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Rh sisxlis jgufi

Rh sisxlis jgufi. Brian Poirier, MD UCDavis Medical Center 2010. Temebi. terminologiis sistemebi Rh antisxeulebi Rh SeuTavseblobis Sedegebi ucnauri fenotipebi. miznebi. Rh terminis gansazRvreba Rh diferencireba LW- sgan

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Rh sisxlis jgufi

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  1. Rh sisxlis jgufi Brian Poirier, MD UCDavis Medical Center 2010

  2. Temebi • terminologiis sistemebi • Rh antisxeulebi • Rh SeuTavseblobis Sedegebi • ucnauri fenotipebi

  3. miznebi • Rh terminis gansazRvreba • Rh diferencireba LW-sgan • SevadaroT mTavari genotipebi, maT Soris, Fisher-Race, Wiener, da Rosenfield terminologiebi • ganvsazRvroT Rh-isZiriTadi bioqimiuri struqtura

  4. miznebi (gagrZeleba) • aRvweroT da ganvasxvavoT susti D-sgamoxatvis 3 meqanizmi • aRvweroT Rh-antisxeulebis 3 maxasiaTebeli • Rh D –Ti imunizaciis prevencia

  5. Rhsisxlisjgufi • mniSvnelobiT meore sisxlis jgufi (ABO-sSemdeg)

  6. Rh sistemis istoria • 1939 Levine-maaRwera HTR (hemolizuri transfuziuli reaqcia) OB pacientSi: • mSobiarobisTanave, qals dasWirda transfuzia. • meuRlis sisxlis gadasxmis Semdeg (ABO Tavsebadi), mas ganuviTarda mwvave HTR. • Sratidan gamoyves antisxeuli, romelic moqmedebda mamis RBC-ze 37 C-zec da 20 C-zec.

  7. Rh sistemis istoria (gagrZeleba)

  8. Rh sistemis istoria (gagrZeleba) • 1940 wels Landsteiner da Wiener gamoaqveynes informacia: • antisxeulis Sesaxeb, romelic daamzades zRvis goWisa da kurdRlisagan, romelTac gadausxes Rhesus maimunis RBC. • antisxeuls, romelic iwvevda adamianis RBC-s 85%-is aglutinacias, uwodes “Rh.” • antisxeuls gadaarqves da uwodes anti-LW (Landsteiner and Wiener). • saxeli Rh daarqves adamianis gamomuSavebul antisxeuls.

  9. Rh sistemebis nomenklatura • Fisher-Race: DCE terminologia • Wiener (Rh-Hr): Rh-Hr terminologia • Rosenfield: Aalfa/numeruli terminologia • ISBT (International Society of Blood Transfusion): numeraciuli terminologia

  10. Fisher-Race (DCE an CDE) • 5 mTavari antigeni: D, C, E, c, e • Rh dadebiTi realurad niSnavs D dadebiTs. • D-sararseboba aRiniSneba “d”-Ti (mogvianebiT aRmoCenil iqna ararealuri – “amorfuli” antigeni). • 8 potenciur haplotips ewoda saxeli zemoTnaxsenebi antigenebisaTvis arsebuli genebis mixedviT (eg, Dce, dce).

  11. Wiener (Rh-Hr) • sxvadasxva dasaxeleba 5 mTavari antigenisaTvis • Rho=D • rh’=C • rh”=E • hr’=c • hr”=e

  12. Wiener (Rh-Hr) (gagrZeleba) • zemoTnaxsenebi 8 potenciuri kombinaciisaTvis darqmeuli saxelebi dResac xmarebaSia R1=DCe r’=dCe R2=DcE r”=dcE Ro=Dce r=dce Rz=DCE ry=dCE

  13. Wiener (Rh-Hr) –is gadayvana Fisher-Race-Si Fisher-Race terminologia gamosayeneblad iolia: • R=D, r=d • 1 an prime=C • 2 an double prime=E • 0 an blank=ce • any superscript letter =CE

  14. Rosenfield-isterminologia (alfa/numeruli) • Rosenfield sistemas ar aqvs genetikuri safuZveli, gviCvenebs mxolod eriTrocitze antigenis arsebobas an ararsebobas. • nomris win “minus” niSani gviCvenebs antigenis ararsebobas. nomris ararseboba gviCvenebs,rom antigeni ar aris tipirebuli.

