ȯºîÀÌë´Ö¥Ø¥â¥°¥í¥Ó¥óÇ¢¾É paroxysmal nocturnal hemoglobinuria-PNH †PIGA(phosphatidylinositol glycan anchor biosynthesis class A)°äÅÁ»Ò¤¬·ç»¤·¤¿Ã±ÆÈ¤¢¤ë¤¤¤ÏÊ£¿ô¤Î¤·ì´´ºÙ˦¥¯¥í¡¼¥ó³ÈÂç¤Ë¤è¤ë¸åÅ·À¤·ì´´ºÙ˦¼À´µ.*1
PNH¤ÎÍÏ·ì¤È¾É¾õ †PNH¤Ë¤ª¤¤¤ÆÌë´Ö¤ÎÍÏ·ì¤Ë¤è¤ë¡ÖȯºîÀ¡×ÁáÄ«¥Ø¥â¥°¥í¥Ó¥óÇ¢¤ò¤¤¿¤¹¤Î¤Ï1/4¡Á1/3¤Ç¤¢¤ê, ´ðËÜÉÂÂÖ¤ÏËýÀ·ì´ÉÆâÍÏ·ì¤Ç¤¢¤ë. ·ì´ÉÆâÍÏ·ì¤Ë²Ã¤¨¤Æ, ·ìÀò¾É, ¹ü¿ñÉÔÁ´¤¬»°Âç¼çħ¤È¤µ¤ì¤ë. ËýÀ¿ÕÉÔÁ´¤ä¤Þ¤ì¤Ç¤¢¤ë¤¬Çò·ìɲ½¤¬¤¢¤ë. GPI¥¢¥ó¥«¡¼¥¿¥ó¥Ñ¥¯, CD55, CD59¤ò·ç»¤¹¤ë·ìµå¤ÏÊäÂΤˤè¤ëºÙ˦Ç˲õ¤Î¥¿¡¼¥²¥Ã¥È¤Ë¤Ê¤ê, ÀÖ·ìµå¤ÏÊäÂδ¶¼õÀÀÖ·ìµå¤È¤è¤Ð¤ì¤ë¡£´¶¼õÀ¤Î°ã¤¤¤Ë¤è¤ê, PNH-¶, ·¤È¤µ¤ì, ·¤Ç¤ÏGPI¥¢¥ó¥«¡¼Ë쥿¥ó¥Ñ¥¯¤¬´°Á´¤Ë·ç»¤·¤Æ¤¤¤ë¡£
PNHÀè¹Ô¿ïȼÉÂÊÑ
FLAER PNH¤Îflowcytometry¸¡ºº¤Ç, ºÙ˦ɽÌ̾å¤ÎGPI¥¢¥ó¥«¡¼·¿¥¿¥ó¥Ñ¥¯¼Á¤Î¥¢¥ó¥«¡¼Éô¤ËÆÃ°ÛŪ¤Ë·ë¹ç¤¹¤ë°äÅÁ»ÒÁȤߴ¹¤¨¥¢¥¨¥í¥ê¥¸¥ó¤È¤¤¤¦·Ö¸÷ºÙ¶Ý¥¿¥ó¥Ñ¥¯¥Õ¥ì¥¢(fluorescent-labeled inactive toxin aerolysin; FLAER)¤ò»È¤Ã¤ÆPNH·ìµå¤ò¸¡½Ð¤¹¤ë¿·¤·¤¤ÊýË¡¤¬ÍѤ¤¤é¤ì¤Æ¤¤¤ë¡£FLAER¤ÏÀÖ·ìµå¤ò¶Å½¸¤µ¤»¤ë¤¿¤á¤Ë, ÀÖ·ìµå²òÀϤˤϻÈÍѤǤ¤Ê¤¤¤³¤È¤¬·çÅÀ¡£ CD55/ CD59 Àµ¾ïºÙ˦¤Ç¤ÏCD55, CD59¤¬¤È¤â¤ËÍÛÀ. PNH¾ÉÎã·ìµå¤Ç¤ÏCD55-, CD59-¥¯¥í¡¼¥ó¤¬½Ð¸½¤¹¤ë¡£ÄøÅ٤ϾÉÎã¤Ë¤è¤ê¤µ¤Þ¤¶¤Þ¤Ç1¡Á99%. 