CHUBU1215
·ì±Õ¼ðáçÉÂÍý³Ø
µÞÀµð³ËµåÀÇò·ìÉ¡¡acute megakaryoblastic leukaemia (M7) †
WHO 4th ed. revised ¤ÎÄêµÁ*1¡§Aute myeloid leukemia, NOS¤Î°ì·¿¤Ç¤¢¤ëAcute megakaryoblastic leukemia. FABʬÎà¤ÎAML-M7
µÞÀÇò·ìɤΰ췿¤Ç20%°Ê¾å¤ËÁý¤¨¤¿²êµå¤Î¾¯¤Ê¤¯¤È¤âȾ¿ô(50%)¤¬µð³Ëµå·Ï¤Ç¤¢¤ë¤â¤Î¡£
MDS leukemia¤«¤é°Ü¹Ô¤·¤¿Îã¤ä, t(1;22)(p13;q13), inv(3)(q21;q26.2), t(3;3)(q21;q26.2)¤ò¤â¤ÄAMLÎã(AMKL¤Î·ì±ÕÁü¤ò¼¨¤¹), Down¾É¸õ·²´ØÏ¢¤Î¾ÉÎã¤Ï, ¤³¤Î¥«¥Æ¥´¥ê¡Ý¤Ë¤Ï´Þ¤Þ¤Ê¤¤¡£
- t(1;22)(p13;q13): RBM15-MKL1
- inv(3)(q21q26.2) or t(3;3)(q21;q26.2): RPN1 - EVI1
°Ê²¼3·²¤Î´µ¼Ô¤µ¤ó¤Ë, ¤ª¤Î¤ª¤Î°Û¤Ê¤Ã¤¿¥¿¥¤¥×¤ÎAMKL¤¬µ¯¤³¤ê¤¦¤ë.
1) Down¾É¤Î¾®»ù¡§WHOʬÎà¤ÇDown¾É¸õ·²´ØÏ¢¹ü¿ñÁý¿£À¼À´µ(Myeloid proliferations associated with Down syndrome)¤È¤·¤ÆÆÈΩ¤·¤¿¼À´µ¤È¤µ¤ì¤ë.
2) Down¾É¸õ·²°Ê³°¤Î¾®»ù: Æý»ù¤Ç¤ÏÂçÉôʬ¤Î¾ÉÎ㤬WHOʬÎà¤ÎAML with t(1;22)(p13;q13); RBM15-MKL1¤ËÁêÅö. ǯĹ»ù¤Ç¤Ï, t(10;11); CALM-AF10, t(9;11), +8, +21¤Ê¤ÉÍÍ¡¹¤ÊÀ÷¿§ÂÎ, °äÅÁ»Ò°Û¾ï¤òȼ¤Ã¤¿¾ÉÎ㤬¸«¤é¤ì¤ë.
3) À®¿Í; ÆÃÄê¤Î°äÅÁ»Ò°Û¾ï¤Î¤ß¤é¤ì¤Ê¤¤¾ÉÎã(AML-NOS)¤¬Â¿¤¤. AML with inv(3)(q21q26.2) or t(3;3)(q21;q26.2); RPN1-EVl1¤Ç¤ÏAMKL¤ÎºÙ˦·ÁÂֽ긫¤ò¼¨¤¹¤³¤È¤¬¤¢¤ë.
AMKL-NOS
- À÷¿§ÂÎ; -5, -7, +8, 11q ´ØÏ¢¤Î°Û¾ï¤Ê¤É¤¬Ç§¤á¤é¤ì¤ë.
- Young adult¤Ë¤ª¤±¤ë½Ä³ÖæõºÙ˦¼ðáç¤ÈAMKL¹çÊ»Îã¤Ë¤Ï¤¤¤¯¤Ä¤«¤ÎÀ÷¿§Âΰ۾郎ÃΤé¤ì¤Æ¤¤¤ë. i(12p)¤¬ÆÃħŪ¤Ç¤¢¤ë.
