214700976 proteine plasmatice
DESCRIPTION
proteine plasmaticeTRANSCRIPT
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= lichid galben pai clar ce tine celulele sangvine in suspenie in sangele integral,
obtinuta separat in urma centrifugarii (+anticoagulant=ser=plasma fara factori coagulare) sangelui integral
(separare – celule la baza, supernatant - superior)
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= 75% reziduu uscat – precipitat – plasma = 7/9 g Tipuri
Simple-neconjugate- = doar aminoacizi
Conjugate cu grupari prostetice Nucleoproteine, glico-, fosfo-, metalo-, lipo-
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Other Plasma Proteins (1%)
Fibrinogen
(4%)
Albumin
(60%)
Globulin
(35%)
Albumin (60%)
Globulin (35%)
Fibrinogen (4%)
Other Plasma
Proteins (1%)
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Restul de 1% din plasma Hormoni peptidici
Insulina
Prolactina
Glicoproteine
TSH (thyroid- simulating hormone)
FSH (follice stimulating hormone)
LH (luteinizing hormone)
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Transport- albumina, globuline Coagulare, fibrinoliza Mentinere presiune coloid-osmotica plasmatica- albumine Imunologice- Ig, proteine de faza acuta Mentinere echilibru acido-bazic EAB Inhibitori de proteaze- alfa1 antitripsina Depozit proteine pentru crestere si reparare tisulara periferica, esp cond de denutritie
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Sinteza Ficat- majoritara Secundar – plasmocite (sistem limfatic SRE), glande (hormoni)
Degradare sistem reticulo-endotelial
Reutilizare AA
Renal – glomerular filtrarereabsorbtiecatabolizare
Proteine cu GM mica
intestin Traversare in lumen intestinalDigestiereabsorbtie AA
Cantitate mica proteine
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Electroforeza – orientativa – cu densitometrie Salting-out – in functie cantitati de Na/ Sulfat de amoniu 3 grupuri: Fb, albumine, globuline Dozare imunologica– mai rapida+exactade electie Cantitative
Proteine totale = 6-8 g/dl ser !!! Val cn cu crestere 1 fractie si scadere alta fractie -
Anumit tip de proteine
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5 fractii principale Albumine Alfa1, globuline Alfa 1 antitripsina Alfa1 glicoproteina acida Alfa 1 fetoproteina Retinol binding protein
Alfa2 globuline Ceruloplasmina Haptoglobina Alfa2 macroglobulina
Beta globuline Proteina C reactiva Transferina beta2 microglobulina Fractia c3 complement
fibrinogen Gama globuline
Imunoglobuline A<D<E<G<M
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Separare in 6 grupe:
Albumin
α1 - globulins
α2 - globulins
β1 - globulins
β2 - globulins
γ - globulins
Figure is found at http://www.sebia-usa.com/products/proteinBeta.html#
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• Geluri cu 15 sau30 godeuri
Figure is found at http://www.sebia-usa.com/products/proteinBeta.html#
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Folosire densitometru pentru scanare proteinele separate din gel informatie cantitativa despre fractiile proteice
Figure is found at http://erl.pathology.iupui.edu/LABMED/INDEX.HTM
Valori referinta: Total protein 6.0 – 8.0 g/dL Albumin 3.5 – 5.0 g/dL α1-globulins 0.1 – 0.4 g/dL α2-globulins 0.4 – 1.3 g/dL β-globulins 0.6 – 1.3 g/dL γ-globulins 0.6 – 1.5 g/dL
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Hexamer cu 3 tipuri de lanturi: Aα2 Bβ2 δ2 Legat prin legaturi disulfurice
Sinteza ficat Rol- coagulare
Trombina Fibrinogenfibrina
Dispunere fibrina in retea intre ochiurile careia se depoziteaza trombocite
Concentratie plasmtica normala 200-400mg/dl
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Hiperproteinemie Falsa- deshidratare Adevarata- crestere una/ mai multe fractii
Hipoproteinemie Sinteza hepatica deficitara Aport exogen scazut precursori
Malnutritie Malbasorbtie
Excretie exces Boala renala Arsuri
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Proteina majoritara plasma, approx. 60% plasma = 3,8-4,8g/dl Din total albumina: 40% in plasma, si 60% in spatiu extracelular Alcatuire
1 lant polipeptidic cu 585 Aa si 17 lanturi bisulfurice Simpla, fara grupare prostetica Forma elipsoida (cantitate crescuta nu creste densitatea
plasma la fel de mult ca forma alungita tip fibrinogen) Masa moleculara mica 69 kDa Ph izoelectric de 4,7 Timp ½ 20 zile Migreaza cel mai rapid la electroforeza in ph fiziologic, si ultima la metodele de desalinizare Puternic Electronegativa – ph fiziologic Sinteza hepatica-
