WPC "98t{)<{V0X=A%Pf Y%Ytv#OA+R  /{$؟+ `VQ`}y.5Rޫ[K}hUd[Yc^|nf#.U. %#8 C f a B*1 B[ x n 045 Ni k ^ l 0PxEUB  0PU Bw94=Q mSU:jU@UN 0w2 0 0I  1u t 0g: 0UU BU >8 72v 0 0c222<6X9`(CourierX'  -9. Other Blood Group SystmesLecture  0 .   hp LaserJet 1300 PCL 5e,,,,0 dTable_A&0 d d d#|x<6X9`(CourierXx6X@`7X@( U$    )   _9A ) xdEW!A X XXXX\rXX0   0$$   \rIX.OtherBloodGroupSystems@$$$MLAB2431#\r #%\rԀ%X>X%#XXX%X>#&XrXXX $"0@..  #&rXX&Xr##\rX&r# \rԀ  84  #\r Ǜ#ԩG2 ((3$  U!   ,hAZ*Helvetica Regular  *   _99A ) xdSE9Wf!A X XXXX\rXX \rMLAB2431#\r #%\rԀ%X>X%#XXX%X>2#&XrXXX#XXX&Xr#%X>XXX#%X%X>R#Ԁ%X>X%#XXX%X>#&XrXXX $"0   #&rXX&Xr##\rX&r[#&rX\r&XrXX&r#&rXX&Xr ##\rX&r# \r#\r o#%\r%X>X%#XXX%X>#&XrXXX#&rXX&Xr##\rX&r# \rԀ  84  0A $$0 A $A $0 $ $0I$$0I$I$0$$0Q$$0Q$Q$IX.OtherBloodGroupSystems#\r w#((3$  U!   ,hAZ*Helvetica Regulara1aUvAZ"Arial Regular(9 Z(Times New Roman (9 Z 6Times New Roman Regular(O;$0  2#  a  .3  0` (#(#(xir$0  0` (#(#0 ` (#` (#2#(  0  )3  0 (# (#<::D_:QuickFormat1 J& J   J& J  H3#37=CIQYag1.a.i.(1)(a)(i)1)a)a, b, c,Level 1Level 2Level 3Level 4Level 56#04Quick a. "  .0 ">$"Small Circle"0..,h Z*Helvetica Regular<\  `(&Roman 10cpi12pt!i)(b$0  0` (#(#2#   .3  0 ` (#` (#(;3$2#  0  .3  0      U!   _XXHHT   U-/XrXXXIX.OtherBloodGroupSystems X  *X,X` XX*&Q %XXrA.0  Introduction0$$ <X,X XX,X` X<0  1.0$$Inadditiontoantigenspreviouslydiscussed,over500otherscanbedetectedonhuman_rbcs_.$$ 0  2.0$$Antigensthatarecarriedbyaparticularcelllineofalmostallpersonsareknownas highincidence or 8  publicantigens. l$$ 0  3.0$$Antigenscarriedbyfewindividualsarecalled lowincidence or privateantigens.   $$ 0  4.0$$Eachoftheknownantigensdescribedinthislecturewereinitiallyidentifiedthroughdetectionofits  x  specificantibodyinaserum. P $$ 0  5.0$$Knowledgeoftheserologicbehaviorandcharacteristicsofthemajorbloodgroupantibodiesiscritical X  foridentification.0 $$ BX,X, XX,X XB0  0$$a.0$$Characteristicsofthe antibodies tobloodgroupantigensisveryimportantwhenevaluatingpanel  _workups_Ԁintheidentificationofirregularantibodies.d$$ 0  0$$b.0$$Considerationshouldbegiventophaseofreactivity,antibodyclassinvolved,anditsabilitytocause l _HDN_Ԁand/orhemolytictransfusionreactions.D$$ B.0  I/_i_$$ EX ,X XX,X, XE0  1.0$$TheIbloodgroupsystemisrelatedtotheABOandtheLewissystemsbyitsbiochemicalstructure.L$$ 0  2.0$$AntigenicdevelopmentT$$ 0    a.0$$Fetal_RBCs_Ԁarerichin _i_ antigen,andlack I antigen.$$ 0  0$$b.0$$Duringthefirst2yearsoflife,the I antigengraduallydevelops,andthereisaconcomitantlossof _i_. `$$ 0  0$$c.0$$Thusthe_RBCs_ԀofmostadultsreactstronglywithantiIwhilecordcells(babycells)arenonreactive l! withantiI.D"$$ 0  0$$d.0$$Inrareinstances,theIantigenneverdevelops.Theseindividuals,knownas_i_Ԁadults,retaintheI=_i_+  $ phenotypethroughouttheirlives.!t%$$ 0  3.0$$AntibodiestoI/_i_|#$!'$$ 0  0$$a.0$$The unexpectedantibodiesmostfrequentlyencountered whenserologictestareperformedatRT ,%") arethosedirectedagainsttheIand_i_Ԁantigens.&#*$$ 0  0$$b.0$$AntiIisacommoncauseofnonspecificagglutinationencounteredatRTwhenperformingantibody '`%, screens,_crossmatches_ԀandmayevencauseABOdiscrepanciesbyagglutinatingthereversecells.(8&-$$ 0  0$$c.0$$AntiIcanbedetectedintheserumofmostnormaladultsiftheserumistestedat4C.@*'/$$ 0  0$$d.0$$AntiIisalsoassociatedwith atypicalpneumonia causedbyMycoplasmapneumoniae.Cold +)1 agglutinintitersareusedtomonitortheirprogressandaidindiagnosis.,t*2$$  |.$,4 0  0$$e.0$$Anti_i_Ԁisclassicallyassociatedwithcertainviraldisorders,suchas infectiousmononucleosis ,caused X bytheEpsteinBarrvirus,and_cytomegalovirus_Ԁ(_CMV_).4$$ 0  4.0$$ClinicalSignificance$$ 0  0$$a.0$$AntiIisusuallyseenasabenign,naturallyoccurringcold\reactive_autoantibody_.<$$ "#0  0  0$$#0I"b  .0$$  ClinicallysignificantexamplesofantiIareveryrareandoccurinColdAgglutininSyndrome#0I"یD$$ Ќ  K"X ,X  hXX ,X XK0  0$$0$$1)0 $$Antibodiesareofhightiter(greaterthan1000).   $ $ 0  0$$0$$2)0 $$Havehighthermalamplitude. t  $ $ 0  0$$0$$3)0 $$Causehemolyticanemia. L  $ $ 0  0$$0$$4)0 $$Patient'sbloodmustbegiventhroughabloodwarmer.| $  $ $ 0  0$$c.0$$TheantibodyclassinvolvedisIgM,soantiIdoesnotcause_HDN_., $$ 0  0$$d.0$$AntiIdoesnotcause_HTRs_,butcancausesevereautoimmunehemolyticanemia. $$ 0  5.0$$SerologicalTestingtoConfirm4$$ 0  0$$a.0$$Testthepatient'sserumagainstthreegroupOadultcells(whichareIpositive),threegroupOcord < cells(whichare_i_Ԁpositive),andan_autocontrol_.$$ 0  0$$b.0$$AgglutinationoftheIpositiveadultcellsandthe_autocontrol_Ԁalongwithnegativeorveryweak l reactionswiththecordcellsprovesthepresenceofantiI.D$$ 0  0$$c.0$$Additionalconfirmationisobtainedbynegativereactionswhenthe_prewarmed_Ԁtechniqueisusedin L theantibodyscreen.AntiIwillbeundetectablewhenthetestisperformedstrictlyat37C.$$$ 0  0$$d.0$$InunusualcasestheantiImaybesostrongthatreactivitywillbeobtainedat_AHG_._Autoabsorption_ | orabsorptionusingrabbiterythrocytestroma(REST)mayhavetobedone.T$$ C.0  TheLewisSystem\ $$ 0  1.0$$Firstidentifiedin1946,when_Mourant_ԀdiscoveredanantibodythathenamedantiLea,thathenamedafter  " themaker,Mrs.Lewis.AntiLebwasdiscoveredin1948.0`$$#`$`$ 0  2.0$$LeaandLebarethemajorantigensoftheLewissystem;otherantigensinthesysteminclude_Lec_Led,and !<% Lex.l" &$$ 0  3.0$$Antigenicdevelopment$!($$ 0  0$$a.0$$Lewisantigens arenot intrinsicto_RBCs_,butarecarriedonplasma_glycosphingolipids_Ԁthatare %t#*  adsorbed fromtheplasmaandinsertedintotheRBCmembrane.&P$+$$ 0  0$$b.0$$Thegeneticcontrolofthetwoantigensappeartoresideinasinglegene,calledLe.Individualswho \(&- inheritatleastoneLegenewillhaveLewisantigens,individualswhoarele/le(_amorph_)willnothave 4)&. Lewisantigens. *'/$$ 0  0$$c.0$$TheLegenecausesproductionofa_transferase_ԀwhichcausesattachmentofL_fucose_Ԁtothe +d)1 subterminalchainofprecursorchaintoformtheLeaantigen.,<*2$$  D.+4 -/ -U -  0  0$$d.0$$ThiscanthenbemodifiedbytheLewisactiveenzymetoformLebantigen.X$$ 0  0$$0$$1)0 $$WhenLebisproduced,itisadsorbedpreferentiallyoverLeatoRBCmembranes. $ $ 0  0$$0$$2)0 $$BecauseLebantigenisproducedinmuchgreaterquantitiesthanLeaantigensonlyLebcanbe ` routinelydemonstrated.8 $ $ 0   $$ 0  0$$e.0$$TheLewisphenotypeof_RBCs_Ԁdependsonthephenotypeoftheplasmainwhichtheyaresuspended @ and canbechanged byincubatingthecellsinplasmacontainingdifferentLewisactive_glycolipids_. $$ 0    0$$1)0 $$IfLe(ab)_RBCs_ԀareincubatedwithplasmacontainingLeaorLeb_glycolipid_,theywilltakeup  r  theantigenfromtheplasma.TheywillsubsequentlytypeaspositivefortheLeantigenthey  J  wereincubatedin.z "  $ $ 0  0$$0$$2)0 $$IfLepositivecellsareincubatedinplasmafroman_lele_Ԁperson,thecellswilllosetheirLewis *  antigensandtypeasLe(ab).  $ $ 0  0$$f.0$$ Lewisphenotypesandtheirfrequencies Z$$ 0  0$$0$$0 $$  $ $ 0  0$$0$$0 $$0| $ $0 | $| $0X $ $Whites0X$X$Blacksf$$ 0  0$$0$$0 $$ | Le(a+b)0X $ $22%0X$X$0$$23%>$$ 0  0$$0$$0 $$ | Le(ab+)0X $ $72%0X$X$0$$55%$$ 0  0$$0$$  | Le(ab)0X$$6%0X$X$0$$22%$$ 0  0$$0$$0 $$ | Le(a+b+)0X $ $rare0X$X$0$$raren$$ 0  4.0$$Leantigensare absentorextremelyweakatbirth. v$$ 0    a.0$$CordbloodspecimensareessentiallyLe(ab).*$$ 0    b.0$$ExpressionoftheLebantigendevelopsgradually,andtheinfantwhoisgeneticallyLe(ab+)maytype  asLe(a+b+)duringthetransitionperiod.Z$$ 0  0$$c.0$$Thus,thenewbornwhoappearstobeLe(ab)atbirthcantypeasLe(a+b)attwomonthsofage, b   Le(a+b+)by12to18monthsandLe(ab+)by2or3yearsofage.:!$$ 0  0$$d.0$$Lewistypingcannotbedoneonbabiesforpaternitytesting.#$$ 0  5.0$$Lewisantigensduringpregnancy.!B%$$ 0  0$$a.0$$Lewisantigenstrengthmaydeclinedramaticallyduringpregnancy.J# '$$ 0  0$$b.0$$ThetransientlyLe(ab)pregnantwomanmayproduceLewisantibodiesduringpregnancy"$!($$ 0  0$$c.0$$TheseantibodiesdisappearafterdeliveryasthenormalLewisphenotypeisrestored.$")$$ 0  6.0$$InteractionsofLe,SeandHgenes.