¼±Åà - È­»ìǥŰ/¿£ÅÍŰ ´Ý±â - ESC

 

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 À̸鼭 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