20g/zi = 25% din sinteza hepatica proteica
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Functii presiune coloid osmotica – rol major – mentine volum sangvin si
distributia lichide corp Prin concentratie mare si Gm mica = 80%presiune plasmatica Hipoalbuminemie retentie tisulara apa = edem
Transportor si ligant = poteina “taxi” pentru substante cu solubilitate redusa in apa (izoleaza componenta hidrofoba) etc- Saruri biliare, Acizi grasi, Hormoni, Bilirubina neconjugata, ioni (Ca), Droguri (aspirina- fat administrare disociaza legaturi albB si depozitare snc B cu aparitie icter nuclear, peniciline, dicumarinice, fenitoin etc; administrare simultana 2 medicamente cu afinitati similarecreste componenta libera activa a unuia afecteaza potenta medicament)
Rol nutritional- Depozit de AA pt tes periferice – sinteza proteica limitata in special in conditii de aport scazut nutritional Aa
Rol tampon – cea mai mare capacitate tampon comparativ cu restul proteine plasmatice (cantitate cea mai mare, resturi de histidina multe)
Viscozitate – visozitate redusa Regleaza activare plasminogen – influenteaza viteza fibrinoliza Antioxidant –sechestrare ioni Cu-previne formare specii reactive O
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Rara Sindroame de deschidratatre Regenerare hepatica
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Fiziologica- sarcina- Hemodilutie
Repaus prelungit la pat Patologica
Aport scazut precursori Malnutritie Malabsorbtie
Sinteza hepatica deficitara Boli hepatice
Excretie excesiva Gastroenteropatii exudative Sdr nefrotic
Consum exagerat Infectii severe Traumatism Inflamatie Arsuri
Sinteza scazuta Reactie inflamatorie acuta - proteina de RIA “neagtiva “ Reactie inflamtorie cronica
Hiperhidratare Analbuminemie – hipoalbuminemie idiopatica
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Haemodilution
Loss from the body
Acute phase response
Decreased synthesis
Pregnancy
Chronic illness
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Grupa a doua de desaturare cu Na
Electroforeza tipuri: α1,α2,β,δ
Gm 90.000- 1.300.00 kDa
Sinteza Hepatica pt a si b
SRE – g- plasmocite si tesut limfoid
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Glicoproteine
Exemple: Alfa1 antitripsina
Orosomucoid= alfa 1 glicoproteina acida
Alfa1 fetoproteina
Retinol binding protein
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Nume- descoperita prin inhibare in vitro tripsina - glicoproteina (394 Aa, 3 lanturi oligozaharidice) Fractia majoritara a1globuline, > 90% = 100-200mg/dl = proteina de faza acuta Inhibitor de serin-proteaze (formare de complexe cu acestea- legare proteaze de rest metionina al AAT)
Ex: elastaza neutrofilica, tripsina Eliberata de leucocite in caz de infectie Lezare plaman- lipsa control de catre AAT
Activitate - 3 categorii Normala- nivele plasmatice normale Deficit – nivele plasmatice scazute dar detectabile – risc crescut pt
boala hepatica, pulmonara Absenta- nivele plasmatice nedetectabile, risc crescut emfizem
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Sinteza Principal celule hepatice, monocite, eliberata pasiv in circultie Secundar- celule epiteliale si macrofagele alveolare Polimorfism genetic - 75 forme - numerotate de la AZ
M forma normala – migreaza la centru gel elforeza Rest forma notate AL daca migreaza dupa; sau N daca migreaza pre Nivele AAT in ser in functie de genotip
PiMM: 100% (normal)
PiMS: 80% normal ser A1AT
PiSS: 60% normal ser A1AT
PiMZ: 60% normal ser A1AT
PiSZ: 40% normal ser A1AT
PiZZ: 10-15% deficit sever alpha 1-antitripsina
genotipul majoritar MMfenotip Pi (proteaze inhibitor) MM Enzima poate fi
deficitara deficit plicaturare = degradare excesiva in ribozomi nivele scazute serice,
alterata Polimerizare in RE retinute in RE
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Mutatie genetica (gena SERPINA1) forma anormala proteina Hepatic:
secretie hepatica de molecule anormale tripsinapolimerizare moleculeblocare in reticul endoplasmatic hepatociticter colestatic obstructiv + formare de colagen (mecanism necunoscut) fibrozaciroza hepatica
Pulmonar Lipsa control elastaza neutrofilica distructie alveolaraboala pulmonara
Deficit AAT(alfa1 ATD, AATD) = Defect homozigot alela pulmonar
