ABSTRAK
Myelodysplastic Syndrome MDS adalah kelainan klonal sel p o enito hematopoietik an ditandai den an sitopenia. tiolo i pasti MDS
masih bel m diketah i sepen hn a tetapi hasil penelitian klinis dan labo ato i m men n kkan adan a pe an espons im n. e bahan asio
CD4 : CD se in ditem kan pada peme iksaan labo ato i m dan penelitian molek la enetik men n kkan tin in a kada sel sitotoksik.
Mani estasi a toim n a didapatkan pada pasien MDS hal ini did a ka ena penin katan p od ksi sitokin p oin lamasi.
ABSTRACT
M elod splastic s nd ome MDS is an ac i ed clonal diso de o haematopoietic p o enito cells cha acte i ed b c topenia. he etiolo o
MDS is still nknown b t clinical and labo ato t ials had shown the ole o imm ne s stem. lte ation o CD4 : CD atio is the most e ent
labo ato indin and enetic molec la st d had shown hi h level o c totoxic -cell. toimm nit o nd in MDS p obabl ca sed b
excessive p od ction o p oin lammato c tokine. Danny Jaya Jacobus. Autoimmune Process in Myelodysplastic Syndrome (MDS)
si ni ikan.19 Ka ena it lah m lai be kemban sel le itas s ms m t lan pada pasien an diketah i pen ebabn a. ebe apa hipotesis
teo i bahwa mediasi sel dapat memic tidak dite api akan te adi pe sistensi espons men atakan te dapat an an sistem im n
inhibisi s ms m t lan pada pasien MDS im n sel . Data ekspe imental men imp lkan an salah sat n a ka ena p od ksi sitokin
an men ebabkan te adin a sitopenia pada bahwa ind ksi apoptosis oleh sel t isomi p oin lamasi be lebihan. Seba ian besa
20-30 pasien.20 an ditandai penin katan aktivitas caspase o an ta et a toim n pada MDS adalah
dan C -2 adalah nt k mence ah p oses o an non-hematopoietik.29 illst om et al,
kspansi klonal pe sisten tidak selal apoptosis den an bant an molek l survivin dan Cast o et al, melapo kan 10 pasien
men n kkan p oses mali nansi hal ini bisa Gambar .2 Implikasi klinisn a adalah MDS memiliki mani estasi e matoid sepe ti
a men adi amba an ekspansi klonal pasien MDS setelah te api im nos p esi vask litis sistemik ak t vak litis k lit demam
ata p n oli oklonal da i sel a to eakti akan men alami pen nan kada sel a t itis in ilt at p lmonal poline opati pe i e
dan se in di mpai pada pop lasi sel CD . sitotoksik an akan meno malisasi s ms m inflammatory bowel disease lome lone itis
enin katan eksp esi CD5 oleh sel CD t lan dan mence ah espons im n sel dan bebe apa pen akit a in an ikat lain.30 31
di mpai a pada eksp esi CD5 oleh sel an men ebabkan sitopenia sedan kan akto p edisposisi te adin a abno malitas
C an men adi ka akte istik pen akit sitopenia menin katkan p o esivitas MDS di atas did a ka ena dis n si sistem im n
an dise tai aktivasi sistem im n sepe ti pada men adi le kemia. ada penelitian dib ktikan an ditem kan te mas k men nn a
kondisi acute graft vs host disease skle osis te api im nos p esi men nkan an ka aktivitas sel NK natural killer an an
m ltipel dan a t itis e matoid.21-23 ada p o esivitas men le kemia.2 antibody dependent cell be k an n a mlah
kondisi a toim n ekspansi sel men e pai sel CD4 dan an an n si sel se ta kada
p oli e asi dominan s b amili C im no lob lin an endah.32
men n kkan espons spesi ik sel a to eakti
te hadap anti en te tent . enem an te akhi SIMPULAN
adan a ekspansi klonal sel sitotoksik pada MDS den an mani estasi a toim n
pasien MDS mem nc lkan hipotesis bahwa me pakan s at kelainan an dimediasi oleh
MDS dihasilkan da i eaksi a toim n seca a mekanisme im nolo i oleh sel sitotoksik.