AML with t(1;22)(p13;q13); RBM15-MKL1
- RBM15-MKL1fusion¤Ë¤è¤êRBM15(RNA-binding motif protein-15; OTT¤È¤â¤è¤Ð¤ì¤ë)¤ÈMKL1(megakaryocyte leukemia-1; MAL¤È¤â¤è¤Ð¤ì¤ë)¤ÎÍ»¹ç¥¥á¥é¥¿¥ó¥Ñ¥¯RBM15-MKL1(OTT-MAL)¤¬»ºÀ¸¤µ¤ì¤ë.
- RBM15¤ÏHox¤Îµ¡Ç½¤äRas¥·¥°¥Ê¥ëÅÁã¤ÎÄ´Àá¤Ë¤«¤«¤ï¤ê, MKL1¤Ï¥¯¥í¥Þ¥Á¥ó¹½Ãۤ˴ØÏ¢¤¹¤ëDNA·ë¹ç¥â¥Á¡¼¥Õ¤ò¤â¤Ä.Í»¹ç°äÅÁ»Ò¤Ï¥¯¥í¥Þ¥Á¥ó¤Î¹½ÃÛ¤äHox¤ÇͶƳ¤µ¤ì¤ëʬ²½¤È¥·¥°¥Ê¥ëÅÁã¤Ë±Æ¶Á¤òÍ¿¤¨Çò·ìÉÂȯ¾É¤Ë´Ø¤ï¤ë²ÄǽÀ¤¬¤¢¤ë.
ÉÂÍýÁÈ¿¥, ºÙ˦½ê¸« †
Ëö¾¿·ì¤Îsmall megakaryoblast(micromegakaryoblast); ÃæÄøÅÙ¤ÎN/CÈæ, ºÙ˦¼Á¤Îbudding¡Ê½Ð²ê), ÈùºÙ¤ÊÌÖÌܾõ¥¯¥í¥Þ¥Á¥ó¤ò¤â¤Ä±ß·Á³Ë¤ò¤â¤Ä¡£
megakaryoblast¤Ï¿ºÌ¤Ê·ÁÂ֤Τ¿¤áµð³Ëµå·Ï¤Ç¤¢¤ë¤³¤È¤Î¾ÚÌÀ¤Ë, ¤·¤Ð¤·¤ÐÌȱÖÀ÷¿§¤¬É¬ÍפȤʤ롣CD41, CD42b, CD61À÷¿§ÍÛÀ¤¬µð³Ëµå·Ï¤Î¾ÚÌÀ¤È¤Ê¤ë.
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CD41(anti-GP¶b)¤Ïµð³Ë²êµå¤ÎÍļå¤ÊÃʳ¬¤è¤ê¸¡½Ð¤µ¤ìÉÑÍѤµ¤ì¤ë¡£CD41(=platelet fibrinogen receptor alpha subunit)¤Ï·ì¾®ÈĶŽ¸¤ËɬÍפÊÅü¥¿¥ó¥Ñ¥¯.
¤¿¤À¤·CD41¤Ï¥Û¥ë¥Þ¥ê¥ó¸ÇÄê¥Ñ¥é¥Õ¥£¥óÁÈ¿¥¤Ë¤¦¤Þ¤¯Å¬±þ¤Ç¤¤ë»ÔÈι³ÂΤ¬¤Ê¤¤¡£ÁÈ¿¥¤Ë¤ÏCD42b, CD61¤ò»È¤Ã¤Æ¤¤¤ë¡£FCM¤Ç¤ÏCD41¤¬»È¤ï¤ì¤ë.
·ì¾®ÈĤˤϡ¤10¼ïÎà°Ê¾å¤ÎÀÜÃåʬ»Ò¤¬Â¸ºß¤¹¤ë. ¤½¤Î¤¦¤ÁCD41(GP¶b/·a)¡¤CD42b(GPµb¦Á)¤Ï, ·ì¾®ÈĤÀ¤±¤Ë¸ºß¤·¡¤Ç´Ãå(ºÙ˦-´ð¼Á)¡¤¶Å½¸(ºÙ˦-ºÙ˦)¤Ë¿¼¤¯´Ø¤ï¤Ã¤Æ¤¤¤ë·ì¾®ÈÄËìÅüÃÁÇò¤Ç¤¢¤ë.