BPOC Adulti – esp emfizem Fumatorti- fact risc – ( fumatul oxidare rest metionina metionin sulfoxid = inactiv ;
creste activiatatea proteazelor) Fumatori- varsta debut boala- 40-50 ani Nefumatori- debut boala 60 ani, durata viata normala
Boala hepatica adulti, copii Nn- rara- manifestare precoce
Aminotransferaze serice crescute icter colestatic cu ciroza copilarie, neo hepatic adult, hepatomegalie
Adulti (; 45 ani) Incidenta creste cu varsta Ciroza, (fibroza),carcinom hepato-celular + hemoragie – transformare AAT in inhibitor factori de coaulare (Pi Pittsburgh-
forma f rara), anevrisme, colita ulcerativa, boala glomerulara Heterozigoti- boala clinica rara, esp fumatori Diagostic- clinic+ determinare valori serice Tratament- AAT purificata/recombinata in studiu, stop fumat, bronhodilalatoare,trat precoce infectii respiratorii, transplant hepatic sau pulmonar
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Proteina de faza acuta Sinteza hepatica = conjugata - glicoproteina
Grad glicozilare mare, componenta Ch= 45% = 0,6-1,4 g/dl, 1-3% proteine plasma Rol – necunoscut inca
imunomodulator In vitro- scadere capacitate fagocitica neutrofile, inhibitie formare
anion superoxid, inhibitie migrare Timocite murinice - Ajuta macrofagele sa inhibe efectul proliferativ
al IL1 Scade agregare plachetara Inhiba raspuns proliferativ anti CD3 al limfocite Studii in vitro – lant CH – moduleaza raspunsul leucocite Studii- moduleaza inflamatia, si raspunsul imun – mecanism
necunoscut Capacitate intrinseca de a induce raspunsuri citosolice legate de ca
la neutrofile- posibil molecula semnal-direct implicata in reglarea functii neutrofile
Legare si transport- medicamente bazice si neutre lipofilice ex: fenobarbital (acide – albumina)
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Clinica – folosit ca marker pt boala inflamatorie intestinala – marker evolutivitate boala Crohn patologie
Raspuns inflamator acut- valori crescute Pattern de glicozilare- alterat in anumite conditii fizio-patologice
Ramificare scazuta a N-glicani atasati- raspuns faza acuta
Ramificare crescuta si grad crescut fucozilare- boli inflamatorii cronice – ex artita reumatoida
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Familie genetica albuminoida : ALB VIT D binding (Gc) protein DBP AFP Alfa-ALB – afamina
Productie Initial- canal vitelin Apoi-ficat fetal
Rol- nu f bine cunoscut Dezvoltare embrionara si fetala
?- posibil- moduleaza raspunsul fetal la estrogen Estradiolul- induce o modificare conformationala in AFP
Expune un epitop ce inhiba cresterea- inhiba cresterea sensibila la estrogen si alte procese dependente de estrogen
Induce apoptoza in celulele tumorale- prin activare caspaza3 Regleaza si faciliteaza intrarea acizi grasi (AA, DHA)in celule ce
trec prin procese de crestere si diferentiere Traverseaza placenta cirulatia sangvina
Fat excreta AFP LA Reingestie AFPdegradare de ficat fetal
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Malformatii care scad integritate bariere naturale intre fat-LA- DTN, gastroschizis, defecte renale, omfalocel
Transfer substante fetale LA crescute, creste AFP Asociere AFP- hipotiroidism congenital- corelatie intre AFP serica-TSH crescut, T4 scazut postnatal Anomalii cromozomiale- AFP scazuta – cauze necunsocute- corel sdr Down Teste genetice – include AFP- III test(16-18s)+BHCG+estriol neconjugat, 4 test + Inhibina; test combinat = teste serologice+ eco
Nivele – dublul sau jumatatea medianei- valori patologice- testare aditionala pt a exclude boala , risc > 1/250 amniocoenteza( AFP, colinesteraza serica crescute+leziuni/ viz cod genetic- pt sindroame genetice)
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Valori crescute in ser pac cu carcinom hepatocelular primitiv, neoplasme primitive, carcinom embrionar ovarina / testicular,
!!! Tumora hepatica, AFP > = risc f mare pt cancer hepatic
Valori mai scazut- hepatita fulminant, hepatite ac/cr
Valoare patologica> 550ng/ml Dupa primul an de viata valoare normala < 20ng/ml
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a
cranioschisis
anencefalie
encefalocel
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Spina bifida oculta
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= glicoproteina, 1 lant polipeptidic Sinteza hepatica, eliminare renala Rol
