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[À§/½ÊÀÌÁöÀå ¾Æ¹Ð·ÎÀ̵åÁõ. Gastric and duodenal amyloidosis] - ðû

1. Gastric amyloidosis. À§ ¾Æ¹Ð·ÎÀ̵µÁõ

[Áõ·Ê 1]

¼úÀ» ¸¹ÀÌ µå½Ã´ø ºÐÀ¸·Î °©ÀÛ½º·± ÅäÇ÷·Î ÀÀ±Þ½ÇÀ» ¹æ¹®ÇÏ¿© ³»½Ã°æ¿¡¼­ ÀÌ»óÇÑ ¸ð¾çÀÇ º´¼Ò°¡ ¹ß°ßµÇ¾ú½À´Ï´Ù. Á¶Á÷°Ë»ç´Â 'Chronic gastritis, active, with amorphous deposit, consistent with amyloidosis. No H. pylori identified.'¿´½À´Ï´Ù.

Albumin 4.2, globulin 3.6, A/G ration 1.2·Î Á¤»óÄ¡¸¦ ¾à°£ ¹þ¾î³ª´Â Á¤µµ¿´À¸³ª, serum IgG 2104, free kappa light chain 555·Î ¸Å¿ì ³ô¾ÆÁ® ÀÖ¾î MM with AL amyloidosis·Î Áø´ÜµÇ¾úÀ½.


[Áõ·Ê 2]

70´ë ÃÊ¹Ý ¿©¼ºÀ¸·Î °ËÁø ³»½Ã°æ¿¡¼­ À§Á¡¸· ÀÌ»ó¼Ò°ß ¹ß°ßµÇ¾î Á¶Á÷°Ë»ç¸¦ ½ÃÇàÇÏ¿´°í amyloidosis·Î Áø´ÜµÇ¾ú½À´Ï´Ù. ´ç½Ã¿¡´Â stomach localized formÀ¸·Î Æò°¡µÇ¾ú½À´Ï´Ù.

À§Á¶Á÷°Ë»ç, H&E

¾à 2³â ÈÄ weakness¿Í ±â¸³¼º ÀúÇ÷¾ÐÀÌ ¹ß»ýÇÏ¿© ´Ù½Ã °Ë»ç¸¦ Çß½À´Ï´Ù. À§ amyloidosisÀÇ ¹üÀ§°¡ ÈξÀ ³Ð¾îÁö°í Ç¥¸é ºÒ±ÔÄ¢¼ºµµ ½ÉÇØÁ³½À´Ï´Ù. °ñ¼ö Á¶Á÷°Ë»ç¿Í ½ÉÀå Á¶Á÷°Ë»ç¸¦ ÇÏ¿´´Âµ¥ ¸ðµÎ amyloid ħÀ±ÀÌ È®ÀεǾú½À´Ï´Ù. Systemic Ä¡·á¸¦ ½ÃÀÛÇÏ¿´½À´Ï´Ù.

½ÉÀå Á¶Á÷°Ë»ç, Congo red staining ÈÄ ÀÏ¹Ý Çö¹Ì°æÀ¸·Î °üÂûÇÏ¿´À» ¶§ Salmon colorÀÇ amorphous materialÀÌ Àß °üÂûµÇ¾ú½À´Ï´Ù.


2. Duodenal amyloidosis ½ÊÀÌÁöÀå ¾Æ¹Ð·ÎÀ̵µÁõ


[Brief review]

2017³â 6¿ù 1ÀÏ ¸ñ¿äÁý´ãȸ¿¡¼­ ÀÓ»ó°­»ç ±èµ¿Çö ¼±»ý´Ô²²¼­ Àß Á¤¸®ÇØ Áּ̽À´Ï´Ù.


[Cases]

À§ ¾Æ¹Ð·ÎÀ̵µÁõ. º¸¸¸ 4Çü ÁøÇ༺ À§¾Ï°ú °¨º°Áø´Ü¿¡ À¯ÀÇÇØ¾ß ÇÕ´Ï´Ù.

