-Hematologie

7/30/2019 -Hematologie

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PARTILE COMPONENTE ALEPARTILE COMPONENTE ALE

SANGELUISANGELUI


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Din ce este compus SANGELE?

plasma

Elemente figurate


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ELEMENTELE FIGURATEELEMENTELE FIGURATE


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GLOBULELE ROSIIGLOBULELE ROSII


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EritropoiezaEritropoieza

eritroblasteritroblastortocromatofilortocromatofil

reticulocitreticulocit

eritroblasteritroblastpolicromatofipolicromatofi

ll

hematie adultahematie adulta

proeritroblastproeritroblast eritroblasteritroblast

bazofilbazofilcelulacelula

stemstem
http://www.som.tulane.edu/classware/pathology/Krause/Blood/EP8.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP8.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP6.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP5.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP4.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP3.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP1.jpg


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Formarea monocitelorFormarea monocitelor

celula stemcelula stem monocitmonocitmonoblastmonoblast promonocitpromonocit
http://www.som.tulane.edu/classware/pathology/Krause/Blood/Monocyte.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/promonocyte.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/Monoblast.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/StemCell.jpg


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Formarea limfocitelorFormarea limfocitelor

limfocitlimfocitprolimfocitprolimfocitlimfoblastlimfoblastcelula stemcelula stem
http://www.som.tulane.edu/classware/pathology/Krause/Blood/BL8b.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/prolymphocyte.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/Lymphoblast.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/StemCell.jpg


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Producerea plachetelorProducerea plachetelor

(trombocitelor)(trombocitelor)


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ema opo ezaema opo eza(locurile in care se formeaza elementele(locurile in care se formeaza elementele

figurate)figurate)


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Diferentierea celulelor inDiferentierea celulelor in

cursul hematopoiezeicursul hematopoiezei

INTRAMEDULAR

EXTRAMEDULAR


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)mastocite(


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EritropoiezaEritropoieza

eritroblasteritroblastortocromatofiortocromatofi

reticulocitreticulocit

eritroblasteritroblastpolicromatofipolicromatofi

ll

hematie adultahematie adulta

proeritroblastproeritroblasteritroblasteritroblast

bazofilbazofilcelulacelula

stemstem

5zile

2zile + 1 ziPierdere nucleu + maturatie 120zile
http://www.som.tulane.edu/classware/pathology/Krause/Blood/EP5.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP5.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP8.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP6.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP5.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP4.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP3.jpghttp://www.som.tulane.edu/classware/pathology/Krause/Blood/EP1.jpg


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HematocritulHematocritul


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HEMOGLOBINA ( Hb )HEMOGLOBINA ( Hb )


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Parametrii globulelor rosii

Parametri Abreviere Valori normale

Hemoglobina Hb 1418 g/dl, 1216 g/dl

Hematocritul Ht 4052%, 3748%

Numarul eritrocitelor GR 4.35.7 106/l, 3.95.3 106/l)RBC(

Volumul eritrocitar mediu VEM )MCV( 8598 fl

Hemoglobina eritrocitara HEM )MCH( 2834 pgmedie

Concentratia medie a Hb CHEM )MCHC( 3237 g/dldin eritrocit

Diametrul eritrocitului 6.87.3 m

Reticulocite Ret 0.31.5%


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CLASIFICAREA ANEMIILORCLASIFICAREA ANEMIILORAfectarea

celulelor stem

Anemia aplazica

Mielodisplazia

Hemoglobinuriaparoxisticanocturna )HPN(

Anemia

hipocroma

Anemia hemolitica

corpusculara

Anemia

posthemoragica

Anemia feriprivaTalasemia

Anemia dinboli cronice

TalasemiileSiclemia

Sfero-eliptocitozaDeficitul de G6PDH

HPN

Hemoragia cronicaHemoragia acuta

Infiltratiemedulara

Anemiamegaloblastica

Anemia hemoliticaextracorpusculara Hipersplenism

Leucemie

LimfomTumori solide

Deficit de

vitamina B12/acid folic

Hemoliza imunaTrauma

MicroangiopatiileNoxe/infectiiPerturbari

metabolice

Splenomegalia

Deficit deeritropoietina

Anemia renala


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Clasificarea anemiei dupa VEM si HEMClasificarea anemiei dupa VEM si HEM