  15. Rosenfield-isterminologia (gagrZeleba) • D aRiniSneba rogorc Rh1; C – rogorc Rh2; E - rogorc Rh3; c – rogorc Rh4; e – rogorc Rh5 • magaliTi 1: D+ C- E+ c+ e+ iqneba: Rh: 1, -2, 3, 4, 5 • magaliTi 2: DCe/dcE iqneba: Rh: 1, 2, 3, 4, 5

  16. ISBT terminologia • ISBT –miiRo 6-cifriani nomrebi sisxlis yvela jgufis specifikaciisaTvis. • pirveli 3 nomeri warmoadgens sistemis, xolo, bolo 3 – antigenis specifikacias. • 004 mieniWa Rh sisxlis jgufis sistemas; Rh sistemis yvela antigens daemata unikaluri nomeri 6-cifriani kompiuteruli nomris Sesavsebad. • magaliTi: “D” antigeni iqneba “004001”

  17. “Rh fenotipebis “didi oTxeuli” • R1, R2, R0, da r aris yvelaze xSirad nanaxi fenotipebi. • R0 -yvelaze xSiria zangebSi, iSviaTi TeTrkanianebSi. • R1> R2 • r -sixSiriT yovelTvis meorea • TeTrkanianebi: R1 > r > R2 > R0 • zangebi: R0 > r > R1 > R2

  18. genebis sixSire Rh antigenebisTvis genisixSire % D 85 d 15 C 70 E 30 c 80 e 98

  19. xSiri Rh tipebi 3 nomenklaturiT Wiener Fisher-Race Rosenfield % R1r DCe/dce Rh: 1,2,-3, 4,5 33 R1R1 DCe/DCe Rh: 1,2,-3, -4,5 18 rr dce/dce Rh: -1,-2,-3, 4,5 15 R1R2 DCe/DcE Rh: 1,2,3, 4,5 11 R2r DcE/dce Rh: 1,-2,3, 4,5 9 R2R2 DcE/DcE Rh: 1,-2,3, 4,-5 2

  20. Rh antigenebi • ara-glikolizirebuli proteinebi eriTrocitebis membranaSi. • gadaecema, rogorc kodominanturi alelebi. • arian transmembranuli polipeptidebi da eriTrocituli membranis ganuyofeli nawili. • yvela Rh antigeni (D,C,E) aris Zalian msgavsi, gansxvavdebian mxolod 44 wyvili nukleotidiT. • C da c gansxvavdeba 4 -T • E da e gansxvavdeba 1 -T

  21. Rh antigenebi Hillyer et al 2009

  22. Rh antigenebi(gagrZeleba) • Rh antigenebi aris Zlier imunogenuri: D > c > E > C > e • D antigeni aris yvelaze imunogenuri antigeni ABO sistemis gareT. • 0.5 ml-c ki iwvevs anti-D allo-imunizacias janmrTel moxaliseebSi (Gunson et al 1970).

  23. Rh antisxeulebi • ar boWavs komplements • eqstravaskularulia • IgG • SeuZlia gaiaros placentaruli barieri da gamoiwvios HDFN • HTR – hemolizuri transfuziuli reaqcia • saWiroebs zemoqmedebas (orsuloba an transfuzia)

  24. Rh SeuTavseblobis Sedegebi • gamouvleneli: janmrTeli, D-negatiuriindividebis 80% gamoimuSavebsanti-D-s erTi erTeulis gadasxmisas. daaxloebiT 22% hospitalizirebuli (ara-onkologiuri) pacientebisa (Yazer et al 2007). • gamovlenili: HTR eqstravaskularuli hemoliziT • yvelaze mZime HDFN