&R$+$$ 0  0$$a.0$$Interactionsof3geneticlociinfluencetheproductionoftheLeaandLebantigens.Z(&-$$ 0  0$$b.0$$Peoplewhotypeas_lele_ԀdonotproduceLeaorLebantigens,typeasLe(ab)anddonothaveLewis  *'/ antigensintheirplasmaorsecretions.ButiftheSegeneispresenttheywillhavetheappropriateA, *(0 BandHsubstancesintheirsecretions.+b)1$$  B.+4 0  0$$c.0$$_Nonsecretors_Ԁ(genotype_sese_)whohaveatleastoneLegenewillproduceonlytheLe(a)antigen,their X _RBCs_ԀwilltypeasLe(a+b)andtheirplasmaandsecretionswillcontaintheLe(a)antigen.0$$ 0  0$$d.0$$ExpressionoftheLe(ab+)phenotypedependsonthepresenceofatleastoneLegene,oneSegene,  andoneHgene.TheseindividualswillhavebothLeaandLebantigensintheirsecretionsaswellas ` theappropriateA,B,andHsubstancesintheirsecretions.8$$ @  LewisPhenotypesand_ABH_ԀSecretions @ 9H *ddd Xdd Xdd XH$H$,V d , td , t +  6'    p6RBCLewisPhenotype ; ,! @ " ;_ABH_Ԁ_Secretor_ԀStatus ? ,! @ "  ?Lewis_Secretor_ԀStatus S D, @ " p   PS 󀀀Le(a+b) ; ,! "  ; All_ABH_Ԁnon_secretors_ ? ,! "  ? All_secretors_ԀofLea  S D, " P   SЀ Le(ab+) ; ,!X "  ; All_ABH_Ԁ_secretors_ ? ,!X "  ? All_secretors_ԀofLeaandLeb Q @,X "    HQЀLe(ab) = ,!d "  = 80%_ABH_Ԁ_secretors_ d  20%_ABH_Ԁnon_secretors_ ? ,!< "  ? None <20d " 0 H    < H90  7.0$$ Lewisantibodies t$$ 0  0$$a.0$$Theyarealmostalways IgM andmayreactstronglyatRT,evencausingABOdiscrepanciesifthe ( reversecellsarepositivefortheappropriateLewisantigen.$$ 0  0$$0$$ $$ 0  0$$b.0$$LewisantibodiesoccuralmostexclusivelyintheseraofLe(ab)persons,andusuallywithoutknown \ RBCstimulus( naturallyoccurring ).4$$ 0  0$$c.0$$PersonswhoseRBCphenotypeisLe(ab+)donotmakeantiLeabecausesmallamountsof @ unconvertedLeaarepresentintheirsalivaandplasma. $$ 0  0$$d.0$$AntiLeaisaverycommonlyencounteredantibodybutitisunusualtofindantiLeb.p"$$ 0  0$$e.0$$ItisnotunusualtofindantiLeaandLebtogetherintheseraofLe(ab)individuals.x $$$ 0  0$$f.0$$Mostexamplesoftheseantibodies reactbestatroomtemperature ,butreactivitymaybeseenat (& Ѐ37CbutismuchweakerthanthatseenatRT.Theyalsomaybedetectedweaklyatthe_AHG_Ԁphase ' if_polyspecific_Ԁ_coombs_Ԁisusedasthe_AHG_Ԁreagent.($$ 0  0$$g.0$$Theantibody canbindcomplementandcauseinvitrohemolysis .Hemolysisismoreoftenseen !4* withenzymetreatedcells.h" +$$ 0  0$$h.0$$BecauseLewisantibodiesdonotcrosstheplacentaandtheantigensarepoorlydevelopedatbirth,the $!- antibodies havenotbeenimplicatedin_HDN_.$".$$ 0  0$$ _i_.0$$Lewisantibodiescanbeneutralizedinvitrobytheadditionofsoluble Lewissubstance thepatient's &L$0 serum.'(%1$$ 0  0$$0$$1)0 $$Thisprocedureisveryhelpfulwhenmultipleantibodiesaresuspectedandoneofthemisa 0)&3 Lewis.*'4 $ $ 0  0$$0$$2)0 $$TheLewisantibodyactivityiseliminatedleavingonly"other"antibodyactivity,ifpresent.+`)6 $ $  @.+9 0  8.0$$TransfusionPracticeX$$ 0  0$$a.0$$LewisantigensreadilyabsorbtoandelutefromRBCmembranes.Transfused_RBCs_Ԁassumethe  Lewisphenotypeoftherecipientwithinafewdaysofenteringthecirculation.$$ 0  0$$b.0$$Lewisantibodiesintherecipient'sserumarereadilyneutralizedbyLewisbloodgroupsubstancein 8 donorplasma.h$$ 0  0$$c.0$$Forthesereasons,itis exceedinglyrare forLewisantibodiestocauseinvivohemolysis. $$ 0  0$$d.0$$Itis notnecessarytotypedonorbloodforthepresenceorabsenceofLewisantigenspriorto  t  transfusionor_crossmatching_.  P $$ 0  0$$0$$1)0 $$Reactionsobtainedinthe_crossmatch_Ԁwiththedonorbloodprovideagoodindexoftransfusion \   safety.4  $ $ 0  0$$0$$2)0 $$Ifagglutinationand/orhemolysisareobservedat37Cor_AHG_,thenthebloodshouldnotbe    given.  $ $ D.0  ThePBloodGroupSystem<$$ 0  1.0$$ThePbloodgroupsystemwasdiscoveredin1927,when_Landsteiner_Ԁimmunizedrabbitswithhuman D _RBCs_ԀandusedtheresultingimmuneserumstotestforantigenicdifferencesbetweenindividualRBC  donors.$$ 0  0$$a.0$$_Landsteiner_ԀnamedthenewantigenP,butinthefollowingyears,asthecomplexityofthesystem L begantounfold,theterminologywaschanged.