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IMMUNOIMMUNO--HEMATOLOGY.HEMATOLOGY.IMMUNOIMMUNO HEMATOLOGY.HEMATOLOGY.

P f Adi K A S PK(KH)P f Adi K A S PK(KH)Prof. Adi Koesoema Aman SpPK(KH)Prof. Adi Koesoema Aman SpPK(KH)Dr. Zulfikar Lubis SpPK(K)Dr. Zulfikar Lubis SpPK(K)

DIVISI HEMATOLOGI DEPARTEMENT PATOLOGY KLINIK DIVISI HEMATOLOGI DEPARTEMENT PATOLOGY KLINIK FK USU/RSUP.H.ADAM MALIK MEDAN .FK USU/RSUP.H.ADAM MALIK MEDAN .

IMMUNO IMMUNO HEMATOLOGI .HEMATOLOGI .

Mengandung arti reaksi Mengandung arti reaksi i l ik b k iti l ik b k itimmunologik yg berkaitanimmunologik yg berkaitandengan sel darah merah .dengan sel darah merah .Tidak saja mencakup Tidak saja mencakup Immunologi , hematologiImmunologi , hematologig gg gtapi juga genetika &tapi juga genetika &biokimia biokimia biokimia biokimia

The ABO SystemThe ABO SystemThe ABO SystemThe ABO System

Discovered in 1901 by Dr. KarlDiscovered in 1901 by Dr. KarlDiscovered in 1901 by Dr. Karl Discovered in 1901 by Dr. Karl LandsteinerLandsteiner4 Main Phenotypes (A, B, AB, O)4 Main Phenotypes (A, B, AB, O)yp ( , , , )yp ( , , , )ABOABO gene located on long arm of gene located on long arm of chromosome 9chromosome 9

The ABO AntigensThe ABO AntigensThe ABO AntigensThe ABO Antigens

Added to Proteins or Lipids in Red CellsAdded to Proteins or Lipids in Red CellsSubstrate Molecule is H (fucose)Substrate Molecule is H (fucose)Substrate Molecule is H (fucose)Substrate Molecule is H (fucose)A antigen is NA antigen is N--acetylacetyl--galactosamine galactosamine (GalNAc)(GalNAc)(GalNAc)(GalNAc)B antigen is Galactose (Gal)B antigen is Galactose (Gal)A and B genes code for transferase A and B genes code for transferase enzymesenzymesenzymesenzymes

ABO AntibodiesABO AntibodiesABO AntibodiesABO Antibodies

A and B substances very commonA and B substances very commonA and B substances very commonA and B substances very commonAntibodies produced to nonAntibodies produced to non--selfselfP d d ft fi t f th f lifP d d ft fi t f th f lifProduced after first few months of lifeProduced after first few months of lifeA & B people have mainly IgMA & B people have mainly IgMO people have IgGO people have IgGMay fade in old ageMay fade in old ageMay fade in old ageMay fade in old age

Antigens & AntibodiesAntigens & AntibodiesAntigens & AntibodiesAntigens & Antibodies

Blood Group Antigens on RBCs Antibodies in Serum Genotypes

A A Anti-B AA or AO

B B Anti-A BB or BO

AB A and B Neither AB

O Neither Anti-A and anti-B OO

Inheritance of ABO GroupsInheritance of ABO Groups

Allele from the mother

Allele from the father

Genotype ofoffspring

Blood types ofoffspring the mother the father offspring offspring

A A AA A

A B AB AB

A O AO A

B A AB AB

B B BB B

B O BO B

O O OO OO O OO O

ABO TypingABO TypingABO TypingABO Typing

Cell GroupCell Group Reverse GroupReverse GroupCell GroupCell Group Test Washed Cells Test Washed Cells

With:With:

pp Test plasma/serum Test plasma/serum

with:with:Known AKnown A cellscells Monoclonal AntiMonoclonal Anti--AA

Monoclonal AntiMonoclonal Anti--BBI t t lI t t l

Known AKnown A11 cellscells Known B cellsKnown B cells Known O cellsKnown O cells

Inert controlInert controlAgglutination is a Agglutination is a positive resultpositive result

? Known A? Known A22 cellscellsReactions may be Reactions may be

k th llk th llpositive resultpositive result weaker than cell weaker than cell groupgroup

Distribution of ABO GroupsDistribution of ABO GroupsDistribution of ABO GroupsDistribution of ABO Groups

Population O A B AB

Aborigines 61 39 0 0Basques 51 44 4 1Basques 51 44 4 1Blackfoot (N. Am. Indian) 17 82 0 1Bororo 100 0 0 0Chinese-Canton 46 23 25 6Chinese Peking 29 27 32 13Chinese-Peking 29 27 32 13English 47 42 8 3Hawaiians 37 61 2 1Irish 52 35 10 3M 98 1 1 1Mayas 98 1 1 1Navajo (N. Am. Indian) 73 27 0 0Peru (Indians) 100 0 0 0United Kingdom (GB) 47 42 8 3g ( )USA (blacks) 49 27 20 4USA (whites) 45 40 11 4

Distribution of theDistribution of the AA allelealleleDistribution of the Distribution of the AA alleleallele

Distribution of the Distribution of the BB AlleleAllele

Distribution of the Distribution of the OO AlleleAllele

Significance of ABO GroupSignificance of ABO GroupSignificance of ABO GroupSignificance of ABO Group

ABO mismatched transfusions:ABO mismatched transfusions:ABO mismatched transfusions:ABO mismatched transfusions: RareRare

