Acute leukemia ´ëÀå°æ Á¤¸®
1. acute leukemia·Î ÁøÇàÇÒ¼ö ÀÖ´Â Áúȯ
MDS, PNH, PV, AA, AIDS
2. secondary leukemia
radiation(5-7³âÈÄ) : AML, ALL, CML¸ðµÎ Áõ°¡, ¿¹¿Ü> CLL, hairy cell leukemia´Â
°ü°è¾ø´Ù.
alkylating agent: AML(2-3³âÈÄ), chromosome 5, 7 aberration
topoisomerase II inhibitors(1-3³âÈÄ), chromosome 11q23
3. CD 13, 33¸¸ ¾ç¼ºÀÎ °Í: AML, M0
4. acute leukemiaÀÇ Áø´Ü¹æ¹ýÁß ¿¹Èĸ¦ ¾Ë±âÀ§ÇÑ °¡Àå ÁÁÀº ¹æ¹ý: chromosomal study
5. cytochemical stain
myelperoxidase, SBB : M2-3
NSE : M4,5
PAS : M6,7, ALL
6. immunophenotype
CD 19, 20 : B cell ALL
CD 2, 5, 7 : T cell ALL
CD 13, 14, 15, 33 : myeloid
CD 10 : pre-B ALL(CALLA)
7. chromosomal classification: ¿¹ÈÄ¿¡ °¡Àå Áß¿ä
AML M2: t(8;21), CBF¥á gene: AML1/ETO
AML M3: t(15;17) PML/RAR¥á gene
AML M4E: inv(16) CBF¥â gene
AML M5: translocation involving 11q23 ALL L1(MLL) gene
ALL, CML¿¡¼ÀÇ t(9;22) bcr/abl gene
Burkitt's lymphoma: t(8;14) myc gene
follicular lymphoma: t(14;18) bcl-2 gene
8. AML M4EÀÇ Æ¯Â¡
organomeglay, leukocytosis°¡ ÈçÇÏ´Ù.
Áø´Ü´ç½Ã CNS disease°¡ ÈçÇÏ´Ù.
inv(16), CNS prophylaxis°¡ ÃßõµÈ´Ù.
9. acute leukemiaÀÇ CR±âÁØ
1) PB: neutrophil>1500, plt>10¸¸, blast(-)
2) BM: cellularity > 20% with trilineage maturation
blast<5%, Auer rod(-)
3) extramedullary involvement(-)
ÀÌ»óÀÇ Á¶°ÇÀÌ 4ÁÖÀÌ»ó Áö¼ÓµÉ ¶§ CRÀ̶ó ÇÔ
10. AML¿¡¼ inv(3)Àº ¿¹Èİ¡ ¸Å¿ì ³ª»Ú´Ù. ÀϹÝÀû Ç׾Ͽä¹ýÀ¸·Î´Â CRÀÌ µÇÁö ¾Ê´Â´Ù.
11. AMLÀÇ ¿¹ÈÄ¿Í °ü·ÃÇÑ »çÇ×µé
secondary leukemia(MDS, PNHÈÄ, ¹æ»ç¼±, Ç×¾ÏÁ¦ °ü·Ã)´Â ¿¹Èİ¡ ³ª»Ú´Ù.
CD34 expressing myeloblast´Â ¿¹Èİ¡ ³ª»Ú´Ù.
CD13, 14¿Í CD 34´Â co-expressÇÏ´Â °æ¿ì´Â ¿¹Èİ¡ ³ª»Ú´Ù.
12. AML¿¡¼ CRÈÄ °íÀ§Ç豺Àº BMTÇϵµ·Ï Çϰí, ÀúÀ§Ç豺 ¹× Áß°£À§Ç豺Àº
high dose ara-C CTxÇϵµ·Ï ÇÑ´Ù.
ÀúÀ§Ç豺 ¹× Áß°£À§Ç豺¿¡¼ high-dose ara-C therapyÈÄ¿¡ ¹ÝÀÀÀÌ ¾øÀ¸¸é
allo- or autologous BMT¸¦ °í·ÁÇϵµ·Ï ÇÑ´Ù.
13. catheter-related infection
exit-site infectionÀÌ tunnel infectionº¸´Ù ´õ ÈçÇϸç
¿øÀαÕÀÌ coagulase-negative Staphylococcal infectionÀ϶§´Â vancomycinÀ» »ç¿ëÇϰí
coagulase-positive Staphylococcus, P. aeruginosa, CandidaÀ϶§´Â catheter¸¦ Á¦°Å
ÇØ¾ß ÇÏ¸ç ¿¹Èİ¡ ³ª»Ú´Ù.
14. Typhlitis ¿øÀαÕ: G(-) bacteria, ƯÈ÷ pseudomonas aeruginosa
15. AML M3 pathogenesis
t(15;17)¿¡ ÀÇÇØ PML gene°ú RAR¥á geneÀÌ ÇÕÃÄÁ® PML/RAR¥á proteinÀÌ Çü¼ºµÇ´Âµ¥
À̰ÍÀÌ ATRA Ä¡·áÀÇ targetÀÌ µÈ´Ù.