lan s n te hadap sel s ms m t lan . Mekanisme a toim n men ebabkan
Selain it a mem nc lkan d aan bahwa te adin a hiposel le itas s ms m t lan an
espons im n mencet skan e ekto lim osit men ebabkan sitopenia. e api im nos p esi
melal i eksp esi onko en dan si en pada Gambar. ipotesis ind ksi espons im n pada MDS dapat menekan p oses a toim n dan
dan seleksi sel t isomi . Sel t isomi men alami
sel p o enito hematopoietik ata melal i mempen a hi p o esivitas MDS. Meskip n
penin katan eksp esi pada anti en te tent . Sel
de ivat s pe anti en bakte i ata p n vi s.24 25 sitotoksik an men enali anti en akan men alami ban ak akta men enai mekanisme a toim n
ekspansi. poptosis dipic oleh sel t isomi akan pada MDS pen ebab pasti p oses a toim n
Selain it h b n an MDS den an t isomi tetapi mendapatkan hambatan da i caspase ka ena pada MDS masih bel m diketah i pasti
te dapat eksp esi molek l survivin.2
an dideteksi den an metode IS sehin a memb t hkan penelitian lebih
fluorescent in situ hybridization men n kkan lan t.
aktivitas sel sitotoksik te hadap sel t isomi FENOMENA AUTOIMUN PADA PASIEN MDS
di s ms m t lan .26 Setelah te api Mani estasi a toim n an be va iasi pada
im nos p esi akan te adi no malisasi st kt pasien MDS sampai saat ini masih bel m
DAFTAR PUSTAKA :
1. o mann K bbe t M oel e D hillip K . M elod splastic s nd omes. emtol . 2004 5 1 : 1- .
2. lbita M Mansho i Shen i D e an M Kata ian M et al. M elod splastic s nd ome is not me el p ele kemia. lood 2002 100: 91- .
3. ckstein ells . M elod splastic s nd ome MDS Inte net . 200 . vailable om: http://s nn b ook.ca/ ploads/M elod splastic S nd omes.pd
4. Molld em Caples M Mav o dis D lante M o n NS a ett . ntith moc te lob lin o patients with m elod splastic s nd ome. aematol. 199 99:
690- 05.
5. Killick S M ti Cavena h D Mi ovic eacock o don-Smith C et al. pilot st d o antith moc te lob lin in the t eatment o pastient with low-
isk m elod splasia. aematol. 2003 120:6 9- 4.
6. oshida . potesis: poptosis ma be the mechanism esponsible o the p emat e int amed lla cell death in the m elod splastic s nd ome. e kemia
1993 :144-6.
. a a e e S M ndle S ao X lvi S o ok et al. poptosis in bone ma ow biops samples involvin st omal and hematopoietic cells in 50 patients with
m elod splastic s nd omes. lood 1995 6:26 - 6.
. a apaksa in ton N ott S eenbe . lte ed oncop otein exp ession and apoptosis in m elod splastic s nd ome ma ow cells. lood 1996 11:42 5- .
9. ose sen D M kleb st nch D Stokke lomho K Smeland . as li and p omotes cell s vival o immat e h man bone ma ow CD34 CD3 -
hematopoietic p o enito cells b s pp essin apoptosis. xp ematol. 1999 2 :1451-9.
10. M ndle SD Mativi a ai K eldman Cheema a tam et al. Spontaneo s down- e lation o as-associated phosphatase-1 ma cont ib te to excessive
apoptosis in m elod splastic ma ows. Int ematol. 1999 0: 3-90
11. andowski leason- man MC Dalton S. Selection o d esistance es lts in esistance to as-mediated apoptosis. lood 199 9: 1 54-61.
12. Stadle M e min Kliche K osten KM K se o mann K et al. p ospective andomi ed phase II st d o ho se antith moc te lob lin vs abbit
antith moc te lob lin as imm ne-mod latin the ap in patients with low- isk m elod splastic s nd omes. e kemia 2004 1 : 460-5.
13. Ko dasti S In am a den Da lin D a be ali et al. CD4 CD25 hi h ox 3 e lato cells in m elod splastic s nd ome MDS . lood 200 110:
4 -50.