Proteina plasmatica de transport principala pt retinol Capacitate legare 1:1; scade retinol- scade secretia hepatica
RBP hepatic celular>RBP plasmatic, creste retinol invers Solubilizeaza retinol Protejeaza de oxidare
Transfer complex receptori de pe retina, piele, gonade, plamani, glde salivare, alte tesuturi
Mediaza efecte antitumorale deficit
boala hepatica Malnutritie Deficit vit A Inflamatie-infectie
Clinica – folosit ca marker alternativ la retinol seric (metoda scumpa ed determinare , retinol instabil termic)de determinare al vit A in organism
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Ceruloplasmina
Haptoglobina
Alfa2macroglobulina
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= glicoproteina cu 6 atomi Cu in structura (culoare albastra) 20-25 mg/dl Sinteza hepatica Rol-
Proteina de faza acuta
Trasportor- transporta 95% Cu plasmatic (1 molec leaga 6 atomic Cu strans ), rest legare cu albumina dar nu la fel de strans
Activitate enzimatica – Feroxidaza- oxideaza ioni ferosi toxici Fe2 forma ferica Fe3 cuplare cu
transferina explicatie -Deficit transformare Fe2 in Fe3 Acumulare intracel Fe2 =
hemocromatoza primara
Cu oxidaza histaminaza
Antioxidant- oxideaza ion feros toxic ion feric netoxic = previne leziuni oxidative proteine, lipide, ADN Protectie cel neuronale de stres oxidativ – ex: leziuni SNC-
traumatism mecanic, ischemie, (conditii cu nivele crescute de radicali liberi, ioni Fe liberi)
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Nivele crescute- Sarcina, Limfom, inflamatie acuta sau cronica: PAR, angina, bla alzheimer, schizofrenie, sindrom obsesiv
compulsiv, traumatism nerv optic Nivele scazute Deficit Cu Boala hepatica, Boli genetice- boala MENKE, boala Wilson,
aceruloplasminemie
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Adm exogena ½ din cantitate ingerata = absorbit Exces absorbit- eliminat prin bila Seric- legat de proteine – 95% ceruloplasmina, rest albumina Ion liber= toxic
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Dobandit- Cauze - aport scazut- malnutritie, exces Zn, malabsorbtie- sprue etc Clinica :
Calcificare scazuta osoasa Anemie hipocroma neresponsiva la trat cu fe Neutropenie
Dg Niv serice scazute ceruloplamina, Cu
Tratament- Cu po Genetic – sdr Menke
Copii sex M mai frecvent ( fetite – au nevoie de 2X) Transmis X linkat recesiv – deficit/anomalie ATP7A = atpaza transportoare de Cu cel
intestinala cu transport Cu din Golgi in sist venos port Dispozitie anormala Cu in tesuturi: crescut in intestin subtire+renal si scazut SNC,
ficat, ser, rest tesuturi = Cu scazut- proteine Cu= citocrom C oxidaza, ceruloplasmina, lisil oxidaza scade
structura si functie oase, piele, par, sange, SNC Simptome
Retard mental sever, retardcrestere, hipotonie musculara, piele scazuta, sclrere albastrii, varsaturi, diaree, enteropatie cu piedere proteica, hipopigmentare, modif osoase, rupturi arteriale, par-steely/kinky/brittle, vase creier leziuni interne, incovoiate si fragile , rupturi frecvente, osteoporoza,
Dg- Cu, ceruloplasmina, scazute Trata Cu- iv/po
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Toxicitate dobandita Cauze- Absorbtie/ ingestie Cu in exces
Ingestie bautura acida / mancare acida ce a stat in container de cupru; ingestie suicidara sulfat Cu, absorbtie transcutanata prin comprese cu sare Cu pt arsuri, lapte fiert in vase Cu/alama
Clinic - Gastroenterita limitata, greata, varsaturi, diaree, anemie hemolitica, anurie, ciroza hepatica
Tratament-- lavaj gastric, dimercaprol, penicilamina, hemodializa
Deficit congenital- bla Wilson Bla homozigota AR- cr13 – deficit ATP7B – atpaza transportoare
de Cu – in cel hepatica leaga Cu de Ceruloplasmina care il transporta prin sange la tesuturi, elibereaza Cu in exces in bila
Acumulare cu si depozitare ficat, SNC predominant dar si in alte organe
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Patologie Depozite Cu intrahepatic, inflamatie, fibroza, ciroza Difuzie extrahepatica Cu tesuturi
Creier Rinichi Org reproductive Mad os- anemie hemolitica Cornee-
Simptome Debut adolescenta-6-30 ani Hepatita acuta/cr/fulminant, tulb coagulare cu sangerari, oboseala, hipertensiune portala
(oboseala), varice esofagiene, splenomegalie, ascita, metabolism proteine alterat cu acumulare de amoniu- encefalopatie hepatica
