PathologyAtlas

Mantle cell lymphoma

Mantle cell lymphoma¤Î¾ÉÎã01, ¾ÉÎã02, ¾ÉÎã03

Mantle cell lymphoma¤ÎÉÂÍýÁÈ¿¥Áü

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ÄãÇÜΨ¤Ç¤ÎÁý¿£¥Ñ¥¿¡¼¥ó¤Ë¤Ï, nodular, diffuse pattern¤Î2¥Ñ¥¿¡¼¥ó¤È, ¤½¤Îº®¹ç·¿¤¬¤¢¤ë¡£

nodular pattern¤¬Ç§¤á¤é¤ì¤ë¾ÉÎã¤Ï½é¿Ç»þ¤Ç40%¤Û¤É¤Ç,ɽé´ü¤ËÌÀÎÆ¤Ê·ëÀá·ÁÀ®¤Þ¤¿¤ÏÉÔÌÀÎÆ¤Ê·ëÀá·ÁÀ®(= vaguely nodular growth, vague nodule)¤¬Ç§¤á¤é¤ì¤ë¡£·ëÀ᤬¸«¤é¤ì¤ë¤Î¤ÏÉôʬ¤Ë¸Â¤é¤ì¤ë¡£

¤³¤Î¾, nodular MCL¤Ë¤Ï´û¸¤Î¥ê¥ó¥ÑßÉ˦æõÃæ¿´¤ò¼è¤ê°Ï¤ó¤Ç¥Þ¥ó¥È¥ëÁؤ¬¹âÅÙ¤ËÈî¸ü¤·¤Æ¤¤¤ë¤è¤¦¤Ë¸«¤¨¤ë¾ì¹ç¤¬¤¢¤êmantle zone pattern¤È¸Æ¤Ð¤ì¤ë¡£¤³¤Îpattern¤ÎÁý¿£Îã¤Ë¤Ï,æõÃæ¿´¤ò·ç¤¯°ì¼¡ßÉ˦¤¬Ç§¤á¤é¤ì¤ë¤³¤È¤¬¤¢¤ë¡£°ì¼¡ßÉ˦¤ÎÃøÌÀ¤Ê¾ÉÎã¤Ç¤Ïsmall cleaved cell type¤Îfollicular center cell lymphoma ¤È¤Þ¤®¤é¤ï¤·¤¤¡£

É´ü¤¬¿Ê¹Ô¤¹¤ë¤È¼ðáçºÙ˦¤Î¿»½á¤Ë¤è¤êÈ¿±þÀ­ßÉ˦¤ÈßÉ˦´Ö¼ðáçºÙ˦¤Î¶èʬ¤Ï¾Ã¼º¤·diffuse pattern¤È¤Ê¤ë¡£1/4¤Û¤É¤Î¾ÉÎã¤Ç¤Ï, ¤³¤Îdiffuse pattern»þ´ü¤Ç¤âÉÔÌÀÎÆ¤Ênodular pattern¤¬°ä»Ä¤·¤Æ¤¤¤ë¡£

Àµ¾ï¤Êlymphocytic cuff¤ò·ç¤¯Íç¤ÎßÉ˦(=naked follicle)¤¬¤Ó¤Þ¤óÀ­Áý¿£Æâ¤Ë¸«¤é¤ì¤ë¤³¤È¤¬¤¢¤ë¡£

CLL/SLL¤Ë¸«¤é¤ì¤ëpseudofollicle/ proliferation center¤òǧ¤á¤Ê¤¤¡£--->marginal zone-like variant MCL

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ÄãÇÜΨ¤Ç¸«¤ëÁý¿£¼ðáçºÙ˦¤ÎñĴ¤µ, ¶Ñ¼Á¤µ¤¬¤­¤ï¤À¤Ä¤³¤È¤¬MCL¤Î¿ÇÃǤ˽ÅÍפʽ긫¤Ç¤¢¤ë¡£¶Ñ°ì¤ÊºÙ˦¤Î·ÁÂ֤ˤÏ, ¤¤¤í¤¤¤í¤Ê¥ô¥¡¥ê¥¨¡¼¥·¥ç¥ó¤¬¸«¤é¤ì¤ë¡£