|$$$ 0  0$$b.0$$_Landsteiner_'sPantigenisnowcalledP1,withthenamePbeingreassignedtoanantigenpresenton , almostallhuman_RBCs_.$$ 0  0$$c.0$$_RBCs_ԀlackingP1,butshowntopossessP,areoftheP2phenotype.\$$ @  PhenotypesandFrequenciesOfP 1 andP 2 d   0  0$$0$$0 $$0| $ $ 0X| $| $Whites0X$X$Blacks0$$"$$ 0  0$$0$$0 $$ | P10 $ $0X $ $79%0X$X$  94%#$$ 0  0$$0$$ 0| $$P20 | $| $0X $ $21%0X$X$  6% l$$$ 0    d.0$$OtherPphenotypesexist,butareexceedinglyrare(<1%)andinclude:P,P1k,andP2k.t" &$$ 0  2.0$$ AntiP 1$$!($$ 0  0$$a.0$$TheseraofP2personscommonlycontainantiP1.%#*$$ 0  0$$b.0$$Theantibodyreacts optimallyat4C butmayoccasionallybedetectedat37C.'0%,$$ 0  0$$c.0$$Rarelymaycause invitro hemolysis.<)&.$$ 0  0$$d.0$$ AsitisnearlyalwaysIgM,itdoesnotcrosstheplacentaandhasnotbeereportedtocause_HDN_ *(0  (itispoorlyexpressedonfetalcells) . +t)1$$ 0  0$$e.0$$Ingeneral,antiP1haslittleclinicalsignificanceunlessitisreactiveat37C.-(+3$$  X.,4 0  0$$f.0$$AntiP1 hasrarely beenreportedtocausehemolysisinvivo.X$$ 0  3.0$$The strength oftheP1antigen varies amongdifferentRBCsamples,andantigenstrengthhasbeen   _reportedtodiminishwhen_RBCs_Ԁarestored.$$ 0  0$$a.0$$Thesecharacteristicssometimescreatesdifficulties,bothintesting_RBCs_Ԁfortheantigenandinthe @ identificationoftheantibody.p$$ 0  0$$b.0$$AntiP1bloodtypingreagentsareusuallysufficientlypotenttodetectweakformsoftheantigen.H$$ 0  4.0$$AnantibodythatisweaklyreactiveatRTtestingcanoftenbeshowntohaveantiP1specificityby    loweringtheincubationtemperatureorusingenzymetreated_RBCs_. x $$ 0  5.0$$ _Hydatid_ԀcystfluidorP 1 substancederivedformpigeoneggs inhibitstheactivityofantiP 1. ( $$ 0  0$$a.0$$InhibitionisausefulaidtotheidentificationofantiP1,especiallyiftheantibodyispresentinaserum 4  withmultipleantibodies.  $$ 0  0$$b.0$$TheantiP1isneutralized(becomesnonreactive)revealingother_specificities_Ԁ(ifpresent). $$ 0  6.0$$Donothavetoconfirmantigennegativefortransfusion.<$$ E.0  TheMNBloodGroupsD$$ 0  1.0$$TheMandNantigens(MNS1andMNS2in_ISBT_Ԁterminology)werediscoveredin1927by_Landsteiner_  andLevine.t$$ 0  2.0$$Studiesofinheritanceindicatesthegenesbehaveasallelicpairsthatarecloselylinked:MM,MNorNN.|$$$ 0  3.0$$FrequenciesoftheMNgenotypes.,$$ 0  0$$0$$0 $$0| $ $0 | $| $Whites0X $ $BlacksX$X$ 0  0$$0$$0 $$M+_N_0 $ $28%0X $ $26%\X$X$ 0  0$$0$$0 $$M+N+0 $ $50%0X $ $44%4X$X$ 0  0$$0$$0 $$MN+0 $ $22%0X $ $30%d  X$X$ 0  4.0$$AntiM"$$ 0  0$$a.0$$Detectedquitefrequentlyinhumansera,usuallyoccurringasanaturallyoccurringsalineagglutinin  l$ inantibodytestsperformedatRT.!D%$$ 0  0$$b.0$$MostexamplesoccurwithoutRBCstimulus.L# '$$ 0  0$$c.0$$WeakantibodyreactivitybyantiMcanbeenhanced byloweringthepHofthetestsystem to6.5.$")$$ 0  0$$d.0$$AntiMseraarepredominantlyIgM,butrareexampleshavebeenfoundthatarepartlyorwhollyIgG. &V$+ TheMantigenoccursinsufficientdensityonthe_RBCs_Ԁthatagglutinationinasalinetestmayoccur '.%, evenwhentheantibodyiswhollyIgG.^(&-$$ 0  0$$e.0$$Rarelyclinicallysignificant,althoughexamplesthatreactat37Cor_AHG_Ԁshouldbeconsidered *'/ potentiallysignificant.*(0$$ 0  0$$f.0$$Hasrarelybeenassociatedwith_HDN_Ԁor_HTR_.,>*2$$ "#0  0  0$$#0#w"g  .0$$  DonotneedtoconfirmunitsareMnegative,mustbe_crossmatch_Ԁcompatible.#0#w^w"یF.+4$$ Ќ  0  5.0$$AntiNX$$ 0    a.0$$Comparativelyrare.$$ 0    b.0$$InvariablyIgMandtypicallybehavelikeweaklyreactivecoldagglutinins.`$$ 0    c.0$$UsuallyconsideredclinicallyinsignificantalthoughsomepowerfulandpotentiallysignificantIgG h exampleshavebeenobserved.@$$ 0  6.0$$AntibodiesShowingDosage  $$ 0    a.0$$SomeindividualexamplesofantiMandantiNdemonstratedosage,showingsignificantlygreater  H  reactionstrengthswithhomozygouscellsthanwithheterozygouscells.x $$ 0    b.0$$ExamplesofantiMthatreact only with_RBCs_ԀcarryingadoubledoseoftheMantigenarefrequently (  encountered. $$ 0    c.0$$AntiNshowingdosagearerareencountered.\$$ 0    d.0$$Problemarisesduetothespecificitynotbeingimmediatelyapparentfromthereactionpatterns d  obtainedwithapanelof_RBCs_.