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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÷ºÐÈ­¸¦ 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 ºÐÈ­¸¦ 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