Â礭¤µ¤Î¶Ñ°ì¤Ê¾®·¿¤Ê¤¤¤·Ãæ·¿¤ÎºÙ˦¤¬Áý¿£¤·¤Æ¤¤¤ë¡£ºÙ˦¼Á¤Ï˳¤·¤¯, ÉÔÀ°¤Ç, ¤Ç¤³¤Ü¤³¤Ê(indented)³Ë¤ËÃæÅùÅÙ¤ËÁƤ¤¥¯¥í¥Þ¥Á¥ó¤¬Ç§¤á¤é¤ì¤ë¡£³Ë¾®ÂΤϸ«¤¨¤Ë¤¯¤¤¡£(³Ë¾®ÂΤ¬ÌÀÎÆ¤Ê¤â¤Î¤Ïpleomorphic variant¤äMCL°Ê³°¤Î¥ê¥ó¥Ñ¼ð¤òµ¿¤¦¡£) ÀÚ¤ì¹þ¤ß¤Î¤¢¤ë³Ë¤â¤·¤Ð¤·¤Ð½Ð¸½¤¹¤ë¡£¾®·¿±ß·Á³Ë¤Î¥ê¥ó¥Ñµå¤ÏTºÙ˦¤Ç¼ï¡¹¤ÎÄøÅ٤˺®ºß¤¹¤ë¡£

  • ÉÔÀ°¤Ê³Ë¤ÎºÙ˦¤¬¤´¤¯¤ï¤º¤«¤Ç, ¤Û¤È¤ó¤É¤¬Îà±ß·Á³Ë¤Î¾ÉÎã¤ä, ³ÑÄ¥¤Ã¤¿¤êÀÚ¤ì¹þ¤ß¤Î¤¢¤ë³Ë¤ÎºÙ˦(centrocytic¤Î·ÁÂÖ),¤Þ¤¿¤ÏǾ²óÍͳˤò¤â¤ÄºÙ˦¤ÎMCL¤â¤¢¤ë¤Î¤Ç¤¹¡£
  • Áý¿£ºÙ˦¤Ëparaimmunoblast¤älarge noncleaved cell/ centroblast(FL¤Ë¸«¤é¤ì¤ë)¤òǧ¤á¤Ê¤¤¡£plasma cell¤Îº®ºß¤Ï¤Ê¤¤¤«, ¤´¤¯¤ï¤º¤«¤Ç, polyclonal¤Ç¤¢¤ë¡£
  • Ë­¤«¤ÇøÌÀ¤ÊºÙ˦¼Á¤ò¤â¤Ámarginal zone/ monocytoid B cell¤Ë»÷¤¿Áý¿£ºÙ˦¤¬ÉôʬŪ¤Ë¸«¤é¤ì¤ë¾ÉÎ㤬¤¢¤ë¡£¤³¤ì¤é¤Î¾ÉÎã¤Ë¤Ïŵ·¿Åª¤Ê¥¨¥ê¥¢¤¬Â¸ºß¤¹¤ë¤Î¤Çartefact¤Ê¤Î¤«¤â¤·¤ì¤Ê¤¤¡£
  • mitosis¤ÏÄ̾ï¡ã20/hpf¤È¿¤¯¤Ï¤Ê¤¤¡£

Large cell(anaplastic centrocytic)/ blastic forms of MCL

MCL¤Î20%¤Ë¼ðáçºÙ˦¤¬Ä̾ï¤ÎMCL¤è¤êÂ礭¤¯, ¥¯¥í¥Þ¥Á¥ó¤Ï¤­¤áºÙ¤«¤¯Ê¬»¶¤·, ¾®¤µ¤Ê³Ë¾®ÂΤ¬¸«¤é¤ì¤ëhigh-grade variant¤¬Ç§¤á¤é¤ì¤ë¡£

  • mitosis¤Ïlymphocytic form MCL¤ËÈæ¤Ù¤ÆÂ¿¤¯benign histiocyte¤Îº®ºß¤¬¸«¤é¤ì¤ë¡£
  • high-grade MCL¤Ïde novo¤Î¼À´µ¤Çlymphocytic type¤«¤éhigh-grade¤Ø¤Îtransformation¤Ï¿¤¯¤Ê¤¤¡£
  • MCL¤è¤êdiffuse large B cell lymphoma¤Ø¤Îtransformation¤Ï¤Þ¤ì¡£

Mantle cell lymphoma histological variant ÁÈ¿¥Áü--->Variant¤ÎÁÈ¿¥Áü(pdf file)

 
MCL-classicOK.jpg(91.4KB)MCL-pleo.jpg(100.2KB)MCL-blastoid.jpg(123.8KB)MCL-smallcell.jpg(122.9KB)
classical MCLpleomorphic variantblastoid variantsmall cell variant

1. classical MCL

¾®·¿¤«¤éÃæ·¿¥ê¥ó¥Ñµå¤Ç³Ë¤Ï¤ï¤º¤«¤ËÉÔÀ°¤Ê¤â¤Î¤«¤é¶¯¤¯¤æ¤¬¤à¤â¤Î¤Þ¤Çǧ¤á¤é¤ìcentrocyte¤Ë»÷¤Æ¤¤¤ë. ¥¯¥í¥Þ¥Á¥ó¤Ï´öʬ, ʬ»¶¤·¤Æ¤ß¤é¤ì, ³Ë¾®ÂΤÏÉÔÌÀÎÆ¤Ç¤¢¤ë.