- CD41(GP¶b/·a)¤Ï·ì¾®ÈĤ¬»É·ã¤ò¼õ¤±¤Æ³èÀ²½¤µ¤ì¤ë¤È¡¤¥ê¥¬¥ó¥É·ë¹çÉô°Ì¤¬Ïª½Ð¤·¥Õ¥£¥Ö¥ê¥Î¥²¥ó¥ì¥»¥×¥¿¡¼¤È¤·¤Æ¤Îµ¡Ç½È¯¸½¤¬Í¶Æ³¤µ¤ì¤ë.
- CD42b(GPµb¦Á)¤ÏÆâÈé²¼ÁÈ¿¥¤Ë´Þ¤Þ¤ì¤ë¥³¥é¡¼¥²¥ó¤Ë·ë¹ç¤·¤Æ¹½Â¤ÊѲ½¤òµ¯¤³¤·¤¿vWF¤Î¥ì¥»¥×¥¿¡¼¤È¤·¤Æ·ë¹ç¤¹¤ë
µð³Ë²êµåmegakaryoblast
- Ä̾ï¤ÏÃæ·¿¤Ê¤¤¤·Âç·¿ºÙ˦¤Ç³Ë¤Ï±ß·Á, ¤Ê¤¤¤·¤ï¤º¤«¤ËÉÔÀ°·Á¡£³Ë¥¯¥í¥Þ¥Á¥ó¤Ï¾¤ÎAML²êµå¤ËÈæ¤Ù¤Æ¤è¤êÇ»½Ì¤·¤¿·¹¸þ¤ò¤·¤á¤¹¡£¤·¤Ð¤·¤Ð°Û·¿µð³Ëµå¤Î¤è¤¦¤ÊÀ®½Ï¤·¤¿½ê¸«¤òÄ褹¤ë¤³¤È¤¬¤¢¤ë¡£1-3¸Ä¤Î³Ë¾®ÂΤò¤â¤Ä¡£
- À¸¸¡ÁÈ¿¥¤Ç¤â, ÆÃħ¤Î¤Ê¤¤²êµå¤«¤é°Û·¿µð³Ëµå¤È·ÁÂÖŪ¤ËƱÄê¤Ç¤¤ëºÙ˦¤Þ¤Ç¤Î°Ü¹ÔÁü¤¬¤ß¤é¤ì¤ë¤³¤È¤¬¤¢¤ë¡£
- ºÙ˦¼Á¤Ï¹¥±ö´ðÀ¤Çðù㤬¸«¤é¤ì¤ë¤³¤È¤â¤¢¤ë¡£
- ºÙ˦¼ÁÊÕ±ï¤Ï¤®¤¶¤®¤¶¤ÇºÙ˦ÆÍµ¯¤äµ¶Â¤¬µð³Ë²êµå¤ÎÆÃħ¤Ë¤Ê¤ë¡£
- ¤È¤¤ËN/CÈæ¤¬¹â¤¤ºÙ˦¼Á¤Î˳¤·¤¤¾®·¿¤Î²êµå¤Ç¡¢¥ê¥ó¥Ñ²êµå¤È¤Î´ÕÊ̤¬º¤Æñ¤Ç¤¢¤ë¡£
- ¹ü¿ñ¥¹¥á¥¢¤Çµð³Ë²êµå¤¬ºÙ˦²ô¤ò¤Ä¤¯¤ë¤è¤¦¤Ë¶Å½¸¤·Å¾°Ü¼ðáçºÙ˦¤Ë¤è¤¯»÷¤ë¤³¤È¤¬¤¢¤ë¡£
- MPO, naphtolAS-DCAE, SudanB¤Ï¤Ä¤Í¤Ë±¢À¡£
- PASÀ÷¿§, AcP(acid phosphatase)À÷¿§¤¬ÍÛÀ¤È¤Ê¤ë¤³¤È¤¬¤¢¤ë¡£
- ÈóÆÃ°ÛŪ¥¨¥¹¥Æ¥é¡¼¥¼¤¬ºÙ˦¼Á¤ËÈÃÅÀ¾õ¤¢¤ë¤¤¤ÏÉôʬŪ¤ËÍÛÀ¤Î¤³¤È¤¬¤¢¤ë¡£
¹ü¿ñÀ¸¸¡ÁÈ¿¥

1. ²êµåÁýÀ¸¤ÎÃøÌÀ¤Ê¿ñÍÍÁÈ¿¥¤ò¤·¤á¤¹megakaryoblastic leukaemia¤È