0.01%ÄøÅÙ¤ÎPNH·ìµå¤ò¸¡½Ð¤Ç¤¤ë¹âÀºÅÙFCM¤¬ÍѤ¤¤é¤ì¤ë. ðùγµå¤ËÂФ·¤Æ¤Ï¾åµFLAER¤òÍѤ¤¤¿¸¡ºº¤¬¿ä¾©¤µ¤ì¤Æ¤¤¤ë. CD55, CD59¤¤¤º¤ì¤â, ·ì±ÕºÙ˦¤ÎGPI-anchor(glycosyl-phosphatidylinositol anchor)¤ÎÉôʬ¤ÇËì¤Ë·ë¹ç¤·¤Æ¤¤¤ëGPI Ëì·ë¹çÃÁÇò¤Ç, ÊäÂγèÀ²½¤òÍÞÀ©¤¹¤ëÊäÂÎÄ´À᥿¥ó¥Ñ¥¯¼Á. CD59¤¬ÊäÂΤˤè¤ëÍÏ·ìÁ˻ߤˤè¤ê½ÅÍפȹͤ¨¤é¤ì¤Æ¤¤¤ë.
PNH¤Î¼£ÎÅ-¹³ÊäÂÎÌô¤ÎÅÐ¾ì †¥¨¥¯¥ê¥º¥Þ¥Öeculizumab¤Ï, PNH¤Ë͸ú¤Ê¹³C5¹³ÂÎÌô¤È¤·¤Æ³«È¯¤µ¤ì¤¿.2007ǯ¤Ë³«»Ï¤µ¤ì¤¿AEGIS»î¸³¤Ë¤è¤ê°ÂÁ´À¡¦¸ú²Ì¤¬³Îǧ¤µ¤ì2011ǯ¤ËÌô²Á¼ýºÜ. ¥¨¥¯¥ê¥º¥Þ¥Ö¤ÏÊäÂÎC5¤Îepitope¤È·ë¹ç¤·±ê¾ÉÀ¥¿¥ó¥Ñ¥¯¤Ç¤¢¤ëC5a(¥¢¥Ê¥Õ¥£¥é¥È¥¥·¥ó)Êü½Ð¤òÁ˳²¤¹¤ë¤È¤È¤â¤Ë, C5ž´¹¹ÚÁǤκîÍѤòÁ˳²¤·, ¤³¤ì¤Ë³¤¯C5b-C9Ë쿯½±Ê£¹çÂÎ(MAC)¤ÎÀ¸À®¤ò¤µ¤»¤Ê¤¤. ɸ¶ÂÎ¥ª¥×¥½¥Ë¥ó²½¤äÌȱÖÊ£¹çÂνüµî¤Ë¤Ï±Æ¶Á¤ò¤¢¤¿¤¨¤Ê¤¤¤¿¤á, ½ÅÍפÊÊäÂε¡Ç½¤ÏÊÝ»ý¤µ¤ì¤ë. ¥¨¥¯¥ê¥º¥Þ¥ÖÅêÍ¿¤ÇPNHÀÖ·ìµå¤ÏÁý²Ã¤¹¤ë(²õ¤ì¤Ê¤¤).LDH¤Ï¸º¾¯¤··ì´ÉÆâÍÏ·ì¤ÏÍÞÀ©¤µ¤ì¤ë¡£¤·¤«¤·CD55¤¬·ç»¤·¤Æ¤¤¤ë¤¿¤áC3b¤È¤½¤Îʬ²ò»ºÊª¤Ç¤¢¤ëC3d, C3dg¤¬PNHÀÖ·ìµå¾å¤ËÃßÀѤ·, ·ì´É³°ÍϷ줬¸²ºß²½¤·¤Æ¤¯¤ë.
|