<$$ 0  7.0$$TheMandNantigensaredenaturedwhenthecellsarewithenzymes,andareusefulinantibody  identification.l$$ 0  8.0$$Bloodgroupingreagentsareavailablefromavarietyofsources:human,rabbit,andmonoclonal.t$$ 0    a.0$$Themostwidelyused_lectin_ԀreagentistheantiNlikeextractof Vicia_graminea_ seeds.$$$ 0    b.0$$InterpretationofreactionswithantiMandantiNreagentsalwaysrequiresspecialcare,anditis  particularlyimportantthatthemanufacturer'sinstructionsbefollowedcarefully.X$$   9.0  AveryrarevariantoftheMNbloodgroupsystemisMg.` $$ 0    a.0$$Personwiththegenotype_MgN_ԀwillgivethereactionsMN+,leadingtothefalseconclusionthatthe " genotypeisNN.#$$   0  b.0$$Ofprimaryimportanceinpaternitytesting.!@%$$ F.0  S,sandUAntigensH# '$$   1.0  TheantigensS(MNS3)ands(MNS4)areproducedbyapairofallelicgenesfoundatalocusclosely $") linkedtotheMNlocus.%x#*$$   2.0  FrequenciesoftheS,sandUPhenotypes&P$+$$ 0  0$$0$$0 $$0| $ $0 | $| $ X Whites0 $ $BlacksX(&-$$ 0  0$$0$$ S+sU+0 $$0X $ $11%0X$X$  3%0)&.$$ 0  0$$0$$ S+s+U+0 $$0X $ $44%0X$X$  28%*'/$$ 0  0$$0$$ Ss+U+0 $$0X $ $45%0X$X$  69%*(0$$ 0  0$$0$$ SsU0 $$0X $ $00X$X$0$$Lessthan1%+`)1$$ 0  3.0$$AntibodiestoS,sandUusuallyoccurfollowingRBCstimulation.Allareconsideredclinically h-+3 significantandcapableofcausing_HDN_Ԁand_HTRs_.@.+4$$ Ї0    a.0$$Afewsalinereactiveexampleshavebeenreported,buttheantibodiesare usuallydetectedbythe X _IAT_. 4$$ 0    b.0$$AntiSoccursaboutasinfrequentlyasantiN.$$ 0    c.0$$Antisisseenevenlessoften,partlybecausethesphenotypeislessfrequentlythantheS,butalso < becausetheantigenisless_immunogenic_ԀthanS.l$$ 0    d.0$$AntiUisrarebutshouldbeconsideredwhenserumfromapreviouslytransfusedorpregnantBlack   personcontainsanantibodytoahighincidenceantigen.EstablishedasUnegativebyprovingthey    areSs. t $$ 0  4.0$$Santigenisdenaturedby_ficin_Ԁtreatment,thesantigenmayormaynotbedenatured,dependinguponthe | $  enzymeused.T $$   5.0  Mustconfirmthatunitsfortransfusionareantigennegativeaswellas_crossmatch_Ԁcompatible. $$ G.0  LutheranBloodGroupSystem\$$   1.0  Thefirstexampleofanti_Lua_wasfoundin1946.d $$ 0  2.0$$Antigensare_Lua_(LU1)and_Lub_(LU2)andhavethefollowingphenotypicfrequenciesinthewhite  population:$$ 0  0$$0$$0 $$Lu(a+b)0 $ $0X $ $0.15%DX$X$ 0  0$$0$$0 $$Lu(a+b+)0 $ $0X $ $7.5%tX$X$ 0  0$$0$$0 $$Lu(ab+)0 $ $0X $ $92.35%LX$X$ 0  0$$0$$0 $$Lu(ab)0 $ $0X $ $VeryRare$X$X$ 0  3.0$$Anti_Lua_|$$ 0  0$$a.0$$Theantibodyisuncommonandisusuallyanaturallyoccurringsalineagglutinin.,$$ 0  0$$b.0$$Lutheranantigensarepoorlydevelopedatbirth,anti_Lua_hasnotbeenreportedtocause_HDN_.4!$$ 0  0$$c.0$$Hasnotbeenassociatedwith_HTRs_Ԁandisnotconsideredclinicallysignificant.#$$ 0  0$$d.0$$MayagglutinatesalinesuspendedcellsinaMFmanner.Thisischaracteristicofreactionswithsome !<% examplesofAnti_Lua_.l" &$$ 0  4.0$$Anti_Lub_$!($$ 0  0$$a.0$$Antibodyproductioncausedbyexposuretoantigenthroughtransfusionorpregnancyandis %t#* consideredclinicallysignificant.&L$+$$ 0  0$$b.0$$Hasbeenreportedtocausediminishedsurvivaloftransfused_RBCs_.T(%-$$ 0  0$$c.0$$Hasbeenimplicatedin mild _HDN_.*'/$$ 0  0$$d.0$$Mayagglutinatesalinesuspendedcells,althoughitmostcommonlyreactsatthe_IAT_.+`)1$$ 0  0$$e.0$$Verydifficultfindingantigennegativebloodasapproximately99%ofthepopulationisantigen h-+3 positive.@.+4$$ ЇH.0  _Kell_ԀBloodGroupSystemX$$ 0  1.0$$TheK(KEL1)antigenwasfirstidentifiedin1946asthecausativeantibodyinacaseof_HDN_.$$ 0  2.0$$ThealleleofKis_Cellano_,k(KEL2).`$$ 0  3.0$$Phenotypesfrequenciesareasfollows:h$$ 0  0$$0$$0 $$0| $ $0 | $| $White0X $ $Blacks X$X$ 0  0$$0$$0 $$K+k0| $ $0 | $| $0.2%0X $ $Rare  X$X$ 0  0$$0$$0 $$K+k+0 $ $8.8%0X $ $2% p X$X$ 0  0$$0$$0 $$Kk+0| $ $0 | $| $91%0X $ $98% H X$X$ 0  4.0$$The _Kell_Ԁantigenisstrongly_immunogenic_ ,andisfrequentlyfoundinserafromtransfusedpatients.P $$ 0  5.0$$AntiKandantik $$ 0    a.0$$Mostexamplesdetectedareofimmuneorigin,clinicallysignificantandarereactivebythe_IAT_,some \ bindcomplement.4$$ 0    b.0$$AntiKhascaused_HTRs_Ԁonnumerousoccasions,bothimmediateanddelayed.