14. a ett Sa ntha a a ah Molld em . M elod splastic s nd ome and aplastic anemia: Distinct entities o diseases linked b a common pathoph siolo Semin
ematol. 2000 3 : 15-29.
15. Ma tine - a amillo lo es- i e oa ome -Mo ales Sanche - alle Ma ani . mo nec osis acto -alpha levels in lon -te m ma ow c lt es om patients
with aplastic anemia: Mod lation b an loc te-mac opha e colon -stim latin acto . m ematol. 2001 6 :144- .
16. o n NS Macie ewski . he pathoph siolo o ac i ed aplastic anemia. N n Med. 199 336:1365- 2.
1 . Sloand Kim S Macie ewski isdale ollmann D o n NS. Int acell la inte e on- amma in ci c latin and ma ow -cells detected b ow c tomet and
the esponse to imm nos pp essive the ap in patients with aplastic anemia. lood 2002 100: 11 5-91.
1 . Smith M Smith . he occ ence s bt pe and si ni cance o haemopoietic inhibito cells I cells in m elod splasia: n in vit o st d . e k es 1991 15:59 -
601.
19. Molld em ian Stetle -Stevenson M Mav o dis D ensel N a ett . aematolo ical esponse o patients with m elod splastic s nd ome to antith moc te
lob lin is associated with a loss o l mphoc te-mediated inhibition o C - M and alte ations in -cell ecepto beta p o les. aematol. 199 102 5 : 1314-22.
20. Sloand M C eenbe o n NM a ett . acto s a ectin esponse and s vival in patients with m elod splasia t eated with imm nos pp essive
the ap . Clin ncol. 200 26:2505-11.
21. on an C e ada-Simon M Ko ovska M i S Sin h et al. common C -D- se ence in beta 13.1 cells eco ni in an imm nodominant peptide
o m elin basic p otein in m ltiple scle osis. Imm nol. 1999 163:3530- .
22. Mima hshima S Sasai M Nishioka K Shimi M M ata N et al. Dominant and sha ed cell ecepto beta chain ba iable e ions o cells incl din s novial
h pe plasia in he matoid a th itis. iochem ioph s es Comm n. 1999 263:1 2- 0
23. Diet ich m Cai na d im Ch n ico annetie C et al. In vivo -cell clonal ampli cation at time o ac te a t-ve s s-host disease. lood 1994 4:2 15-
20.
24. ppe son D Nakam a Sa ntha a a ah Melenho st a ett . li oclonal cell expansion in m elod splastic s nd ome: vidence o an a toimm ne
p ocess. e k es. 2001 25:10 5- 3.
25. a a . potesis: M elod splastic s nd omes ma have a vi al etiolo . Int aematol. 199 6 :245-56.
26. Sloand M Mainwa in h e M amkissoon S isitano M Ke vana a K et al. e e ential s pp ession o t isom compa ed with no mal hematopoietic cell
owth b a tolo o s l mphoc tes in patients with t isom m elod splastic s nd ome. lood 2005 106: 41-51.
2 . Sloand M annes Chen Shah S Solomo a ett et al. CD34 cells om patients with t isom m elod splastic s nd ome MDS exp ess ea l apoptotic
ma ke s b t avoid p o ammed cell deth b p- e lation o antiapoptotic p oteins. lood 200 109:2399-405.
2 . Sloand M C eenbe o n N a ett . acto a ectin esponse and s vival in patients with m elod splasia t eated with imm nos pp essive the ap .
Clin ncol. 200 26:2505-11.
29. o l a elis M ianno li S itis K io as . M elod splasia-associated a toimm nit : Clinical and pathoph siolo ic concepts. Clin Invest. 2004 34:690- 00.
30. Cast o M Conn D S a ton . he matic mani estations in m elod splastic s nd omes. he matol. 1991 1 : 21-
31. illst o m ohansson ohansson Mietelman . Imm ne-mediated complications in patients with m elod splastic s nd omes-clinical and c to enetic
eat es. ematol. 1995 55:42- .
32. conomopo los conomido iannopo los e o lo C apa eo io De veno las et al. Imm ne abno malities in m elod splastic s nd omes. Clin
athol. 19 5 3 :90 -11.