Simptome snc- tremor, distonie, disartrie, disfagie, coree, lipsa coordonare, fara siimptome snezitive, doar motorii, parkinsonism (trigiditatem bradikinezie, lentoare, tulburari de echilibru, tremor, ataxie, distonie), convulsii, dementa, psihiatrice(dementa, psihoza, depresie, anxietate), depozitare in ganglioni bazali
Ocular- Inele keiser—fleischer- aurii, verzui-aurii, cataracte Renal- acidoza tubulara renala, nefrocalcinoza, hematurie Avorturi repetate, amenoree, hipoparatiroidism Efecte directe de lezare membrana si de inhibare enzime eritrocit
Dg Ex fanta- ochi Ceruloplasmina- secretata fara Cu= Aceruloplasmina si degradata rapid = nivele scazute, Cu
seric scazut Excretie urinara cu/24 ore - crescuta Biopsie hepatica Viz Cu liber crescut = Cu total seric- Cu ceruloplasmina Cu urinar = crescut Tratament – penciliamina- leaga Cu si eliminare renala, transplant heptic etc
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Proteina de faza acuta =
Alfa2 sialoglicoproteina Heterogenicitate moleculara 2 forme alelice Hp1 si Hp2 cu 3 izforme umane- Hp1-1, Hp1-2, HP2-2 2 lanturi polipeptidice diferite
Lant greu beta – identic la toate haptoglobinele Lant usor alfa – 2 forme- alfa 1 si alfa2
Rol Captare Hb compex Hp-Hbeliminat prin SRE splina;
Rol- previne pierderea de fe previne leziuni stres oxidativ prin fe liber Previne leziuni renale prin depunere HB, si Fe necroza tubulara acuta
Protectie impotriva leziuni celulare- distructie radicali liberi- ex radical hidroxil + Hb complex cu
activitate inhibitorie pe efect vasodilatator al Oxid nitric , aparere nespecifica pe invazie bacteriana (Hem liber necesar pt crestere bacteriana)
Clinica- Monitorizare si screening anemie hemolitica = valori scazute Hp (deja complexata cu Hb); sangerari
extravasculare- Hp normala
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Scadere Mutatie gena/ regiuni reglatorii = ahaptoglobinemei/hipo-
Nefropatie diabetica .
Anemie hemolitica-sistem reticuloendotelial indeparteaza compexul hem-Hapto din organism
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8-10% proteine plasmatice tetramer Glicoproteina 718 kDa 130-300mg/dl Concentratii cerscute ser, LCR Proteina de faza acuta rol
Inhibitor natural serin proteaze – catepsina, chemotripsina, colagenza, elastaza, kalikreina, plasmina, factor Xa, trombina, tripsina – formare de complexe ireversibile
anticoagulant in vivo puternic – inhiba trombina, inhiba fibrinoliza Transportor pentru GF Gena pt A2M- gena candidat plauzibila pt boala alzheimer-cr 12 ( varianta A2M- crescuta
in bla Alzheimer) Factor antiapoptotic important- cel musculare netede vasculare-endoteliale
Patologie Scazute- SRIS, in stari hiperfibrinolitice ( sepsis), insuficienta hepatica, pancreatita –
severitate pancreatita Crescute- sindrom nefrotic prin dimensiune mare (altele scad), boala colagen
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Transferina
Beta2microglobulina
Proteina c reactiva
C3
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Glicoproteina Sinteza hepatica Proteina de faza acuta Functie
Transport ioni Fe de la intestin ficat,splina, muschi, maduva osoasa Leaga ioni Fe liberi din sist circulator Efect bacteriostatic
Patologie Crestere- deficit fe, graviditate, terapie estrogenica Valori scazute- talasemie, anemie falciforma, hepatita cronica, ciroza
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Proteina mica Localizare- suprafat celulara- esp leucocite Patologie
Valori crescute Productie crescuta/
distructie crescuta leucocite- crestere valori b2m serica – cancere Leucemie cronica limfocitara - nr crescut leucocite, gangl largiti Mielom multiplu – cancer plasmocit Limfom non H
Boala glomerulara renala- filtrare deficitara, creste valoarea serica, scade val urinara
Valori scazute Boala tubulara renala- lipsa reabsorbtie --: eliminare urinara
crescuta, valori serice scazute, post transplant renal- valori crescute- semn rejet grefa
Infectii virale – CMV, HIV
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Proteina de faza acuta Sinteza hepatica sub factori prezentati de catre macrofage si adipocite Cel mai specific marker de faza acuta- superior VSH Denumire- ser pacienti cu boala inflamatorie acuta interactioneaza (perecipita)cu polizaharidele C din extractul de pneumococ Alcatuire-
5 unitati neglicozilate polipeptidice –aranjate sub forma pentamer Inflamatie corp- valoare poate creste de 200X normalul Functie
