\u7642\u6cd5<\/strong><br \/>\u514d\u75ab\u8abf\u7bc0\u7642\u6cd5\u53ef\u4ee5\u901a\u904e\u523a\u6fc0\u6216\u6291\u5236\u514d\u75ab\u7cfb\u7d71\u4f86\u7e2e\u5c0f ECD \u75c5\u8b8a\u3002<\/p>\n<ul>\n<li><strong>\u5e72\u64fe\u7d20<\/strong><br \/>\u767c\u73fe\u7684\u7b2c\u4e00\u500b ECD \u6cbb\u7642\u65b9\u6cd5\u662f\u5e72\u64fe\u7d20\uff0c\u4e00\u7a2e\u514d\u75ab\u7642\u6cd5\u3002\u5728\u53ef\u7528\u7684\u60c5\u6cc1\u4e0b\uff0c\u805a\u4e59\u4e8c\u9187\u5e72\u64fe\u7d20 \u03b1 \u662f\u9996\u9078\u7684\u5e72\u64fe\u7d20\u6cbb\u7642\uff0c\u56e0\u70ba\u5b83\u7684\u7d66\u846f\u983b\u7387\u8f03\u4f4e\uff0c\u8010\u53d7\u6027\u66f4\u597d\u3002\u4f46\u662f\uff0c\u4e26\u975e\u6240\u6709\u570b\u5bb6\/\u5730\u5340\u90fd\u63d0\u4f9b\u9019\u7a2e\u914d\u65b9\u3002\u5e72\u64fe\u7d20 \u03b1-2a \u5df2\u7528\u65bc\u6cbb\u7642 ECD \uff0c\u9700\u8981\u66f4\u983b\u7e41\u5730\u670d\u7528\u3002\u5e72\u64fe\u7d20\u901a\u5e38\u5728\u5bb6\u4e2d\u901a\u904e\u76ae\u4e0b\u6ce8\u5c04\u6216\u808c\u8089\u6ce8\u5c04\u7d66\u846f\u3002\u9019\u7a2e\u6cbb\u7642\u901a\u5e38\u5fc5\u9808\u7121\u9650\u671f\u5730\u6301\u7e8c\u3002\u96d6\u7136\u4e00\u4e9b\u60a3\u8005\u5f88\u5bb9\u6613\u8010\u53d7\u5e72\u64fe\u7d20\u6cbb\u7642\uff0c\u4f46\u8a31\u591a\u60a3\u8005\u5728\u9069\u61c9\u6cbb\u7642\u540e\u5831\u544a\u4e86\u985e\u4f3c\u6d41\u611f\u7684\u611f\u89ba\u3002\u5df2\u767c\u73fe\u9019\u7a2e\u6cbb\u7642\u53ef\u4ee5\u6e1b\u7de9 ECD \u7684\u9032\u5c55\u3002       <\/li>\n<li><strong>\u897f\u7f85\u83ab\u53f8\u548c\u4f9d\u7dad\u83ab\u53f8<\/strong><br \/>\u4e00\u9805\u5c0f\u578b\u7814\u7a76\u8868\u660e\uff0c\u897f\u7f85\u83ab\u53f8\u548c\u4f9d\u7dad\u83ab\u53f8\u662f\u514d\u75ab\u6291\u5236\u7642\u6cd5\u7684\u5f62\u5f0f\uff0c\u5c0d\u7d04 60% \u7684 ECD \u60a3\u8005\u5177\u6709\u4e2d\u7b49\u7642\u6548\u3002\u9019\u7a2e\u6cbb\u7642\u901a\u5e38\u4ee5\u7247\u5291\u6216\u6db2\u9ad4\u5f62\u5f0f\u6bcf\u5929\u4e00\u6b21\u3002 <\/li>\n<li><strong>\u751f\u7269\u7642\u6cd5<\/strong><br \/>\u751f\u7269\u7642\u6cd5\u662f\u4e00\u7a2e\u6291\u5236 ECD \u7d30\u80de\u7522\u751f\u7684\u7570\u5e38\u5316\u5b78\u7269\u8cea\uff08\u7d30\u80de\u56e0\u6578\uff09\u5f15\u8d77\u708e\u75c7\uff0c\u5c0e\u81f4\u75c5\u8b8a\u7e2e\u5c0f\u7684\u6cbb\u7642\u65b9\u6cd5\u3002\n<p>&nbsp;<\/p>\n<ul>\n<li><strong>\u963f\u90a3\u767d\u6eef\u7d20<\/strong><br \/>\u5c0f\u578b\u7814\u7a76\u8868\u660e\uff0c\u4e00\u4e9b\u60a3\u8005\u5c0d\u963f\u90a3\u767d\u6eef\u7d20\u4f5c\u70ba ECD \u7684\u6cbb\u7642\u9078\u64c7\u6709\u53cd\u61c9\u3002\u963f\u90a3\u767d\u6eef\u7d20\u901a\u5e38\u4f5c\u70ba\u76ae\u4e0b\u6ce8\u5c04\u7d66\u846f\u3002\u963f\u90a3\u767d\u6eef\u7d20\u4f5c\u70ba ECD \u7684\u7368\u7acb\u6cbb\u7642\u7684\u7814\u7a76\u6709\u9650\uff0c\u4f46\u5c0d\u90a3\u4e9b\u5177\u6709\u666e\u904d\u5168\u8eab\u7665\u72c0\u7684\u60a3\u8005\u53ef\u80fd\u6709\u6240\u8aac\u660e\u3002\u963f\u90a3\u767d\u6eef\u7d20\u4e5f\u88ab\u7528\u65bc\u63d0\u9ad8\u9776\u5411\u6cbb\u7642\u7684\u8010\u53d7\u6027\u3002   <\/li>\n<li><strong>\u5176\u4ed6\u751f\u7269\u6cbb\u7642<\/strong><br \/>\u4e0d\u592a\u5e38\u7528\u7684\u751f\u7269\u6cbb\u7642\u5305\u62ec canakinumab\u3001tocilizumab \u548c infliximab\u3002<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<h3>\u81e8\u5e8a\u8a66\u9a57<\/h3>\n<p>\u7531\u65bc\u9019\u7a2e\u75be\u75c5\u7684\u7f55\u898b\u6027\uff0c\u6b77\u53f2\u4e0a\u6c92\u6709\u9032\u884c <a href=\"https:\/\/erdheim-chester.org\/studies-trials\/\" target=\"_blank\" rel=\"noopener\">\u81e8\u5e8a\u8a66\u9a57<\/a> \u4f86\u8a55\u4f30\u6cbb\u7642\u7684\u6709\u6548\u6027\u3002\u65e9\u671f\u8a18\u9304\u7684\u6cbb\u7642\u57fa\u65bc\u5c0d\u975e\u5e38\u5c0f\u7684\u60a3\u8005\u7fa4\u9ad4\u9032\u884c\u7684\u6848\u4f8b\u7814\u7a76\uff0c\u6709\u6642\u53ea\u662f\u55ae\u500b\u60a3\u8005\u3002\u7136\u800c\uff0c\u9019\u7a2e\u60c5\u6cc1\u6b63\u5728\u6539\u8b8a\u3002\u76ee\u524d\uff0c\u4e00\u4e9b\u570b\u5bb6\/\u5730\u5340\u7684 ECD \u60a3\u8005\u6709\u5e7e\u9805\u7814\u7a76\u548c\u81e8\u5e8a\u8a66\u9a57\u3002\u7576 ECD \u60a3\u8005\u53c3\u52a0\u81e8\u5e8a\u8a66\u9a57\u6642\uff0c\u4ed6\u5011\u53ef\u4ee5\u7372\u5f97\u4e00\u4e9b\u53ef\u4f9b ECD \u60a3\u8005\u4f7f\u7528\u7684\u6700\u65b0\u548c\u6700\u597d\u7684\u6cbb\u7642\u65b9\u6cd5\u3002\u6211\u5011\u9f13\u52f5\u6240\u6709\u60a3\u8005\u8207\u4ed6\u5011\u7684\u91ab\u7642\u5718\u968a\u8a0e\u8ad6\u81e8\u5e8a\u8a66\u9a57\u662f\u5426\u9069\u5408\u4ed6\u5011\u7684\u60c5\u6cc1\u3002\u81e8\u5e8a\u8a66\u9a57\u4f7f\u60a3\u8005\u80fd\u5920\u7372\u5f97\u6700\u65b0\u7684\u6cbb\u7642\u65b9\u6848\uff0c\u4f75\u70ba\u79d1\u5b78\u8b49\u660e\u6cbb\u7642\u5c0d\u6cbb\u7642 