Aplastic anemia ´ëÀå°æ Á¤¸®
1. Ç÷¼ÒÆÇ È£ÀüÀ» ±â´ëÇÒ¼ö ÀÖ´Â ¼ºÀåÀÎÀÚ: IL-11, thrombopoietin
2. pancytopenia + marrow hypercellularity¸¦ º¸ÀÌ´Â Áúȯ(°´)
MDS, PNH, hypersplenism, vit B12, folate deficiency, AIDS
3. acquired aplastic anemiaÀÇ 2 main mechanisms
i) acquired intrinsic stem cell defect
ii) immune mediated mechanism
4. ¾àÁ¦¿Í °ü·ÃÇÑ aplastic anemia
dose-related: anitneoplastic drug, antimetabolites, sulfonamide
idiosyndratic: chloramphenicol, phenylbutazone, oxyphenbutazone, indomethacin, gold
5. aplastic anemia¿Í hypocellular MDSÀÇ °¨º°Áø´Ü¿¡ Áß¿äÇÑ °Í?
i) BM - dysplastic changeÀ¯¹«
ii) clonal chromosomal abnormalityÀ¯¹«
6. A.A¿¡¼ leukocyte infusionÇÏ´Â °æ¿ì?
ANC ¡Â 200 ÀÌ¸é¼ Ç×»ýÁ¦, amphotericin¿¡ ¹ÝÀÀÇÏÁö ¾Ê´Â infection
7. A.A¸¦ ÀÏÀ¸Å°´Â ¿øÀÎÁß °¡Àå ÈçÇÑ °¨¿°¼º ¿øÀÎ? °£¿°
8. iron overload¶§ ¾²´Â chelating agent? deferoxamine
9. AA¿¡¼ Ç÷¾×¼öÄ¡ÀÇ È£ÀüÀ» ÀϺο¡¼ ±â´ëÇÒ¼ö ÀÖ´Â Ç÷¾×¼ºÀåÀÎÀÚ?
IL-1,3,6, G-CSF, GM-CSF
10. AAÄ¡·áÁß BMT¸¦ ½ÃÇàÇÏÁö ¸øÇÒ ¶§ÀÇ ÃÖ¼±ÀÇ Ä¡·á?
immunosuppressive tx(ATG, cyclosporin A)
11. ATG side effect: anaphylaxis, cytopenia, serum sickness
cf. ATGÀÇ long-term complications: MDS, PNH, acute leukemia, secondary solid tumor
(¾Ï±â: MAPs, ¸ðÀÚ¾´ ¾ÆÆ®°¨)
12. ATG Ä¡·á
¼ºÀÎÀÌ ¼Ò¾Æº¸´Ù ¹ÝÀÀÀÌ ÁÁ´Ù.
very severe AA´Â ¹ÝÀÀ·üÀÌ ³·´Ù.
cyclosporin°úÀÇ º´ÇÕ¿ë¹ýÀÌ ´Üµ¶¿ä¹ýº¸´Ù ¼ºÀûÀÌ ÁÁ´Ù.
13. ¸é¿ª¾ïÁ¦Ä¡·á½Ã Ä¡·áÈÄ 2ÁÖÀÌÈÄ¿¡ È¿°ú°¡ ³ªÅ¸³´Ù.
ANC < 200ÀÏ ¶§ G-CSF¿¡ ´ëÇÑ ¹ÝÀÀÀº ³·´Ù.
14. hereditary spherocytosisÀÇ aplastic crisis: parvovirus B19 -> IV immunoglobulin tx
15. pancytopenia + marrow hypocellularity¶§ °¨º°
AA, hypocellular MDS, fanconi's anemia, some lymphoma of BM, hypocellular acute
leukemia
16. MDSÀÇ °æ°ú ¹× ¿¹ÈÄ¿¡ Áß¿äÇÑ 3°¡Áö ¿ä¼Ò
: cytopenia severity, blast percentage, karyotype
cf. cytopenia: Hb <10 g/dL, ANC < 1500/ul, Plt < 100,000/ul
17. MDSÀÇ ¿øÀÎ
i) genetic factor: Down's syndrome, Fanconi's anemia, neurofibromatosis
ii) radiation
iii) chemical: benzene, alkylating agent
iv) aplastic anemia¿¡¼ ¸é¿ª¾ïÁ¦Ä¡·áÈÄ
18. MDSÀÇ good karyotype: normal, Y-, 5q-, 20q-
poor karyotype: complex(3°³ÀÌ»ó) anomaly, chr7 abnormality
19. MDS Ä¡·á
i) supportive tx: transfusion, iron chelating agent(deferoxamine)
ii) hematopoietic growth factor
iii) vit therapy
iv) low dose chemotherapy: topotecan, ara-C
20. 5q- syndrome
70%°¡ ¿©¼ºÀÌ¸ç ºóÇ÷À» ÁÖ ¹®Á¦ÀÎ benign courseÀÇ MDS.
¼öÇ÷ ¹× iron overloadÄ¡·á°¡ ÁÖµÈ Ä¡·áÀÌ´Ù.
21. BM iron stain»ó ¿°»öµÇ´Â ¹°ÁúÀÌ ¾øÀ» ¶§ °¡´ÉÇÑ Áúȯ?
IDA, PNH, polycythemia vera
cf. AA´Â ¾Æ´Ô