Promoveaza adeziunea monocite-endoteliu prin receptroi Fc la nivelul celula umana aortica in cond de flux
Legare de membrana celulara lizataformare complexactivare complement opsonizarefagocitozacitoliza
A macrofagele Recunoaste si elimina toxine endogene eliberate din tes lezate
Patologie Valori crescute- boli acute/cr inflamatorii, infectii bacteriene (cresc in primele 2
ore, maxim 48 ore),trauma severa, procese neoplazice, ima-bla coronariana acuta, bla crohn, par, colita ulcerativa, valori bazale crescute (risc dz, bca,hta)
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= componentul central al sistemluiu complemetului Produs de macrofagele activate Rol
Sistemul imun Indicator puternic de IMA Proteina cheie inflamatorie activata in bla alzheimer Rol neuroprotectiv pe creier
Clinic- scazute- glomerulonefrita postinfectioasa, nefrita de sunt
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= anticorpi - sinteza plasmocite= ly B transformate in prezenta antigen Capacitete productie de 10 la a 15a tipuri de Ac Rol
Leaga antigene, proteine non self = rol aparare
Structura 2 lanturi H, L legate disulfuric Domeniile variabile-
la niv regiuni N terminale H si L – leaga antigene
5 tipuri IgM, IgG, IgD, IgA, IgE - in functie de secventa Aa din regiune constanta lant H(m,g,d,a,e)
In functie regiuni constante lanturi L – tip K/lambda Lanturi glicoproteice- 3-13% CH in functie de tipul de Ac
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75% nivel seric de Ig timp injumatatire 22 zile 4 subclase: 1,2,3,4 – apar in functie de varsta, sex, factori genetici; ex adult IgG seric- 60-70% IgG1 Rol-
raspuns imun secundar
Actiuni Activare sistem c Opsonizare si blocare Ag Functii antivirale si antitoxice
Trav placenta- imunitate umorala fetala si nn
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7% Ig circulante in sange Struct pentamerica- 10 situsuri legare Ag Timp ½ 5-6 zile Rol
Rasp imun Acut, crestere= infectie recenta Activare sistem c, mediere liza celulara
Nu trav placenta Primele Ag sintetizate de fat sub stimul antigenic specific- AB0 aglutinine sangvine
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13% din Ig serice Stuctura dimerica 2 subclase IgA1, IgA2 • Localizare in secretii- nasale, bronhiale,intestinale, salivare, colostru • T ½ 6 zile • Actiune
• Imunitate locala la poarta de intrare • Protectie muc gastro-intestinala nn
• Nivele crescute • Efort fizic, ciroza biliara primitiva, infectii subacute/cronice
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1% din Ig serice Atasate de suprafata Ly B
Rol - Receptor Ag
Nivele crescute- Infectii cronice Mielom IgD
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T ½ 2 zile Cantitati mici in flux sangvin <1mg/ml Rol
Nu sistem imunitar Mediere Hipersensibilitate tip I- imediata, anafilactica
Sinteza IgE de catre plasmocite la reactie cu Ag IgE leaga de mastocite sensibilizare mastocite La al II lea contact –
degranulare mastocitara cu eliberare substante- vasoactive- histamina, serotonina, subst pt sirs- Pg, leucotriene, citokine Il1,2,3,4,5,6, enzime proteolitice vasodilatatare, crestere permeabilitate capilara, reactie
alergica
Valori crescute Pacienti atopici- legate de mastocite circulante, bazolfile Infectii parazitare Boli AI Aspergiloza pulmonara
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Fiziologica- copii primul an de viata- inlocuire IgG , IgA, IgM matern IgG, A,M
nou nascut Tranzitorie- nn prematuri, cei cu sinteza deficitara
Patologica Tip sindr imunodeficienta primara- productie alterata
Hipogamaglobulinemie tranzitorie congenitala- intre 3-6 luni- scadere nivele IgG materne, deficit debut sinteaza Ig fetale
Agamaglobulinemie- limfocite B nu se matureaza in plasmocite disgammaglobulinemia
Imunodeficienta dobandita cu anomalii celulare HIV 1,2 Clinic-
infectii recurente din copilarie, gangl limfatici amigdale subdezvl infectii piogenice sevre- sepsis, penumonie, otita, boli autoimune- par, scleroza multiplas,anemie hemolitica
Paraclinic Deficit selectiv- absenta/ scadere 1 clasa- i IgA
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Monoclonala- paraproteinemie – Paraproteine= IG cu structura identica ce apar prin proliferare 1
clona de Ly B Electroforeza- regiune ingusta compacta Cauze-