2. ²êµå¤ÎÁý¿£¤¬Ë³¤·¤¯, myelofibrosis¤Î¤ß¤¬¤á¤À¤Ä£²¤Ä¤Î¥¿¥¤¥×¤¬¤¢¤ë¡£
- ¹ü¿ñÀ¸¸¡ÁÈ¿¥½ê¸«¤Ï¿ºÌ¤ÊÁü¤ò¤·¤á¤¹¡£µÞÀ¥ê¥ó¥ÑµåÀÇò·ìɤΤ褦¤ÊÁü¤«¤é¡¢Å¾°Ü¼ðáç¤Î¤È¤¤Ë¸«¤é¤ì¤ë¤è¤¦¤Ê¹ÈϰϤÊÀþ°Ý²½¤Þ¤Ç¤¢¤ë¡£°ìÈÌŪ¤Ë¤Ï¾®»ùÎã¤Ç¤ÏºÙ˦¤Ï°ìÍͤǤ¢¤ë¤³¤È¤¬ÂçÊÑ¿¤¤¡£
- ¹ü¿ñÀþ°Ý²½¤Ï t(1;22)¤ò¤â¤Ä¿·À¸»ù acute megakaryoblastic leukaemia¤ËÆÃħŪ¤Ê¤â¤Î¤Ç¤Ï¤Ê¤¯µð³ËµåÀ®Ê¬¤ÎÃøÌÀ¤Ê¤É¤ÎÇò·ìɤˤâÉáÄ̤Ëǧ¤á¤é¤ì¤ë¡£
- Àþ°Ý²½¤Ë¤è¤ê¡Êµð³Ë²êµå¤¬»ºÀ¸¤¹¤ëfactor¤Ë¤è¤ë¤È¿ä¬¤µ¤ì¤Æ¤¤¤ë)¹ü¿ñÀü»ÉµÛ°ú¤ÏÉÔÀ®¸ù¤Î¤³¤È¤¬Â¿¤¯, ¿ÇÃǤ˹ü¿ñÀ¸¸¡¤¬É¬ÍפȤʤ롣
- ǯĹ»ù¤«¤éÀ®¿Í¤Îmegakaryoblastic leukaemia¤Ç¤Ï¿·ÏÅý¤Î°Û¾ï¤¬¤è¤¯Ç§¤á¤é¤ì¤ë¡£
- myeloblast¤¬Áý¿£¤·Auer rod¤òǧ¤á¤ë¤³¤È¤¢¤ê¡£
- ðùγµå·Ï¤·ì¤¬Ãø¤·¤¯°Û·Á¤Ê¤³¤È¤¬¤¢¤ê¡¢Æ±ÍͤËÀÖ²êµå·Ï¤Ë¤â°Û·ÁºÙ˦¤ÎÁý²Ã¤¬¤è¤¯Ç§¤á¤é¤ì¤ë¡£
phenotype
ÍÛÀ¥Þ¡¼¥«:
- CD41, CD61(µð³Ëµå¥Þ¡¼¥«), CD42b*1, CD34(À®½Ïµð³Ëµå¤ËÀ÷¤Þ¤ë¤³¤È¤¬¤¢¤ë), CD36, factor VIII.
- ÍÛÀ¤Ë¥Ð¥é¥Ä¥¤¢¤ê; CD13, CD33, CD71, alpha naphthyl acetate esterase, PAS and HLA-DR
- alpha-1-antitrypsin, alpha-1-antichymotrypsin, lysozyme¤¬¤Þ¤ì¤ËÍÛÀ¤ò¼¨¤¹.*2
±¢À¥Þ¡¼¥«:
- Myeloperoxidase, Sudan Black B, CD14, CD64, glycophorin A
Case 01 †
70ºÐÂå¡¡½÷À.