<$$ 0    c.0$$Bothhavebeenimplicatedin_HDN_.$$ 0    d.0$$90%ofdonorsareKnegative,soitisnotdifficultfindingcompatibleblood.D$$ 0    e.0$$AntikhasclinicalandserologiccharacteristicssimilartoantiKbutoccursmuchlessfrequently L becauseonlyaboutonepersonin500lacksthekantigen.$$$ 0    f.0$$Donorunitsmustbetestedandbenegativefortheantigenaswellas_crossmatch_Ԁcompatible.|$$ 0  6.0$$Otherantigensofthe_Kell_Ԁsystem.,$$ 0    a.0$$_Kpa_(Penney,KEL3),19574!$$ 0  0$$b.0$$_Kpb_(_Rautenberg_,KEL4),1958 "$$ 0  0$$c.0$$_Jsa_(_Sutter_,KEL6),1958#$$ 0  0$$d.0$$_Jsb_(Matthews,KEL7),1963 d$$$ 0  0$$e.0$$Koisanullphenotype.!<%$$ 0  0$$f.0$$_McLeod_Ԁphenotypehasweakenedexpressionof_Kell_Ԁsystemantigensandisassociatedwithstructural l" & andfunctionalabnormalitiesof_RBCs_Ԁandleukocytes.D# '$$   7.0  Antibodiestoother_Kell_Ԁsystemantigens$")$$ 0  0$$a.0$$Showsimilarserologiccharacteristicsandareconsideredclinicallysignificant.&L$+$$ 0  0$$b.0$$Mayoccurfollowingtransfusionorpregnancy.T(%-$$ 0  0$$c.0$$Frequencyinfluencedby_immunogenicity_Ԁoftheantigenanddistributionofrelevantnegativeand *'/ positivephenotypesamongdonors.*(0$$ 0  0$$d.0$$Theseantibodiesarerare,suggestingthattheantigensareoflow_immunogenicity_.,4*2$$  <.+4 0  0$$e.0$$Patientsimmunizedtohighincidenceantigenspresentaproblemthatmayrequireassistancefroma X raredonorfile.0$$ 0  0$$f.0$$Theseantibodiesmaycause_HDN_Ԁand_HTRs_.$$ I.0  DuffyBloodGroupSystem8$$ 0  1.0$$The_Fya_(FY1)antigenwasdiscoveredintheserumofamultiplytransfusedhemophiliacin1950.The @ followingyear_Fyb_(FY2)wasdiscoveredintheserumofamultiparousfemale. $$ 0  2.0$$In1955itwasreportedthatmostAfricanAmericanslackedboththe_Fya_and_Fyb_antigensproducedby  p  whatwasthoughttobe_codominant_Ԁalleles. H $$ 0  0$$a.0$$Indicatedthatathirdallele,asilentgenecalled_Fy_Ԁ(FY0)wasalsopartofthesystem.P $$ 0  0$$b.0$$Blackswhoare_Fy_(ab)areconsideredtobehomozygous_FyFy_. $$ 0  0$$c.0$$The_Fy_(ab)phenotypeprovidesresistancetoinfectionwithPlasmodiumvivax,aspeciesofmalaria.X$$ 0  3.0$$PhenotypesandfrequenciesoftheDuffySystem.`$$ 0  0$$0$$0 $$0| $ $0 | $| $0X $ $Whites0X$X$  Blacks$$ 0  0$$0$$0 $$_Fy_(a+b)0 $ $0X $ $17%0X$X$  9%$$ 0  0$$0$$0 $$_Fy_(a+b+)0 $ $0X $ $49%    1%hX$X$ 0  0$$0$$0 $$_Fy_(ab+)0 $ $0X $ $34%0X$X$  22%@$$ 0  0$$0$$0 $$_Fy_(ab)0 $ $ X veryrare0 $ $68%p$$ 0  4.0$$AntibodiestoDuffyantigens. $$ 0    a.0$$Bothanti_Fya_andanti_Fyb_causeimmediateanddelayed_HTRs_Ԁand_HDN_x$$ 0  0$$b.0$$Anti_Fya_isquitecommonlyencountered,anti_Fyb_isconsiderablylesscommon.($$ 0  0$$c.0$$Theantibodiesreactbestbythe_IAT_.0!$$ 0  0$$d.0$$ Theantigensitesaredestroyedbymostenzymesusedinserologictests ,soanti_Fya_andanti_Fyb_ # antibodiesusuallygivenegativereactionsinenzymetestprocedures. d$$$ 0  0$$e.0$$Duffyantibodiesfrequentlyshowdosageaffect,sometimestosuchanextentthattheymaybe l" & _nonreactive_Ԁwithheterozygouscells.D# '$$ 0  0$$f.0$$WhenDuffyantibodiesareencountereditisextremelyhelpfultosearchintherightethnicpopulation $") forappropriateantigennegativedonorsto_crossmatch_.%t#*$$ J.0  The_Kidd_ԀBloodGroupSystem|'$%,$$ 0  1.0$$Anti_Jk_(wasfirstrecognizedin1951intheserumofawomanwhohadgivenbirthtoachildwith_HDN_. ,)&. Twoyearslater,anti_Jkb_wasfoundintheserumofapatientwhohadsufferedatransfusionreaction.*'/$$ 0  0$$a.0$$Thedesignationforthe_Kidd_Ԁsystemis_Jka_(JK1)and_Jkb_(JK2)+\)1$$ 0  0$$b.0$$The"null"phenotypewasdiscoveredin1959,theindividualstypeas_Jk_(ab).Thisisduetoathe d- +3 silentallele_Jk_.MorecommoninpeopleofPolynesiandescent.<.