May be life threateningMay be life threatening May be life threateningMay be life threatening Can be caused by technical or clerical errorCan be caused by technical or clerical error

Intra asc lar haemol sisIntra asc lar haemol sis Intravascular haemolysisIntravascular haemolysis More severe in group O patientsMore severe in group O patients

Universal Donor and RecipientUniversal Donor and RecipientUniversal Donor and RecipientUniversal Donor and Recipient

Universal DonorUniversal Donor Universal RecipientUniversal RecipientUniversal DonorUniversal DonorGroup OGroup O Carries no A or BCarries no A or B

Universal RecipientUniversal RecipientGroup ABGroup AB Patient has no antiPatient has no anti--AACarries no A or B Carries no A or B

antigensantigens Packed and processed Packed and processed

it h littlit h littl

Patient has no antiPatient has no anti A A or antior anti--B presentB present

Cannot lyse any Cannot lyse any t f d llt f d llunits have little units have little

antibodyantibodytransfused cellstransfused cells

Beware: otherBeware: otherantibodies may beantibodies may be antibodies may be antibodies may be presentpresent

Using the patients own group ASAP will conserve resources

The Rh(D) AntigenThe Rh(D) AntigenThe Rh(D) AntigenThe Rh(D) Antigen

RH is the most complex system, withRH is the most complex system, withRH is the most complex system, with RH is the most complex system, with over 45 antigens over 45 antigens Discovered in 1940 after work on Discovered in 1940 after work on Rhesus monkeysRhesus monkeysSubsequently discovered to be Subsequently discovered to be q yq yunrelated to monkeysunrelated to monkeysRHRH gene located on short arm of gene located on short arm of chromosome 1chromosome 1

Simple Genetics of Rh(D)Simple Genetics of Rh(D)Simple Genetics of Rh(D)Simple Genetics of Rh(D)

86% of caucasians are Rh(D) pos86% of caucasians are Rh(D) pos86% of caucasians are Rh(D) pos86% of caucasians are Rh(D) posThe antithetical antigen d has not been The antithetical antigen d has not been foundfoundfoundfoundThe The dd gene is recessive:gene is recessive: Dd, dD, DDDd, dD, DD, persons are Rh(D) pos, persons are Rh(D) pos Only Only dd dd persons are Rh(D) negpersons are Rh(D) neg

Distribution of Rh(D) TypesDistribution of Rh(D) TypesDistribution of Rh(D) TypesDistribution of Rh(D) Types

PopulationPopulation Rh(D) posRh(D) pos Rh(D) negRh(D) negPopulationPopulation Rh(D) posRh(D) pos Rh(D) negRh(D) neg

CaucasianCaucasian 86%86% 14%14%CaucasianCaucasian 86%86% 14%14%

AfricanAfrican 95%95% 5%5%AfricanAfrican--AmericanAmerican

95%95% 5%5%

OrientalOriental >99%>99% 99%

Significance of Rh(D)Significance of Rh(D)Significance of Rh(D)Significance of Rh(D)

80% of Rh(D) neg persons exposed to Rh(D)80% of Rh(D) neg persons exposed to Rh(D)80% of Rh(D) neg persons exposed to Rh(D) 80% of Rh(D) neg persons exposed to Rh(D) pos blood will develop antipos blood will develop anti--DDAntiAnti--D can also be stimulated by pregnancy with D can also be stimulated by pregnancy with y p g yy p g yan Rh(D) positive babyan Rh(D) positive baby Sensitisation can be prevented by the use of antiSensitisation can be prevented by the use of anti--D D

immunoglobulin, antenatally and post natallyimmunoglobulin, antenatally and post natallyRh(D) neg females of childbearing potential Rh(D) neg females of childbearing potential h ld b i Rh(D) iti bl dh ld b i Rh(D) iti bl dshould never be given Rh(D) positive blood should never be given Rh(D) positive blood

productsproducts

Significance of Rh(D)Significance of Rh(D)Significance of Rh(D)Significance of Rh(D)

80% of Rh(D) neg persons exposed to Rh(D)80% of Rh(D) neg persons exposed to Rh(D)80% of Rh(D) neg persons exposed to Rh(D) 80% of Rh(D) neg persons exposed to Rh(D) pos blood will develop antipos blood will develop anti--DDAntiAnti--D can also be stimulated by pregnancy with D can also be stimulated by pregnancy with y p g yy p g yan Rh(D) positive babyan Rh(D) positive baby Sensitisation can be prevented by the use of antiSensitisation can be prevented by the use of anti--D D

immunoglobulin, antenatally and post natallyimmunoglobulin, antenatally and post natallyRh(D) neg females of childbearing potential Rh(D) neg females of childbearing potential h ld b i Rh(D) iti bl dh ld b i Rh(D) iti bl dshould never be given Rh(D) positive blood should never be given Rh(D) positive blood

productsproducts

InheritanceInheritanceInheritanceInheritance

ABOABO && RHRH genes are not linkedgenes are not linkedABOABO & & RHRH genes are not linkedgenes are not linkedABO & Rh(D) type are inherited ABO & Rh(D) type are inherited independentlyindependentlyindependentlyindependentlyFor example:For example:An A Rh(D) pos mother An A Rh(D) pos mother and a B Rh(D) pos father and a B Rh(D) pos father could have an O Rh(D) neg childcould have an O Rh(D) neg child

Inheritance of ABO and Rh(D)Inheritance of ABO and Rh(D)Inheritance of ABO and Rh(D)Inherit