Aɱ¡¤Ë¤Æ, 4ǯÁ°¤ËÊ¢ÉôÂçÆ°Ì®²òÎ¥ÀÂçÆ°Ì®áî¤Ë¤è¤ê¿Í¹©·ì´ÉÃÖ´¹½Ñ»Ü¹Ô¤·°Ê¸å·Ð²á´Ñ»¡Ãæ. ÈÆ·ìµå¸º¾¯, ¿´ÉÔÁ´¤ÇÆþ±¡. Hb 5.7x104 WBC1370/¦Ìl, plt1.8x104. Ëö¾¿·ì²êµå¡Ü.
¹ü¿ñÀü»É¤Ïdry tap¤Ç¤¢¤Ã¤¿. ¾Ò²ð¤µ¤ì¹ü¿ñÀ¸¸¡¤ò¹Ô¤¦.
 |  |  |  |
ASD-Giemsa x40 | ASD-Giemsa x40 | ÅÕ¶ä x40 | ÅÕ¶ä x40 |
Acute panmyelosis with myelofibrosis (APMF) †
WHO 2017 4th Ed¤ÎÄêµÁ
µÞÀ¤ÎÈÆ¹ü¿ñÀÁý¿£¾Épanmyeloid proliferation¤Ç²êµå¤¬Áý²Ã¤·(¹ü¿ñ¤¢¤ë¤¤¤ÏËö¾¿·ìºÙ˦¤Î¡æ20%) ¹ü¿ñÀþ°Ý¾É¤ò¹çÊ»¤¹¤ë.
¡ü È¿Éü¤¹¤ë°äÅÁ»Ò°Û¾ï¤ò¼¨¤¹AML
¡ü Acute myeloid leukaemia with myelodysplasia-related changes
¡ü ¼£ÎÅ´ØÏ¢AML
¤¤¤º¤ì¤Ë¤â´Þ¤Þ¤ì¤º, ¾åµ¥°¥ë¡¼¥×¤Î¤¤¤º¤ì¤Î¥¯¥é¥¤¥Æ¥ê¥¢¤âËþ¤¿¤µ¤Ê¤¤.
- acute megakaryoblastic leukaemia(M7)¤ÈÎ×¾²Áü, ·ì±ÕÉÂÍý½ê¸«¤¬ÉôʬŪ¤ËOverlap¤¹¤ë.
APMF¤ÏÈó¾ï¤Ë¤Þ¤ì¤ÊAML¤Î°ì·ÁÂÖ¤Ç, de novo¤Î¼À´µ. ´ðËÜŪ¤Ë¤ÏÀ®¿Í¤Î¼À´µ¤Ç¤¢¤ë¤¬, ¾®»ù¤ÎÊó¹ð¤â¤¢¤ë.
¿ê¼å¤äÈèÏ«´¶¤ÇµÞ·ã¤Ëȯɤ¹¤ë. ¿¤¯¤Ï, ȯǮ¤ä¹üÄˤò¤¤¿¤¹. Ä̾ïÁ´¿È¾õÂ֤ε޷ã¤Ê°²½¤¬¤ß¤é¤ì¤ë.
ÈÆ·ìµå¸º¾¯¾É¤¬¾ï¤Ë½Ð¸½¤·, 磼ð¤Ïǧ¤á¤é¤ì¤Ê¤¤¤«¤´¤¯·ÚÅ٤Ǥ¢¤ë.
Ëö¾¿·ì
- ¸²Ãø¤ÊÈÆ·ìµå¸º¾¯¾É¤òÄ褹¤ë.
- ÀÖ·ìµå¤Ïanisopoikilocytosis¤Ï¤Ê¤¤¤«, ¤Þ¤ì. ¼ï¡¹¤ÎÄøÅÙ¤Îmacrocytosis¤¬Ç§¤á¤é¤ì¤ë.
ÀÖ²êµå½Ð¸½¤Ï¤Þ¤ì¤Ëǧ¤á¤é¤ì¤ë¤¬, teardrop-shaped cells(dacrocytosis)¤Ï´Ñ»¡¤µ¤ì¤Ê¤¤.
- ¹¥ÃæµåÁ°¶îºÙ˦, ²êµå¤¬¤·¤Ð¤·¤Ð½Ð¸½¤¹¤ë.