Maligne- mielom multiplu, limfoame, macroglobulinemia waldenstrom, boala cu lanturi grele
Benigne – paraproteinemie benigna asimptomatica, crioglobulinemie, amiloidiza
Hipergammaglobulinemia policlonala difuza- Electroforeza banda larga difuza Boli ai, infetii cr bacteriene
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Dezvl mad os tes periferice (gangl ly, splina, instetin, inele waldeyer, placi peyer)= diferentiere cel – raspund la n nr limitatd e Ag; interactioneaza cu Ag diferentiere clonala in plasmocite (sinteza de proteine monoclonale =prot M
Din lanturi usoare+grele/ 1 tip de lanturi
Efect de autoAc- lez autoimune organe- esp renal
Scadere productie de Ig normale= scadere imunitate
Imbraca plachete, inactiveaza fac coagulare, cresc vascozitate, sangerar
Infiltrare celulara in mad s osteop, hiper Ca, anemie, pancitopenie
Paraclinic- prot serice ridicate, proteinurie, electroforeza prot serice, electroforeza cu imunofixare- viz lanturi usoare si grele
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Boala neoplazica Hiperproductie monoclonala de lanturi grele Ig –
imunoglobuline monoclonale incomplete- paraproteine adevarate: A, G, M,D,- fragmente lant greu intial (deletii in regiune N terminala – nu pot forma punti diS cu lanturi usoare)
Simptome dg, tratament- in functie de clasa hiperprodusa Boala limfoproliferativa Ex:
boala cu lanturi alfa – cea mai frecventa Boala cu lanturi gama Boala cu lanturi miu Boala cu lanturi delta
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Frecevnta maxima Intre 10-30 ani, in Orient mijlociu frecventa Cauza- raspuns aberant la microorganism/parazit Patologie
Forma digestiva Atrofie viloasa, infiltrare cu cel plasmocite mucoasa jejunala, ganglioni mezenterici;
forma cu tract respirator Neimplicate- splina, maduva osoas, ficat, gangl periferici
Clinic Limfoame abdominale difuze, malabsorbtie
Parclinic Electroforza- normala/ creste fractia alfa2, beta, fractie gama scazuta detectie eletroforetica de banda monclonala de lanta alfa, ser/urina, secr intestinala Biopsie
Tratament- coriticosteroizi, ab spectru larg, agenti citotoxici
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Asemanator limfom Frecvent barbati in etate, copii Asociat cu boli Ai (sdr sjorgen, par) si cu infectii recurente clinica
Limfadenopatii , hepatosplenomegalii, febra, infectii recurente Edem palat- hipertrofie inel Waldeyer
paraclinic Valori IgG normale- scazute Dg- fragmente monoclonale de lanturi grele IgG ser/urina-proteinurie Electroforeza cu imunofixare biopsie mad os, ganglionara
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Boala asemanatoare cu leucemie cronica limfocitica Rar Adulti > 50 ani Clinic
Hepatosplenomegalie, marire gangl lmfatici, fara limafedenopatie periferica extensiva Fracturi patologice
Paraclinic Elforeza prot serice- normala/ hipogamaglobulinemie Proteinurie bence jones- Biopse maduva osoasa
Moarte prin proliferare celule limfocitice
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Boala maligna Cauza necunoscuta Barbati> femei Hiperproductie plasmocitara de IgM M proteine
Unele din ele directionate impotriva secretie normala IgG= factori reumatoizi, antigene I= aglutinine la rece, crioglobuline
Clinic Hipervascozitate sangv- oboseala, slabiciune, sanerare mucosae, peiel tulburari vizuale ,
cefalee, europatie periferica, , insuficeinta cardiaca sangerare, infectii recurente, adenopatii generalizate, hepatosplenomegalie, purpura Sensibilitate la rece- fenomene rayaud
Dg- biopsie maduva osoasa, Electroforeza cu imunofixare - proteine M- apartin IgM , masurare crioglobuline, factor reumatoid, alutinine la rece Anemie normocroma normocitara , leucopenie, limfocitoza , trombocitopenie, Tulburari coagulare
Tratamant- plasmafereza, th sistemica corticosteroizi, …
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Boal maligna Varsta 40 ani, bartbati :femei 1,6/1 Etiologie necunoscuta Patologie
Plasmocitoame- tumori cu celule plasmatice- productie IgG 55%%, IgA 20%, 20%doar proteine bence jones, IgD 1% Proteinurie bence jones- lanturi usoare kappa, lambda
Plasmocitoame extraosoase- rare- tract respirator superior Productie monoclonala IG Distructie tisulara
Clinic Dureri osoase- osteoporoza difuza(pelvin, coloana, craniu, coaste), fracturi patologice, comprimare mad
os, paraplegie lizare osoasa prin etindere plasmocitoame/ activare osteoclaste,
