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Extranodal Natural Killer(NK)/T-Cell Lymphoma, Nasal Type †
Case01: †
76year-old female. £±½µ´ÖÁ°¤è¤êÉ¡½Ð·ì¤¬¤¢¤ê¼õ¿Ç. É¡¹ÐÆâ¤Ë¼ðáî¤òǧ¤á¤¿¡£
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CD16
- CD16¤ÏIgG¤ÎÄã¿ÆÏÂÀ¥ì¥»¥×¥¿¡ÊFc¦ÃR·¡Ë¤Ç¡¢Fc¦ÃR·A¡Ê¤Þ¤¿¤Ï·-2¡Ë¤ÈFc¦ÃR·B¡Ê¤Þ¤¿¤Ï·-1¡Ë¤Î2¼ïÎà¤Î°äÅÁ»Ò¤Ç¥³¡¼¥É¤µ¤ì¤Æ¤¤¤ë¥¢¥¤¥½¥Õ¥©¡¼¥à¤¬¤¢¤ë.
- CD16°äÅÁ»Ò¿·¿¤Ë¤è¤ê°Û¤Ê¤ëË쥿¥ó¥Ñ¥¯¤¬»ºÀ¸¤µ¤ì, ¤Ò¤È¤Ä¤ÏËì´ÓÄÌ·¿¥¢¥¤¥½¥Õ¥©¡¼¥à¡ÊFc¦ÃR·A¡¢Ê¬»ÒÎÌ50-65kDa¡Ë¤Ç¡¢NKºÙ˦¤äñµå¡¢¥Þ¥¯¥í¥Õ¥¡¡¼¥¸¤Ëȯ¸½¤¹¤ë¡£¤â¤¦¤Ò¤È¤Ä¤Ï¥°¥ê¥³¥·¥ë-¥Õ¥©¥¹¥Õ¥¡¥Á¥¸¥ë¥¤¥Î¥·¥È¡¼¥ë¡ÊGPI¡Ë·ë¹ç·¿¥¢¥¤¥½¥Õ¥©¡¼¥à¡ÊFc¦ÃR·B1¡¢Ê¬»ÒÎÌ48kDa¡Ë¤Ç¡¢¹¥Ãæµå¤Î¤ß¤Ëȯ¸½¤¹¤ë¡£
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CD56(º¸), CD3¦Å(Ãæ), EBER-ISH(±¦)
ÉÂÍýÁÈ¿¥¿ÇÃÇ¡§extranodal NK/T-cell lymphoma, nasal type
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Case02 ÀºÁã¼ðáî †
87year-old male
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ÉÂÍýÁÈ¿¥¿ÇÃÇ: extranodal NK/T lymphoma, nasal type of the rt. testis
Case03 °öƬɡ¹ÐÉÂÊÑ--Ã×»àÀÀµÃæÀÆù²ê¼ð¤ÎÁÈ¿¥Áü †
80yo male. 1¥ö·îÁ°¤è¤êÓë²¼»þÄˤ¬¤¢¤ê¶á°å¼õ¿Ç. º¸Æð¸ý³¸Ç´Ëì¼ðı¤È¤Ó¤é¤ó, º¸É¡Á°ÄíÉô¼¾¿¾, ÃæÉ¡Æ»¤«¤é¤ÎÇ¿½ÁÇÓÝõ¤ò¤ß¤È¤á¤¿¡£ºÇ½é¥Ø¥ë¥Ú¥¹´¶À÷¾É¤ÈµÞÀÉûÉ¡¹Ð±ê¤È¿ÇÃǤµ¤ì¼£ÎŤò¼õ¤±¤ë¤¬Æð¸ý³¸¼ðı¤¬Áý¶¯¤·¤¿¡£Aɱ¡¤ò¾Ò²ð¤µ¤ì°öƬ¤«¤éÆð¸ý³¸¤Ë¤«¤±ÄÙáç¤òȼ¤¦¼ðáî¤ò»ØÅ¦¤µ¤ì¤ë¡¢Æ±Éô¤è¤êÀ¸¸¡¤ò¤ª¤³¤Ê¤¤NK/T cell lymphoma¤È¿ÇÃǤµ¤ì¤¿. (Äó¼¨ÁÈ¿¥¤Ï¤½¤Î»þ¤Î¤â¤Î¡£Ä󶡤µ¤ì¤¿¥Ö¥í¥Ã¥¯¤è¤êºÆÀ÷¿§ÌȱÖÀ÷¿§¤·¤Þ¤·¤¿)¡£
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ÉÂÍýÁÈ¿¥¿ÇÃÇ¡§Nasal NK/T-cell lymphoma, pharynx, biopsy
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Extranodal NK/T-cell lymphoma, nasal type †
mature NK/T-cell lymphoma¤Î°ì·¿. Îã³°¤Ê¤¯EBV´¶À÷¤È´ØÏ¢¤¹¤ë. ¿Í¼ï¤ÎÁǰø¤¬¤¢¤ê¥¢¥¸¥¢, ¥¢¥á¥ê¥«¸¶½»Ì±¤ËȯÀ¸¤·²¤ÊÆ, Æî¥¢¥¸¥¢, ÃæÅì, ¥¢¥Õ¥ê¥«¤Ë¤Ï¾¯¤Ê¤¤.
