Quiz 3 review | September 21, 2016

Quiz3Review

•  Autoimmunediseases

•  Immunodeficiencies

•  Transfusion

•  Transplanta<on

Quiz3Review

•  Autoimmunediseases

•  Typicalpa<ent:youngwomanwithbuBerflyrash

•  Symptomsunpredictable(relapsing/remiGng)

•  Mul<system(skin,kidneys,joints,heart)

•  An<nuclearan<bodies

ThingsYouMustKnow

Lupus

Lupus:buBerflyrash

•  Symmetric,mostlysmall-jointarthri<s

•  Systemicsymptoms(skin,heart,vessels,lungs)

•  Rheumatoidfactor

•  Cytokines(especiallyTNF)causedamage

ThingsYouMustKnow

RheumatoidArthri<s

Rheumatoidarthri<sjointdeformi<es

•  Inflammatorydiseaseofsalivaryandlacrimalglands

•  Dryeyes,drymouth

•  TcellsreactagainstsomeAgingland;glandgetsdestroyed

•  Increasedriskoflymphoma

ThingsYouMustKnow

SjögrenSyndrome

Sjögrensyndrome:salivaryglandenlargement

•  Excessivefibrosisthroughoutbody:skin,viscera

•  Clawhands,mask-likeface

•  Microvasculardiseasealsopresent

•  Diffuseandlimitedtypes

ThingsYouMustKnow

Scleroderma(SystemicSclerosis)

Systemicsclerosis:clawhands

Quiz3Review

•  Autoimmunediseases

•  Immunodeficiencies

Disease Transmission Defect Clinicalstuff

XLA X-linked NomatureBcells;noIg

Infantwithrecurrentbacterialinfec<ons

CVID nonx-linked Defec<veAbproduc<on

Infec<ons,AIdiseases,lymphoma

IgAdeficiency nonx-linked ↓IgA Mostcommon.Asx.AIdiseases

Hyper-IgM X-linked↓IgG,A,ETcelldefect

Bacterialandfungalinfec<ons

DiGeorge nonx-linked Nothymus Viral,fungalinfec<ons

SCID halfarex-linked ManyBandTcelldefects

Bacterialandotherinfec<ons

ImmuneDiseasesOutline

Quiz3Review

•  Autoimmunediseases

•  Immunodeficiencies

•  Transfusion

Genotype An<gens Bloodtype

AAA A

AO

BBB B

BO

AB AandB AB

OO None O

Genotype An<gens Bloodtype

DD D Rh+

Dd D Rh+

dd none Rh-

•  Transfusionreac<ons•  hemoly<c•  non-hemoly<c

•  Othercomplica<ons•  infec<ons•  ironoverload

Whatcangowrong?

Quiz3Review

•  Autoimmunediseases

•  Immunodeficiencies

•  Transfusion

•  Transplanta<on

parents

fourpossiblehaplotype

combina<onsinchildren

♂ ♀

HLAgenesareinheritedassets(haplotypes)

♂ ♀

1 7 w3 2 1 1

2 8 w2 3 2 2

11 35 w1 7 3 4

3 44 w4 4 1 3

A B CDR DQ DP

Dad’sHLAgenes

Mom’sHLAgenes

HLAgenesarepolymorphic

HLAgenesarecodominantlyexpressed

gram

survival,%

50

100

63 12

<meamertransplant,months

0 0 1 or 2 0

3 or 4 0

0 1 or 2

1 or 2 1 or 2

3 or 4 1 or 2

numberofmismatches

ClassI ClassII