+4$$ Ї  2.0  Phenotypesandfrequenciesofthe_Kidd_ԀSystem.X$$ 0  0$$0$$0 $$0| $ $0 | $| $0X $ $Whites0X$X$  Blacks$$ 0  0$$0$$0 $$_Jk_(a+b=)0 $ $0X $ $28%0X$X$  57%$$ 0  0$$0$$0 $$_Jk_(a+b+)0 $ $0X $ $49%0X$X$  34%`$$ 0  0$$0$$0 $$_Jk_(a=b+)0 $ $0X $ $23%0X$X$  9%8$$ 0  0$$0$$0 $$_Jk_(a=b=)0 $ $0X $ $ExceedinglyRarehX$X$ 0  3.0$$Antibodiesto_Kidd_ $$ 0    a.0$$Occasionallycauses_HDN_,butitisusuallymild. p $$ 0    b.0$$ Theseantibodiesarenotoriousforinvolvementinsevere_HTRs_, especiallyindelayedreactions, x  whichoccurwhenantibody,developingrapidlyinananamnesticresponsetoantigensontransfused T  _RBCs_,destroythestillcirculating_RBCs_., $$ 0  0$$0$$1)0 $$Insomereportedcases,retestingthepatient's_pretransfusion_Ԁserumhasconfirmedthatthe   antibodywasundetectable.\ $ $ 0  0$$0$$2)0 $$Thishighlightstheimportanceofcheckingpreviousrecordspriortoselectingbloodfor d  transfusion.Inseveralcasestheantibodyhadbeenpreviouslydetectedandidentified.< $ $ 0  0$$c.0$$Antibodiesreactbestbythe_IAT_,butsalinereactivityhasbeenobserved.$$ 0  0$$d.0$$Theseantibodiesareoftenweaklyreactiveandlosereactivityuponstorage,evenafterfreezingand D someworkersfeelthat_RBCs_Ԁcarryingadoubledoseof_Jka_or_Jkb_areneededinscreeningteststo t reliabledetecttheseantibodies.L$$ 0  0$$e.0$$ Rareexamplesof_Kidd_Ԁantibodieswillnotbedetectedunlessfreshcomplementispresentand  the_AHG_Ԁserumhas_anticomplementary_Ԁactivity.$$ 0  0$$0$$ 1)0 $$Sincethesearesorare,andduetothenumberoffalsepositivesobtainedwhenusingpoly 0 _coombs_,itisbesttouseIgG.`  $ $ 0  0$$0$$2)0 $$Someworkersreportnodifficultiesdetectingtheseantibodiesinlowionicteststhatincorporate " antiIgG.# $ $ 0  0$$f.0$$Donorunitsmustbetestedwithspecificantiseraandbenegativefortheantigenaswellas !@% _crossmatch_Ԁcompatible.p" &$$ K.0  AdditionalPairsofAntitheticalRBCAntigens $!($$ 0  1.0$$Thebloodgroupsystemspreviouslyreferredtoaretheprincipleonesinwhichtheantibodiesmaybe %x#* frequentlyencountered.&P$+$$ 0  0$$a.0$$Othersystemsofgeneticallydeterminedantigensalsoexistandthestudentshouldbeawareofthem.X(&-$$ 0  0$$b.0$$Theseantigensappeartobeless_immunogenic_Ԁthanthoseoftheothermajorbloodgroupsystems.*'/$$ 0  0$$c.0$$Antibodiesdirectedattheseantigensoccurrarely,usuallyinseracontainingmultiple_specificities_Ԁand +`)1 areusuallyclinicallysignificant.,8*2$$  @.+4 0  0$$d.0$$Theantigensthemselvesmaybeimportantingeneticinvestigationsandpopulationorfamilystudies.X$$ 0  2.0$$Diego(Diaand_Dib_)$$ 0  0$$a.0$$Usefulasaracialmarker,DiaantigenbeingalmostentirelyconfinedtopopulationsofMongolian ` origin,includingNativeAmericans.8$$ 0  0$$b.0$$OnehundredpercentofthewhitepopulationtypesasDi(ab+).h$$ 0  3.0$$_Colton_Ԁ(_Coa_andCob). $$ 0  0$$a.0$$ParallelstheincidenceofKandk. p $$ 0  0$$b.0$$Approximately89%oftheWhitepopulationisCo(a+b=)and10.4%areCo(a+b+). H $$ 0  4.0$$Cartwright(_Yta_and_Ytb_)P $$ 0  0$$a.0$$ParallelstheincidenceofKandk. $$ 0  0$$b.0$$Approximately91.9%oftheWhitepopulationis_Yt_(a+b)and7.9%is_Yt_(a+b+). $$ 0  5.0$$_Dombrock_Ԁ(_Doa_and_Dob_)0$$ 0  0$$a.0$$ParallelsincidenceoftheDuffysystem.8$$ 0    b.0$$Approximate49.5%oftheWhitepopulationisDo(a+b+),33.3%isDo(ab+)and17.2%isDo(a+b).$$ L.0  TheSexLinkedBloodGroupantigen_Xga_h$$   1.0  In1962,anantibodywasdiscoveredthatidentifiedanantigenmorecommonamongwomenthanamong p men.H$$ 0    a.0$$ThiswouldbeexpectedofanXbornecharacteristic.$$ 0    b.0$$FemalesinheritanXchromosomefromeachparent,whereasmaleinheritXonlyfromtheirmother.x$$   2.0  Theantigeniscalled_Xga_inrecognitionofitsXbornemannerofinheritance.($$ 0  3.0$$Anti_Xga_isanuncommonantibodythatusuallyreactonlybythe_IAT_,butsalineagglutininshavebeen 0! observed."$$ 0  4.0$$Thisantibodyhasnotbeenimplicatedin_HDN_Ԁor_HTRs_. `$$$ 0  5.0$$Anti_Xga_maybeusefulfortracingthetransmissionofgenetictraitsassociatedwiththeXchromosome.h" &$$ M.0  HighIncidenceRBCAntigens$!($$ 0  1.0$$ Highincidence(public)antigenisdefinedasthoseantigensoccurringin92to99.9%ormoreofthe %p#* generalpopulation .&J$+$$ 0  2.0$$Personsmake_alloantibody_Ԁtospecificbloodgroupantigensduetothefactthattheirown_RBCs_Ԁlackthe T(%- offendingantigen.,)&.