ATRA´Â ÀÌ ´Ü¹é°ú °áÇÕÇÏ¿© ¼¼Æ÷ºÐȸ¦ ÃËÁøÇÑ´Ù.
16. AML M3¿¡¼ÀÇ ATRA
coagulationÀå¾Ö³ª BM aplasia¸¦ ÀÏÀ¸Å°Áö ¾Ê´Â´Ù.
óÀ½ Áø´ÜµÇ¾ú°Å³ª óÀ½ Àç¹ßÇÑ M3¿¡¼ CR rate´Â ³ôÀ¸¸ç
Ä¡·á 30-80ÀÏÈÄ M3 cellÀÇ ÆÄ±«°¡ ¾Æ´Ñ ºÐȸ¦ ÃËÁø½ÃÅ´À¸·Î½á CRÀ» À¯µµÇÑ´Ù.
CR rate´Â ³ôÁö¸¸ ´Üµ¶À¸·Î cure½Ãų¼ö´Â ¾øÀ¸¹Ç·Î consolidation chemotherapy°¡
ÇÊ¿äÇÏ´Ù.
17. ATRA, CTx, BMTµîÀÌ ¸ðµÎ ½ÇÆÐÇßÀ» ¶§ ½ÃµµÇغ¼¼ö ÀÖ´Â Ä¡·á? Arsenic trioxide(As2O3)
(apoptosis°¡ ÁÖ¿ä±âÀü)
18. AML¿¡¼ minimal residual disease¸¦ ditectionÇÒ¼ö ÀÖ´Â ¹æ¹ý?
RT-PCR, FISH(fluorescence in situ hybridization), flow cytometry, southern blot
19. ALL : ¼ºÀο¡¼´Â L2°¡ ÈçÇϰí, ¼Ò¾Æ¿¡¼´Â L1ÀÌ °¡Àå ÈçÇÏ´Ù.
20. ALL L1,2 : T cell ALL, ³²ÀÚ¿¡°Ô ¸¹´Ù. CNS & mediastinal disease
ALL L3: B cell ALL, extramedullary presentation with metabolic abnormality
tumor lysis syndromeÀÌ Àß »ý±ä´Ù.
21. ALL, extramedullary relapse
CNS, testis¿¡ ÁÖ·Î ÀϾ¸ç, radiation & intrathecal CTx or
RTxÈÄ reinduction(systemic relapse¹æÁö)
¹®> CRÈÄ testis relapseÇßÀ»¶§ÀÇ Ä¡·á? testis RTÈÄ systemic chemotherapy
22. adult ALLÀÇ poor prognostic factors
old age(>60), delay in achieving CR(>4-6ÁÖ)
B cell ALL(L3), high WBC > 50,000
cytogenetic abnormality; t(9;22), t(8;14), t(1;19), t(4;11)
23. ALL¿¡¼ ¼ºÀÎÀÌ ¼Ò¾Æº¸´Ù ¿¹Èİ¡ ³ª»Û ÀÌÀ¯?
myeloid antigen¹ßÇöºóµµ°¡ ³ô´Ù.
philadelphia chromosomeºóµµ°¡ ³ô´Ù.
Àç¹ß½Ã¿¡ MDR-1 gene¹ßÇöÀÌ ³ô´Ù.
** hyperdiploidy´Â ¾Æ´Ô(hyperdiploidy = good prognostic factor)
24. ALL¿¡¼ allo BMT indication
induction failure, 2nd remission after hematologic relapse
BCR/ABL gene(+), MLL(+)¿¡¼ 1st remissionÈÄ
25. ALL¿¡¼ °¡Àå ÈçÇÑ ¿°»öü ÀÌ»ó
¾î¸¥: t(9;22) ¾ÆÀÌ: t(12;21)
26. ALL°ú CMLÀÇ philadelphia chromosomeÀÇ Â÷ÀÌ
ALL: 190 kd, CML: 210 kd
27. intracerebral leukocytostasis(Ball's disease)
blast°¡ 100,000/ugÀÌ»óÀÏ ¶§ Àß »ý±è
blood viscosity°¡ Áõ°¡Çϰí, leukemic cellÀÌ endotheliumÀ» invasionÇÏ¿©
cerebral hemorrhage¸¦ ÀÏÀ¸Å´.
brain RT(600 cGy)·Î ³úÃâÇ÷ ¿¹¹æ
* CLLÀ̳ª CML¿¡¼´Â ¹éÇ÷±¸ ¼ö°¡ ¸¹¾Æµµ µå¹°´Ù.
28. intracerebral leukocytostasisÄ¡·á: leukapheresis, hydroxyurea(>3g/m2), brain RT