2. blastoid variant

¥ê¥ó¥Ñ²êµå·¿¥ê¥ó¥Ñ¼ðºÙ˦¤Î¤è¤¦¤ÊÁ¡ºÙ¤Ê³Ë¥¯¥í¥Þ¥Á¥ó¤ÇN/CÈæ¤¬¹â¤¤Îà±ß·Á¼ðáçºÙ˦¤«¤éÀ®¤ë¡£³ËʬÎö¤¬Â¿¤¤¡£

3. pleomorphic variant

¼ðáçºÙ˦¤ÏǾ²ó¾õ¤Ë¤¯¤Ó¤ì¤¿Ãæ¤Ê¤¤¤·Âç·¿³Ë¤ò¤â¤Ä¡£³Ë¾®ÂΤâÌÀÎÆ¤Ê¤â¤Î¤¬½Ð¸½¤¹¤ë¡£³Ë·ÁÂÖ¡¢ºÙ˦¤Î¥µ¥¤¥º¤È¤â¤Ë¿·ÁÀ­¤ËÉ٤ࡣHE¤Ç¤ÏATLL¤Ê¤É¤ÎTºÙ˦¼ðáç¤ä¤Ó¤Þ¤óÀ­BºÙ˦À­¥ê¥ó¥Ñ¼ð¤Î¼ðáçºÙ˦¤Ë»÷¤ë¡£

4. small cell variant

CLL¤Ë»÷¤¿¾®·¿¥ê¥ó¥Ñµå¤Îº®ºß¤äÍ¥°Ì¤ÊÁý¿£¤¬¸«¤é¤ì¤ë. ¥¯¥í¥Þ¥Á¥ó¤Ï¤è¤ê¶Å½¸¤·¤Æ¤¤¤ë¤è¤¦¤Ë¸«¤¨¤ë.

5. marginal zone -like

øÌÀ¤Ç¹­¤¤ºÙ˦¼Á¤ò¤â¤Ä, ÊÕ±ïÂÓBºÙ˦¤¢¤ë¤¤¤Ï monocytoid B-cell¤Ë»÷¤¿ºÙ˦¤Îfoci¤¬¤á¤À¤Ámarginal zone lymphoma¤ËÎà»÷¤¹¤ë. ¤È¤­¤Ë¤³¤ÎøÌÀ¤Ê¹½Â¤¤ÏCLL/SLL¤Îproliferation center¤Èº®Æ±¤µ¤ì¤Æ¤·¤Þ¤¦.

ÌȱÖÀ÷¿§ immunophenotype*1

MCL¼ðáçºÙ˦¤ÏºÙ˦ɽÌÌIgM/IgD¤òȯ¸½. ·Úº¿¤Ïlambda restriction¤¬kappa¤è¤ê¤â¿¤¯Ç§¤á¤é¤ì¤ë.

BCL2¤¬¶Ñ°ì¤ËÀ÷¤Þ¤ê, CD5, FMC7, CD43¤âÍÛÀ­¤È¤Ê¤ë. »þ¤ËIRF4/MUM-1ÍÛÀ­¤ò¼¨¤¹.

CD10, BCL6¤ÎæõÃæ¿´¥Þ¡¼¥«¤Ï±¢À­. CD23¤Ï±¢À­¤Þ¤¿¤Ï¼åÍÛÀ­¤È¤Ê¤ë.

cyclinD1¤ÏMCL¾ÉÎã¤Î95%¤¬ÍÛÀ­¤ò¼¨¤¹. ¤ï¤º¤«¤ÊÎã¤ÇCD5¤¬±¢À­¤È¤Ê¤ë.ʸ¸¥¤Ç¤Ï6%.*2-->CD5±¢À­MCL¤Î¾ÉÎã