º£Ã¼º´ ȯÀÚ¿¡¼­ ¹ß»ýÇÑ systemic amyloidosis

Localized gastric amyloidosis (2017-4-15. ¼øõ¸¸³»½Ã°æ¼¼¹Ì³ª ¼øõÇ÷¯½º³»°ú ¾È¿ëȯ)

¹«Áõ»ó ¼ºÀÎ °ÇÁø¿¡¼­ ¹ß°ßÇÑ isolated gastric amyloidosis

Postero-GC of high body, biopsy : Amyloidosis, Chronic gastritis, active, with inflamed granulation tissue, No H. pylori identified.
. Amyloid A: Negative
. Amyloid P: Positive
. Lambda : Positive
. Kappa : Focal positive
. TTR : Negative
Àü½Å workupÀ» ÇßÀ¸³ª amyloidosisÀÇ Å¸ Àå±â ħ¹üÀÇ Áõ°Å´Â ¾ø¾ú½À´Ï´Ù. ´Üµ¶Àå±â ħ¹üÀÇ amyloidosis´Â ¿¹ÈÄ°¡ ÁÁÀº °ÍÀ¸·Î µÇ¾î ÀÖ¾î °æ°ú°üÂû ÇÏ´Â °æ¿ì°¡ ¸¹Áö¸¸, ÀÌ È¯ÀÚ´Â ¸Å¿ì ½ÉÇÏ¿´°í ´Ü±â ÃßÀû ³»½Ã°æ¿¡¼­ Á¶±Ý ½ÉÇØÁ® º¸ÀÌ´Â ¼Ò°ßÀÌ À־ Ç×¾ÏÄ¡·á¸¦ ½ÃÀÛÇÏ¿´½À´Ï´Ù.

2018-1-11 ¸ñ¿ä³»½Ã°æÁý´ãȸ Áõ·Ê

¸²ÇÁÁ¾ ȯÀÚÀÇ ½ÊÀÌÁöÀå°ú À§¿¡¼­ amyloidosis È®ÀεÊ.
À¯¼öÇö ¼±»ý´Ô Á¤¸®

Localized asymptomatic gastric amyloisosis

Localized asymptomatic gastric amyloisosis


[FAQ]

[2017-11-5. ¾Öµ¶ÀÚ ÆíÁö (¼øõ Ç÷¯½º ³»°ú ¾È¿ëȯ ¿øÀå)]

±³¼ö´Ô ¾È³çÇϼ̽À´Ï±î? 2017³â 4¿ù ¼øõ¸¸³»½Ã°æ¼¼¹Ì³ª¿¡¼­ ¹ßÇ¥Çß´ø Áõ·ÊÀÎ "localized gastric amyloidosis with kappa and lambda light chain co-expression"ÀÌ Clinical Endoscopy¿¡ accept µÇ°í ¹ßÇ¥µÇ¾î ÀÌ·¸°Ô ¸ÞÀÏ º¸³»µå¸³´Ï´Ù (Ahn. Clin Endosc 2017).

À̹ø Áõ·Ê¸¦ ÁغñÇϸ鼭 localized gastric amyloidosis°¡ rare ÇÑ ÁúȯÀÌÁö¸¸ ³»½Ã°æ °Ë»ç¿¡¼­ biopsy¸¦ ÅëÇØ È®ÁøµÇ¸é systemic ÇÑ Áúȯ ¿©ºÎ¸¦ È®ÀÎÇÏ¿© localized ÇüÅ·ΠȮÀÎ µÇ¸é, EUS ¶Ç´Â EMR/ESD °°Àº invasive inspectionsÀ» ¼öÇàÇϱ⠺¸´Ù´Â ÃßÀû°üÂûÀ» Çϸ鼭 ÁúȯÀÇ º¯È­¸¦ Àß »ìÇÇ´Â °ÍÀÌ Áß¿äÇÏ´Ù´Â ³»¿ëÀ» ÀüÇÏ°í ½Í¾ú½À´Ï´Ù.

"However, only 2% of patients with localized AL amyloidosis exhibited symptom progression, and overall survival was not different from that of the general population, except in cases involving the lungs. Localized AL amyloidosis involving the stomach was also associated with a good prognosis, and long-term outcomes were excellent. Therefore, if localized gastric AL amyloidosis is confirmed by histopathology, it is likely that invasive inspections, such as EUS, EMR, or ESD, are not necessary."

¿äÁòÀº ¾Æħ¿¡ ±³¼ö´ÔÀÇ ºí·Î±×¸¦ Àд °ÍÀ¸·Î ÇϷ縦 ½ÃÀÛÇÏ°í ÀÖ½À´Ï´Ù. Àú´Â °è¼Ó Àоî¿Í¼­ ±×·±Áö EndoToday Update ºí·Î±×·Î ¹Ù²î°í ³ª¼­ ÀбⰡ ´õ ÆíÇØÁø °Í °°½À´Ï´Ù. ^^

³¯¾¾µµ Ãß¿öÁö°í, ¼ÒÈ­±â³»°ú ÀÇ»çµéÀÌ ¹ÙºüÁö´Â ½Ã±â^^ ÀÎ °Í °°½À´Ï´Ù. °Ç°­ÇϽðí, ÁÁÀº ÀÚ¸®¿¡¼­ ¶Ç ºËµµ·Ï ÇÏ°Ú½À´Ï´Ù. °¨»çÇÕ´Ï´Ù.