  25. D imunizaciis prevencia:RhIgG • makro doza (300 g): icavs 30 mL leikocitebis an 15 mL eriTrocitebisagan • mikro doza (50 g): icavs 5 mL leikocitebisgan; sakmarisia orsulobis Sewyvetis, amniocentezis, saSvilosnosgare rupturis dros (orsuloba 12 Tvemde)

  26. ucnauri fenotipebi • susti D fenotipi Du • Rhnullfenotipi • gaerTianebuli Rh antigenebi

  27. susti D fenotipi (Du) • zogi D –dadebiTi individi saWiroebs AHG fazas D antigenis gamosavlenad • mizezebi: • C –s sapirispiro qromosoma D-smimarT (C trans-poziciaSi): mag: Dce/dCe • genetikurad susti D (D-sSesustebuligamoxatva) • nawilobrivi D (“mozaikuri”) • (yvelaze metad midrekili anti-D-s gamomuSavebisken)

  28. D-sstatusis gansazRvra • donorebi: D neg donorebma unda Caitaron AHG testi • recipientebi: D neg recipientebisaTvis AHG testi ar aris aucilebeli

  29. reagentebi Rh antigenis tipirebisaTvis • Saline anti-D (IgM, ar gamoiyeneba Du) • maRalproteinuli anti-D (saWiroebs Rh kontrols) • qimiurad modificirebuli anti-D (dabalproteinuli) • monoklonuri anti-D • monoklonebis narevi (anti-IgM da anti-IgG anti-D)

  30. Rhnullfenotipi • aklia yvela Rh antigeni • Rhnullsindromi warmoadgens saSualo kompensirebul hemolizur anemias, stomatocitoziT, sferocitoziT da retikulocitoziT. • gadaesxmeba Rhnull sisxlTan erTad • Rhmod ffenotipi -s klinikuri simptomebi naklebad mZimea da iSviaTad aRiniSneba.

  31. gaerTianebuli Rh antigenebi • f = antigeni gvaqvs, rodesac c da e arian erT qromosomaSi • G: G gvaqvs umetes D pos da yvela C pos eriTrocitSi. Anti-G Tavdapirvelad gaCnda, rogorc anti-D+C; Semdgomma gamokvlevebma aCvena, rom es anti-G iyo mimarTuli D+G - sken.

  32. LW sistema • LW antigenebi • Anti- LW

  33. LW antigenebi (normaluri gza) winamorbedi substancia ÜDCE genebi normaluri Rh antigenebi LWa LWbgenebißà LW genebi LW pos LW neg

  34. Anti- LW: • Zlier urTierTqmedebs umravles D pos eriTrocitebTan. • sustad urTierTqmedebs D neg eriTrociTebTan. • ar urTierTqmedebs Rhnull eriTrociTebTan. • Tanabrad kargad urTierTqmedebs zurgis tvinis ujredebTan, miuxedavad D tipisa. • iSviaTad aqvs klinikuri mniSvneloba.

  35. miznebi • Rh terminis gansazRvreba • Rh diferencireba LW-sgan • SevadaroT mTavari genotipebi, maT Soris, Fisher-Race, Wiener, da Rosenfield terminologiebi • ganvsazRvroT Rh-is ZiriTadi bioqimiuri struqtura

  36. miznebi (gagrZeleba) • aRvweroT da ganvasxvavoT susti D-sgamoxatvis 3 meqanizmi • aRvweroT Rh-antisxeulebis 3 maxasiaTebeli • Rh D –Ti imunizaciis prevencia

  37. madloba . . . • Rosemary Howard, CLS • Dr Chris Gresens

  38. literatura • Transfusion Medicine and Hemostasis, Hillyer et al, 2009, Elsevier Pub. • Yazer et al, (2007), Detection of anti-D in D- recipients transfused with D + red cells, Transfusion 47 2197-2201 • Avent ND, Reid (2000) The Rh blood group system: a review Blood 95 375-387. • Gunson et al (1970) The Anti-Rh0(D) Responses of Immunized Volunteers following Repeated Antigenic Stimuli, BJH

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