$$ 0  3.0$$Forthisreason,antibodiesdirectedathighincidenceantigensarerarelyencounteredbecauseby *(0 definition,mostofthegeneralpopulationwillhavetheseantigenspresentontheircells.+\)1$$ 0  4.0$$Whenantibodiestohighfrequencyantigensoccuritmaybeexceedinglydifficulttofindcompatibleblood.d- +3$$  <.+4 0    a.0$$Membersofthepatient'sfamily,especiallysiblings,areusuallythemostpromisingsources.X$$ 0    b.0$$MayhavetohavebloodcentercontacttheRareDonorFiletofindpotentialunits.$$ 0  5.0$$Antibodiestothecorrespondingantigensusuallyreactbestbythe_IAT_.`$$ N.0  OtherRBCAntigensofComparativelyHighIncidenceh$$   1.0  Formerlyreferredtoashightiter,lowavidity(_HTLA_). $$   2.0  Amongthemostfrustratingproblemsencounteredintestingseraforunexpectedantibodiesarethose  p  createdbythegroupofantibodiesformedtotheseantigens. H $$ 0  0$$a.0$$Reactionsinvariablyobservedinthe_AHG_Ԁphaseoftesting.P $$ 0  0$$b.0$$Theyarecommonlyweaklyreactive,variableinreactivityandsometimesirreproducible. $$ 0  3.0$$ Theterm_HTLA_Ԁreferstothefactthatfeeblereactionsseenwithundilutedserumwilloftenpersist X inserumsubjectedtoconsiderabledilution. 4$$ 0  4.0$$Reactionssuggestseitherlowaffinityoftheantibodyorweakcellularexpressionoftheantigen,rather < thanlimitedantibodyconcentration.$$ 0  5.0$$Transfusionpractice:l$$ 0    a.0$$Theseantibodies,thoughofdebatableclinicalsignificance,reactwithantigenshavingafairlyhigh D incidenceinthepopulation.t$$ 0    b.0$$Whentheantibodyhasbeenidentified,bloodcannormallybeissuedwithoutdelay.$$$ 0    c.0$$Workcanthencontinuetodeterminetheantibodyspecificity.|$$ O.0  The_Sda_Antigen,$$ 0  1.0$$Thisantigenisoffairlyhighincidenceandiswidelydistributedinmammaliantissuesandbodyfluids. 4!  $$ 0    a.0$$Theantigenisvariablyexpressedonthecells.#$$ 0    b.0$$Maydisappeartransientlyduringpregnancy.!<%$$ 0  2.0$$Anti_Sda_ismostfrequentlydetectedduringthe_AHG_Ԁphasewhenthetestisexaminedmicroscopically.D# '$$ 0  0$$a.0$$Mostexampleswouldbeequallydemonstrableifthecellbuttonwereexaminedmicroscopicallyat $") ISafterresuspension.%t#*$$ 0  0$$b.0$$ Mixedfieldagglutinationisthecharacteristicreaction. |'$%,$$ 0  3.0$$UrinefromguineapigsandfromSd(a+)humansinhibitsthereactivityofanti_Sda_(neutralization).0)&.$$ 0  4.0$$Althoughreportedonceascausingan_HTR_,anti_Sda_iswidelybelievedtohave noclinicalsignificance.*(0$$  D.+4  P.0  LowincidenceRBCAntigensX$$ 0  1.0$$Manylowincidence(private)RBCantigenhavebeenrecognizedinadditiontoagrowingnumberthat  _havebeenassignedtotheMNandRhsystems.$$ 0  2.0$$Theseantigensoccurwithafrequencyof1in500orless.8$$ 0  3.0$$Antibodies@$$ 0    a.0$$Reactwithsofewrandombloodsamplesthattheyvirtuallynevercausedifficultiesinselectingblood    fortransfusion. p $$ 0  0$$b.0$$Theyareonlyencounteredbychanceinantibodyscreeningorcompatibilitytesting,whenascreening x  cellordonorcellselectedhappenstocarrythecorrespondingantigen.P $$ 0  0$$c.0$$Routineantibodyscreeningtestsrarelydetectsuchantibodies,thecrossmatchaffordstheonly   opportunitytodetectanincompatibility,shouldantigenpositivebloodbeselected. $$ 0  0$$d.0$$Thechanceofchoosingdonorbloodpositiveforthatantigen,however,isquiteremote.0$$ 0  4.0$$Theantibodiestotheseareusuallysalineagglutinins,butsomedooccurasIgGreactiveattheIATand 8 havecauseHDN.$$ Q.0  TheBgAntigensh$$ 0  1.0$$Antibodiesdirectedatcertainleukocyteantigenssometimescauseconfusingreactionsinserologictest p withRBCs.H$$ 0  0$$a.0$$TheBgantigensareexpressedtovariabledegreesonRBCs.$$ 0  0$$b.0$$ReactionsofvaryingstrengthsareobservedwhenaserumcontainsantiBgistestedwithdifferent P Bg+RBCs.($$ 0  0$$c.0$$ReactivityismostcommonlyobservedatIATalthoughpotentantiBgmaycausedirectagglutination 0! ofcellswithstrongexpressionofBgantigens."$$ 0  2.0$$AntibodiestotheBgantigenarenotclinicallysignificantinregardtoRBCtransfusionpractice.%