[2017-11-7. ÀÌÁØÇà ´äº¯]

¾È¿ëȯ ¼±»ý´Ô. Áõ·Ê Àß º¸¾Ò½À´Ï´Ù (Ahn. Clin Endosc 2017). Áö³­ ¼øõ¸¸¼¼¹Ì³ª¿¡¼­ ³íÀǵǾú´ø ³»¿ëÀÌ ´õ¿í º¸°­µÇ¾î Àß Á¤¸®µÈ °Í °°½À´Ï´Ù. Discussion Âü Àß ¾²¼Ì½À´Ï´Ù.

°³¾÷°¡¿¡¼­µµ Áõ·Ê¿¡ ´ëÇÏ¿© °ü½ÉÀ» °®°í ÃÖ¼±ÀÇ Ä¡·á¹ýÀ» °í¹ÎÇÏ°í, °£È¤ ÇÐȸ¿¡¼­ ¹ßÇ¥µµ ÇϽô ¼±»ý´ÔÀÇ È°µ¿Àº ¸¹Àº ÈĹèµé¿¡°Ô ¸ð¹üÀÌ µÈ´Ù°í »ý°¢ÇÕ´Ï´Ù. ¿ì¸® ³ª¶ó¿¡ ¼±»ý´Ô °°Àº ºÐµéÀÌ Á» ´õ ¸¹¾ÆÁö±â¸¦ ¹Ù¶ø´Ï´Ù.

Endoscopic findings. A round lesion approximately 20 mm in diameter, with central depression and a pale-colored base on the lesser curvature of the mid-gastric body.

Histopathologic findings. (A) Congo red staining shows amorphous amyloid proteins (Congo red, ¡¿200). (B) Apple-green birefringence is shown using polarized light microscopy after Congo red staining (Congo red, ¡¿200).

Immunohistochemical staining shows the presence of plasma cells with co-expression of kappa (A) and lambda (B) light chains in the mucosal layer.

ÀÏÀü¿¡ ÇÑ ¹ø ¸»¾¸µå¸° ¹Ù ÀÖÀ¸³ª, Àü½Å ħ¹ü ¾øÀÌ ÇѵΠÀå±â¿¡ ±¹ÇÑµÈ localized formÀÇ amyloidosis¸¦ °¡²û °æÇèÇÕ´Ï´Ù. ´ëºÎºÐ ¿¹ÈÄ´Â ÁÁ´Ù°í »ý°¢µË´Ï´Ù. °ü·Ã ¹®ÇåÀ» ¼Ò°³ÇÕ´Ï´Ù.

"Localized deposition of amyloid may occur in individual organs, in the absence of systemic involvement. The reason for localized deposition is unknown, but it is hypothesized that deposits result from local synthesis of amyloid protein, rather than the deposition of light chains produced elsewhere. We identified 20 cases of localized amyloidosis at our institution between 1993 and 2003. There were 11 males and nine females in the group. The mean age at the time of diagnosis was 65.5 years. Organs involved included skin, soft tissues, oropharynx, larynx, lung, bladder, colon, conjunctiva, and lymph node. In six of nine patients typed, the amyloid light chain was lambda. In those patients where follow-up was available (mean 7.6 years), none developed systemic disease. Localized amyloidosis occurs in a variety of organ systems. Evolution into systemic amyloidosis was not seen in our series of patients, supporting the hypothesis of local production of amyloid protein in these cases." (Biewend ML. Amyloid 2006)

2018³â ´ëÇÑ»óºÎÀ§Àå°üÇ︮ÄÚ¹ÚÅÍÇÐȸÁö


[References]

1) EndoTODAY ´ëÀå ¾Æ¹Ð·ÎÀ̵åÁõ



© ÀÏ¿ø³»½Ã°æ±³½Ç ¹Ù¸¥³»½Ã°æ¿¬±¸¼Ò ÀÌÁØÇà. EndoTODAY Endoscopy Learning Center. Lee Jun Haeng.