Sdr hipervascozitate- rar Neuropatie periferica, sangerari anormale, sdr canal carpian
Hipercalcemie- poliurie, polidipsie Rara hepatosplenomegalie, limfadenopatie Anemie, Insuficienta renala- formare de mulaje pe tubulii renali, fibroza interstitiala, atrofie celule epiteliale Infectii recurente- productie scazuta Ig normale
dg Proteine urinare, serice crescute Electroforeza- proteine M serice, daca e cu lant usor- fals negativa, electroforeza urniara Electroforeza cu imunofixare – identificare clasa I(inclusiv bence jones) Beta2 microglobulinemia crescuta Rx, rmn- lez osteolitice Biopsi mad os
Tratament- chimioterapie, corticosteroii, transplant meudular
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Frecvent - depunere lantrui usoare
Frecvent - renala – prteinurie
Ficat - hepatomelaglie, hipertensiune portala
Inima – aritmie, insuficienta cardiaca congestiva
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Paraproteinemie- gamapatie monoclonala- 1 singura gamaglobulina monoclonala = paraproteina
Prin neoplazie limfatica
Cu lanturi grele – boala cu lanturi grele – raman in vase
Cu lanturi usoare – bence jones proteins – mielom multiplu – scapa din vase si excretie urina
Cu Ig intregi- M protein- proteina monoclonala
Crioglobulinemie daca proteine precipita la rece in vase- raman intravascular
Macroglobulinemie- paraproteine – frecvent IG intregi- polimerizeaza intre ele- Macroglobulinemie Waldenstrom- cu Ig M; crioglobulinemie
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Albumina 1 2
scadere gamma globuline Conditie Albumina
Globuline
α1 α 2 β γ
Hypogammaglo-
bulinemia N N N N
M. Zaharna Clin. Chem. 2009
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= proliferare monoclonala de clone
limfocite b, ce produc Ig anormale= paraproteine
Ex: leucemie, mielom multiplu
Paraproteine- localizare in pozitii
diferite: intre fractiile α-2 si
γ.
Figure is found at http://erl.pathology.iupui.edu/LABMED/INDEX.HTM
a sharp gamma globulin band
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M. Zaharna Clin. Chem. 2009
Albumina 1 2
Albumin scazuta
Varf in regiunea gamma
111
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Hiperglobulinemie lant greu G, si lanturi usoare kappa si lambda
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Boala cu lanturi usoare
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Pozitive acute phase proteins Proteine ale caror valori cresc in reactie cu inflamatia/infectia
CRP
Ceruloplasmina
Alfa1 AT
Alfa2MG
Alfa1 acid glicoprotein
C
Fibrinogen
Orosomucoid
Haptoglobina
feritina
Negative acute phase proteins Albumina, transtiretrina, rbp, transferina
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• Prin stress sau inflamatie cauzate de infectie, leziune, sau trauma chirurgicala
• Albumina - normala / ↓
• ↑ α1 and α2 globuline
Figure is found at http://erl.pathology.iupui.edu/LABMED/INDEX.HTM
α1 α2-globulins
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• raspunbs tardiv asocit cu:
(boala autoimuna, boala cronica hepatica, infectie cronica, cancer)
• albumina normala / ↓
•↑α1 or α2 globulins
•↑↑ γ globulins
Figure is found at http://erl.pathology.iupui.edu/LABMED/INDEX.HTM
α1 α2 γ-globulins
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Lanturi usoare
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Orosomucoid
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• Cauzata prin consum cronic alcool, hepatita virala cronica
• ↓ albumin
• ↓ α1, α2 and β globulins
• ↑ Ig A in γ-fraction
Figure is found at http://erl.pathology.iupui.edu/LABMED/INDEX.HTM
γ-globulins
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M. Zaharna Clin. Chem. 2009
Scade sinteza albumina
Crestere gammaglobuline (gamapatie policlonala)
Albumin 1 2
“- bridging”
141
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• Leziune renala – prin pierdere proteine cu GM mica
(↓ albumina si IgG – filtrate renal)
• Retentie de proteine cu GM mare (↑↑ α2-macroglobulin sui ↑β-globulin)
Figure is found at http://erl.pathology.iupui.edu/LABMED/INDEX.HTM
α2-globulin β-globulin fractions
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Condition Albumin Globulins
1 2 β γ
Nephrosis N N
Albumin 1 2
Decreased albumin
Increased 2-macroglobulin
Decreased gamma globulins
145
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