85%¤ÏÉ¡¹Ð¤Û¤«¾åÉôµ¤Æ»¾Ã²½´É¤ËȯÀ¸¤¹¤ë. »Ä¤ë15%¤ÏƱÍͤÎÎ×¾²ÉÂÍýÁü¤ò¼¨¤¹Àá³°¥ê¥ó¥Ñ¼ð¤¬¾åµ°Ê³°¤ÎÉô°Ì¤Ë½Ð¸½¤·, extranasal NK/T-cell lymphoma¤È¸Æ¤Ð¤ì¤ë. NK cellͳÍ褬¤Û¤È¤ó¤É¤Ç¤¢¤ë¤¬T cellͳÍè¤Î¾ÉÎã¤â¤¢¤êNK/T-cell lymphoma¤¬Å¬ÀڤȤµ¤ì¤Æ¤¤¤ë.
Nasal NK/T-cell lymphoma
Extranodal NK/T-cell lymphoma¤Îŵ·¿Îã¤ÏÉ¡¹ÐÎΰè¤ËȯÀ¸¤¹¤ë. ¼ðáçºÙ˦¤ÎºÙ˦¾ã³²À¤Ë¤è¤êÉ¡, ´éÌÌÀµÃæÉôÁÈ¿¥¤ÎÇ˲õ¤¬¶¯¤¯, Ã×»àÀÃæ¿´ÀÆù²ê¼ð(lethal midline granuloma)¤ËʬÎव¤ì¤Æ¤¤¤¿.
- ÃËÀ¤Ë¿¤¯È¯À¸Ç¯ÎðÃæ±ûÃͤÏ50ºÐÁ°¸å¤Ç¾®»ù¤Ë¤Ï¾¯¤Ê¤¤. ¾¯¤Ê¤¤¾®»ù¾ÉÎã¤Ç¤Ï²ã¥¢¥ì¥ë¥®¡¼¤äEB¥¦¥¤¥ë¥¹´ØÏ¢¼À´µ¤È´ØÏ¢¤¬¤¢¤ë¤³¤È¤¬Â¿¤¤.
- ¥ê¥ó¥Ñ¼ð¤Ï¤¤¤í¤¤¤í¤ÊÁÈ¿¥Áü¤ò¼¨¤¹. ¿¤¯¤ÎÎã¤Ï¿·¿¤Î¤¢¤ë¾®, Ãæ, Âç·¿°Û·¿¥ê¥ó¥Ñµå¤¬¼ï¡¹¤Î±ê¾ÉÀºÙ˦(·Á¼ÁºÙ˦, ¥Þ¥¯¥í¥Õ¥¡¡¼¥¸, ¹¥Ãæµå)¤Èº®ºß¤·¤ÆÁý¿£¤¹¤ë.
- ²õ»à, ·ì´ÉÃæ¿´¿»½á¤ä·ì´É¿»½á¤¬Ä̾ï¤Î½ê¸«¤Î¤¿¤á°ÊÁ°¤Ïangiocentric lymphoma ¤È¤µ¤ì¤Æ¤¤¤¿¡£
- ¼ðáçºÙ˦¤¬¾®·¿¥ê¥ó¥Ñµå¤Î¤ß¤Ç¹½À®¤µ¤ì, °Û·¿¤ä²õ»à½ê¸«¤¬¤Ê¤¤¾ì¹ç¤ÏÍÆ°×¤ËËýÀ±ê¾É¤È¸í¿Ç¤µ¤ì¤ë. °ß, ÂçIJ(¾Ã²½´ÉÉÂÊѤǤÏÀü¹¦¾É¾õ¤¬Â¿¤¤), ÈéÉæ¤Ë¤³¤Î¾®·¿¼ðáçºÙ˦¤¬¿»½á¤¹¤ë¾ì¹ç¤Ï¤è¤ê¿ÇÃǤ¬º¤Æñ¤È¤Ê¤ë.