ðùγµå·ÏºÙ˦¤Î°Û·ÁÀ®¤¬¹âÉÑÅ٤ˤߤé¤ì¤ë¤¬, AML with myelodysplasia-related changes¤Î¥¯¥é¥¤¥Æ¥ê¥¢¤Ë¤ÏŬ¹ç¤·¤Ê¤¤.
- °Û¾ï¤Ê·ì¾®ÈĤ¬¤è¤¯Ç§¤á¤é¤ì¤ë.
¹ü¿ñ½ê¸«
- ¹ü¿ñµÛ°ú¤Ï¤·¤Ð¤·¤Ð¤¦¤Þ¤¯¤¤¤«¤Ê¤¤(dry tap)¤¿¤á, ¹ü¿ñÀ¸¸¡¤ÈÌȱÖÀ÷¿§¤¬¿ÇÃǤËɬÍפǤ¢¤ë.
- ²á·ÁÀ®¿ñ¤Ç, ¤Ó¤Þ¤óÀ¤ÎÀþ°ÝÀ´Ö¼Á¤¢¤ê. 3·ÏÅý¤·ìºÙ˦¤ÎÁý¿¤¬Ç§¤á¤é¤ì¤ë;panmyelosis¤È¤è¤Ó, ³Æ·ÏÅý¤ÎÁêÂÐŪÁý²ÃÅ٤Ϥ¤¤í¤¤¤í¤Ç¤¢¤ë.
- ÆÃħŪ¤Ê½ê¸«¤Ï²êµå¤Î½¸äÈÁ㤬, ¹¥»ÀÀ¤ÎºÙ˦¼Á¤ò¤â¤Ä¾®·¿Í¥°Ì¤Ê, °Û·ÁÀ®µð³Ëµå¤È¤È¤â¤Ëǧ¤á¤é¤ì¤ë¤³¤È¤Ç¤¢¤ë.
- °Û·ÁÀ®Mgk¤ÏÄãʬÍճˤä ʬÍÕ¤·¤Ê¤¤¥¯¥í¥Þ¥Á¥ó¤ÎÁ¤ʳˤò¤â¤ÄºÙ˦¤È¤·¤Æ³Îǧ¤Ç¤¤ë. microMgk¤¬Áý¤¨¤ë¤¬²êµå¤È¤Þ¤Á¤¬¤¨¤Æ¥«¥¦¥ó¥È¤·¤Ê¤¤¤³¤È¤¬ÂçÀڤǤ¢¤ë.
- small/ microMgk¤Î³Îǧ¤Ë¤ÏPASÀ÷¿§¤ä, ÌȱÖÀ÷¿§ (CD42b, CD61, CD41¾)¤¬ÍÍѤˤʤë.
- APMF¤Î¹ü¿ñ²êµåÁý²Ã¤ÎÄøÅ٤Ϥµ¤Þ¤¶¤Þ¤Ç¤¢¤ë. Ãæ±ûÃÍ 22.5%¤È¤¹¤ëÊó¹ð¤¬¤¢¤ë. ¿¤¯¤ÎÎã¤Ç¤Ï20-25%.
- ¹ü¿ñÀþ°Ý²½¤ÎÄøÅ٤⤵¤Þ¤¶¤Þ¤Ç¤¢¤ê, ÅÕ¶äÀ÷¿§¤Ç¹õ¿§¤ÎÁ¡ºÙ¤ÊÃÆÀÀþ°ÝÁý²Ã¤¬¸«¤é¤ì¤ë. 籸¶Àþ°ÝÁý¿¤ÏÃÆÀÀþ°ÝÁý²Ã¤Ë¤¯¤é¤ÙÉÑÅÙ¤¬Ä㤤.
immunophenotype ÌȱÖÀ÷¿§
ºÙ˦¤¬ºÎ¼è²Äǽ¤Ê¾ì¹ç¤Ï, ²êµå¤ÏCD34, C-KIT, CD33, CD13¤¬¼ï¡¹¤ÎÄøÅÙ¤ËÍÛÀ¤ò¼¨¤¹. MPO¤ÏÄ̾ﱢÀ¤Ë¤Ê¤ë.Naphtol-ASD-CAE¤âÉÔÀ÷.