ECD\u6709\u6548\u92ea\u5e73\u4e86\u9053\u8def\u3002\u9019\u5c0e\u81f4 FDA \u6279\u51c6\u4e86\u5169\u7a2e ECD \u6cbb\u7642\u3002FDA \u7684\u6279\u51c6\u6709\u52a9\u65bc\u6240\u6709 ECD \u60a3\u8005\u7372\u5f97\u6cbb\u7642\u3002\u6211\u5011\u5f37\u70c8\u5e0c\u671b\u5728\u4e0d\u4e45\u7684\u5c07\u4f86\u627e\u5230\u66f4\u5bb9\u6613\u8010\u53d7\u7684\u65b0\u6cbb\u7642\u65b9\u6cd5\u3002         <\/p>\n<p>\u6709\u95dc\u8a73\u7d30\u8cc7\u8a0a\uff0c\u8acb\u53c3\u95b1\uff1a<\/p>\n<ul>\n<li>\u570b\u5bb6\u7d9c\u5408\u764c\u75c7\u7db2\u8def \uff08NCCN\uff09 <a href=\"https:\/\/jnccn.org\/view\/journals\/jnccn\/19\/11\/article-p1277.xml\" target=\"_blank\" rel=\"noopener\"><strong>\u7d44\u7e54\u7d30\u80de\u816b\u7624\uff0c2021 \u7248 NCCN \u816b\u7624\u5b78\u81e8\u5e8a\u5be6\u8e10\u6307\u5357<\/strong><\/a>\u3002<\/li>\n<li>\u8840\u6db2\u96dc\u8a8c\u6587\u7ae0\uff0c<a href=\"https:\/\/ashpublications.org\/blood\/article\/135\/22\/1929\/452713\/Erdheim-Chester-disease-consensus-recommendations\" target=\"_blank\" rel=\"noopener\"><strong>Erdheim-Chester<\/strong><\/a><\/li>\n<\/ul>\n<p>\u9019\u4e9b\u51fa\u7248\u7269\u7531\u8a3a\u65b7\u548c\u6cbb\u7642 ECD \u7684\u9818\u5148\u5c08\u5bb6\u64b0\u5beb\u3002\u5982\u679c\u60a3\u8005\u7531\u5c0d ECD \u77e5\u4e4b\u751a\u5c11\u6216\u77e5\u4e4b\u751a\u5c11\u7684\u91ab\u751f\u770b\u75c5\uff0c\u5f37\u70c8\u5efa\u8b70\u8207\u4ed6\u5011\u5171\u7528\u9019\u4e9b\u6a94\u3002 <\/p>\n<p>[\/et_pb_text][\/et_pb_column][\/et_pb_row][et_pb_row _builder_version=&#8221;4.27.4&#8243; _module_preset=&#8221;default&#8221; locked=&#8221;off&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_column type=&#8221;4_4&#8243; _builder_version=&#8221;4.27.4&#8243; _module_preset=&#8221;default&#8221; global_colors_info=&#8221;{}&#8221;][et_pb_text _builder_version=&#8221;4.27.4&#8243; _module_preset=&#8221;default&#8221; hover_enabled=&#8221;0&#8243; global_colors_info=&#8221;{}&#8221; sticky_enabled=&#8221;0&#8243; locked=&#8221;off&#8221;]<\/p>\n<p><strong>\u4e0b\u4e00\u500b&gt;<\/strong><a href=\"https:\/\/www.erdheim-chester.org\/zh-hant\/%e8%ad%b7%e7%90%86%e4%b8%ad%e5%bf%83\/\">\u8b77\u7406\u4e2d\u5fc3<\/a><\/p>\n<p>[\/et_pb_text][et_pb_text _builder_version=&#8221;4.27.4&#8243; 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