- °Û·¿¤Î¤Ê¤¤¾®¥ê¥ó¥Ñµå¤Î¿»½á¤Ç¤¢¤Ã¤Æ¤â²õ»à¤Î¤¢¤ë¾ì¹ç¤Ï, CD56¤òǰ¤Î¤¿¤áÀ÷¿§¤·¤Æ¤ß¤ë¡£ÆÃ¤ËÉ¡, °öƬ¤ÎÇ´Ëì¤Î¾ì¹ç. -->¾ÉÎã¤ò¤ß¤ë
- ¹ü¿ñÀ¸¸¡¤Ç¤Ï80%¤¬Èù¾®¤ÊÉÂÊѤΤ¿¤á¥ë¡¼¥Á¥ó¤ÎHEɸËܤǤϸ«¤Ä¤±¤é¤ì¤Ê¤¤¤³¤È¤¬Â¿¤¤¡£
- EBER¤Î¸¡½Ð¤¬¥¹¥Æ¡¼¥¸·èÄê¤ä½éȯÉÂÊѤοÇÃǤËÍÍѤȤʤë.
immunophenotype
- CD2, CD56¤ª¤è¤ÓCD3¦Å¤¬ÍÛÀ. ɽÌÌCD3(sCD3)¤Ï±¢À.
- perforin, granzymeB, TIA-1¤ÎºÙ˦¾ã³²Àðù㤬ÍÛÀ
- ¤È¤¤ËCD30, CD7¤¬ÍÛÀ
- CD56±¢À¤ÎNK/T-cell lymphoma¾ÉÎ㤬¤¢¤ë. CD56±¢À¤Ç, CD4/ CD8¤¬ÍÛÀ¤ò¼¨¤¹¾ÉÎ㤬¤¢¤ê, Î×¾²ÉÂÂÖ¤ÏÊѤï¤é¤Ê¤¤¤³¤È¤«¤é¤³¤Î¼À´µ¤ËʬÎव¤ì¤Æ¤¤¤ë
- CD56±¢À, CD3¦ÅÍÛÀ¤Î¾ÉÎã¤ÏºÙ˦¾ã³²Àðùγ¤ÈEBV¤¬EBER-ISH¤ÇÍÛÀ¤ò³Îǧ¤Ç¤¤ë¤È extranodal NK/T lymphoma¤È¿ÇÃǤµ¤ì¤ë. ξ¼Ô¤¬±¢À¤Î¾ì¹ç¤Ïperipheral T cell lymphoma, not othewise specific¤ËʬÎह¤ë.
genotype
- TCR, Ig gene¤Ïgermline
- TCR¤¬rearrangement¤ò¼¨¤¹¾ÉÎ㤬¾¯¿ô(up to 27%)¤¢¤ë¤¬cytotoxic T cell¤Î¼ðáç¤È¹Í¤¨¤é¤ì¤ë
- p53, c-kit¤Î°äÅÁ»Ò°Û¾ï¤¬»ØÅ¦¤µ¤ì¤Æ¤ª¤êÃϰè¤Ë¤è¤Ã¤ÆÉÑÅ٤κ¹¤¬Ç§¤á¤é¤ì¤ë
- 50%¤Î¾ÉÎã¤Ë¤ÏFas°äÅÁ»ÒÊѰۤ¬Ç§¤á¤é¤ì¤ë
- Ä̾ï¤ß¤é¤ì¤ë³Ë·¿°Û¾ï¤Ï6q deletion¤Ç¤¢¤ë¡£¤·¤Ð¤·¤Ð6q21·ç»¤Ë¤è¤ëHACE1ÍÞÀ©°äÅÁ»Ò¤Îderegulation¤¬·ì´ÉÀ¸Íý¤Î°äÅÁ»Ò, EBV-induced gene, PDGFRA²á¾êȯ¸½¤Ë¤«¤«¤ï¤ë°äÅÁ»Ò¤Ë±Æ¶Á¤·¤Æ¤¤¤ë¤³¤È¤¬¶áǯ¤Îmicroarray¸¦µæ¤Ë¤è¤ê²òÀϤµ¤ì¤Æ¤¤¤ë¡£
prognosis
- °ìÈ̤Ëͽ¸åÉÔÎÉ. 5À¸Î¨<50%.