SOX11¤ÏºÇ¤â´¶Å٤ι⤤¹³ÂΤÇMCL¤Î90%¤¬ÍÛÀ­¤È¤Ê¤ë. cyclinD1±¢À­Îã¤Èblastoid Îã¤âÍÛÀ­¤Ç¤¢¤ë. ¤¿¤À¤·, SOX11¤Î´¶ÅÙ, ÆÃ°ÛÀ­¤Ï»È¤¦¹³ÂΤˤè¤ê¤µ¤Þ¤¶¤Þ¤Ç¤¢¤ë¤³¤È¤ËÃí°Õ¤¬É¬Í×.

blastoid¤¢¤ë¤¤¤Ïpleomorphic case¤Ç¤Ï¤È¤­¤Ë¹³¸¶¤Îaberrant expression¤¬¸«¤é¤ì, CD5¤¬¤½¤Þ¤é¤ºCD10, BCL6¤¬ÍÛÀ­¤È¤Ê¤ë.

¤Þ¤ì¤ÊMCL¤Ç¤ÏCLL/SLL¤Î¥Þ¡¼¥«¤Ç¤¢¤ëLEF1¤ÈCD200¤¬ÍÛÀ­¤ò¼¨¤¹. LEF1¤Ïblastoid/ pleomorphic case¤Ë, CD200¤Ïleukaemic non-nodal variant¤Ëȯ¸½¤¹¤ë¤³¤È¤¬Â¿¤¤.

FDC¤ÎÁƤÊÌÖÌܹ½Â¤¤¬ÌȱÖÀ÷¿§¤Ë¤è¤ê¤·¤Ð¤·¤Ð´Ñ»¡¤µ¤ì¤ë.(CD23, CD21À÷¿§)

 
 

mantle cell lymphoma¤Îphenotype variantÊó¹ð

Mantle cell lymphoma¤ÎÎ×¾²¤Èͽ¸å

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  • ½é¿Ç»þ¿Ê¹Ô¤·¤¿stage (stage­·/­¸)¤Î¤³¤È¤¬Â¿¤¯, Á´¿È¥ê¥ó¥ÑÀá¼ðÂç, ¹ü¿ñ, ´Î¤Ø¤Î¿¯½±¤¬¤¢¤ë¤¬,50%¼å¤Ç¤Ï̵¾É¾õ¡£
     
  • 磤ϿÇÃÇ»þ¤ËÌó60%¤¬¿»½á¤¢¤ê, ¤½¤Î¾¤ÎÀá³°ÉÂÊѤǤϾò½´É¤ÈWaldyer ring¤¬Â¿¤¤¡£
     
  • ¾Ã²½´É¤Ç¤ÏÆÃħŪ¤Êmultiple lymphomatous polyposis¤ò¤­¤¿¤¹
     
  • ½é¿Ç»þÃæ¿õ¿À·Ð¤Î¹çÊ»¤Ï¤Þ¤ì¤Ç¤¢¤ë¤¬·Ð²áÃæ, 10%¤Ë¿¯½±¤¢¤ê
     
  • ·ÚÅÙ¤ÎÉÏ·ì¤Ï¤Þ¤ì¤Ç¤Ï¤Ê¤¤¡£·ì¾®Èĸº¾¯¤Ï<15%
     
  • Ëö¾¿·ì¥ê¥ó¥ÑµåÁý¿¤Ï, >4000 (20-40%)¤Ç¤¢¤ë¤¬>20000¤ò±Û¤¹¾ÉÎã¤Ï¤Þ¤ì¡£
     
  • ¤´¤¯·ÚÅÙ¤Îmonoclonal gammopathy¤¬¸«¤é¤ì¤ë¤³¤È¤¬¤¢¤ë(Èó¼ðáçºÙ˦¤«¤é¤Î»ºÀ¸)

*1  Swerdlow SH, et al. Mantle cell lymphoma. In: Swerdlow SH, Campo E, Harris NL., eds WHO Classification of Tumors of Hematopoietic and Lymphoid tissues. 4th ed. Lyon, France: IARC; 2017:276-277.
*2  Miao Y, et al. CD5-negative mantle cell lymphoma. Clinicopathological correlations and outcome in 58 patients. Am J Surg Pathol 2019; 43(8): 1052-1060
*3  Chuang W-Y, et al. Identification of CD5/cyclinD1 double negative pleomorphic mantle cell lymphoma. A clinicopathologic, genetic, and gene expression study. Am J Surg Pathol 2020; 44(2):232-240.
*4  Xu J, et al., SOX11-negative mantle cell lymphoma. Clinicopathologic and prognostic featues of 75 patients. Am J Surg Pathol 2019; 43(5): 710-716

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Last-modified: 2020-10-21 (¿å) 16:43:24 (98d)