- É¡¹ÐÉÂÊѤÇ, ¹âMIB-1 index >50%, 40%°Ê¾å¤ÎtransformºÙ˦¤Î¸ºß¤Ïͽ¸åÉÔÎɿ䬰ø»Ò¤È¤Ê¤ë¡£É¡¹Ð°Ê³°¤ÎÉô°Ì¤Ë¤ÏŬ±þ¤Ç¤¤Ê¤¤¡£
Extranasal NK/T-cell lymphoma
- ÂΤΤ¤¤«¤Ê¤ëÉô°Ì¤Ë¤âȯÀ¸¤¹¤ë. ÈéÉæ, ÆðÉôÁÈ¿¥, ¾Ã²½´É, âÐ´Ý¤ÏÆÃ¤Ë¿¤¤. ´Î, ÇÙ, Ǿ, Æý˼, ´ããÝ, Àå, ÍñÁã, Éû¿ÕȯÀ¸¤ÎÊó¹ð¤â¤¢¤ë.
- ÈéÉæ¤Ç¤ÏÂ礤ÊÄÙáçÉÂÊѤޤ¿¤Ï¿ȯ¤¹¤ë¹ÈÈÃÍͤÎȯ¿¾¤ò¤Ä¤¯¤ë¾ pyogenic granuloma¤Ë»÷¤¿ÉÂÊѤò¤Ä¤¯¤ë¤³¤È¤â¤¢¤ë¡£
- ¾Ã²½´É¤ÎÉÂÊѤϥݥ꡼¥×¤¢¤ë¤¤¤Ï©Æù¾õ¼ðáî, ÄÙáç, ¿ȯ¤Ó¤é¤ó¤òÄ褹¤ë. ¿Ê¹Ô¤·¤¿¼ðáç¤Ç¤ÏÂç·¿·ëÀáÉÂÊѤägiant fold¤ò¼¨¤¹
- É¡°Ê³°¤ÎÉÂÊѤÎimmunophenotype, genotype¤Ïnasal NK/T-cell lymphoma¤ÈƱ¤¸¤Ç¤¢¤ë¤¬CD30ÍÛÀ¤ÎÉÑÅÙ¤¬¹â¤¤¡£
- ÀÊÌ, ǯÎðʬÉÛ, ÃÏÍýŪʬÉÛ, immunophenotype¤Ï nasal lymphoma¤ÈÊѤï¤é¤Ê¤¤. ͽ¸å¤ÏÉ¡°Ê³°¤ÎÉÂÊѤΤۤ¦¤¬°¤¤¡£
¥ê¥ó¥ÑÀá¤Ë½éȯ¤·¤¿nasal type NK/T cell lymphoma¾ÉÎã
- ÀáÀ¤Ç¤¢¤ì¤ÐÄ̾ï¤ÏPTCL-NOS¤Ç¤¢¤ë¤¬, Îã³°¤¬¤¢¤ë¡Ê?!)
- ·ÛÉô¥ê¥ó¥ÑÀá¤ÎÊó¹ð¤¬Â¿¤¯¼¡¤¤¤Ç²¼³Ü¥ê¥ó¥ÑÀá¤Ë¤ß¤é¤ì¤ë¤¬Ê£¿ô¤Î¥ê¥ó¥ÑÀáÉÂÊÑÎã¤â¤¢¤ë
- Á´ÎãÃËÀ¤Ç, ǯÎðÃæ±ûÃͤÏ55ºÐ
- ÀáÉÂÊѤÎÁÈ¿¥, ÌȱÖÀ÷¿§½ê¸«¤ÏÀá³°ÉÂÊѤȺ¹¤Ï¤Ê¤¤¤¬TCRºÆ¹½À®¤¬È¾¿ô¤Ëǧ¤á¤é¤ìnasal type¤È¤³¤È¤Ê¤êcytotoxic T cellͳÍ褬¿¤¤(¤³¤Î¾ì¹ç¤Ï, PTCL¤Ç¤Ï¤Ê¤¤¤«¡©¡Ë
- ͽ¸å¤ÏÉÔÎɤǤ¢¤ê²½³ØÎÅË¡¤ò¤ª¤³¤Ê¤Ã¤Æ¤â, ¤Û¤È¤ó¤É¤¬1ǯ°ÊÆâ¤Ë»àË´¤¹¤ë