Anemie microcitara(VEM < 80 fl)

hipocroma(HEM < 27 pg)

1) Anemie prin deficit de fier (Fe)2) Talasemiile ( si )

3) Anemia din bolile cronice

Anemie normocitara(VEM 80 100 fl)

normocroma(HEM > 27 pg)

1) Sangerare recenta2) Anemia din bolile cronice

3) Cele mai multe din deficitele nonhematinice4) Deficit combinat (Fe + B12 /folati)

1) Deficit de folati sau de B12

2) Anemie hemolitica

Anemie macrocitara(VEM > 100 fl)

3) Boala hepatica

4) Displazie medulara si insuficienta medulara

inclusiv anemia aplastica

5) Anemia secundara unor antimetabolite (e.g. :

hidroxiuree)

Primele sugestiiPrimele sugestiina zedi i l


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Primele sugestiiPrimele sugestii

Ret.

ANEMIE

Nr. Er. ; Hb

Ht

Inspectia

frotiului de

sange

Ret.

VEM; CHEM

aditionaleVEM CHEM

feripriva cronicasimpla

sideroblastica taratalasemica

VEM CHEM =N

megaloblastica- carentiala- noncarentiala

nonmegaloblastica- boala cronica de ficat- hipotiroidie

VEM = N CHEM =N

aplaziemedulara dislocaremedulara

(neoplazie,fibroza,scleroza)

VEM, CHEM =variabile

hemolize sechestraresplenica stare post-hemoragie acuta

1. Bilantul fierului- compartimentul circulant: sideremie, CTLF- compartimentul de utilizare: feritinemia,

hemosiderina medulara, sideroblastii inelari2. Electroforeza hematoglobinei

1. Examenul maduvei osoase2. Bilantul catalizatorilor: cobalaminemia,

folatemia + folatul eritrocitar3. Testul Schiling, aciditatea gastrica dupa

stimulare chimica

1. Punctie-biopsie medulara2. Bilantul fierului3. Dozarea eritropoietinei circulante4. Culturi medulare5. Examen citogenetic

1. Teste de hemoliza2. Frotiul medular3. Teste pentru hemolize imune: test Coombs,

aglutenine la rece, hemolizine bifazice4. Fragilitatea osmotica; autohemoliza5. Testul Ham

6. Testul de siclizare7. Electroforeza hemoglobinei

Ret.


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Schema de investigatie a anemieiSchema de investigatie a anemiei


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Morfologia eritrocitelor la normali si in diferite tipuri deMorfologia eritrocitelor la normali si in diferite tipuri deanemieanemieMorfologia eritrocitelor la normali si in diferite tipuri deMorfologia eritrocitelor la normali si in diferite tipuri deanemieanemie

Morfologia eritrocitelor La normali si in diferite tipuri deanemie

Disc biconcav Normali

Microcit hipocrom Deficit de fierTalasemie

Macroovalocit Anemie megaloblastica

Microsferocit Sferocitoza ereditaraHemoliza autoimuna

Eliptocit Eliptocitoza ereditara

Eritrocit in tinta Hemoglobina C, SCTalasemie, hepatopatie

Eritrocit in secera Hemoglobina SS, SCS talasemie

Eritrocit in picatura Mielofibroza

Talasemie majora

Eritrocit fragmentat Hemoliza microangiopatica / mecanica

Stomatocit Stomatocitoza ereditara

Acantocit HepatopatieAbetalipoproteinemie

Echinocit Insuficienta renala (uremie)


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ANEMIA FERIPRIVAANEMIA FERIPRIVA


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Metabolismul fieruluiMetabolismul fierului


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AbsorbtiaAbsorbtia

Tf = transferina,implicata intransportul fierului

Impiedica absorbtia fierului


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Utilizarea fieruluiUtilizarea fierului
http://content.nejm.org/content/vol350/issue23/images/large/10f1.jpeg


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Fierul este stocat in ficat

prin intermediul feritinei

Circu

itulfieru

lui
http://content.nejm.org/content/vol350/issue23/images/large/10f1.jpeghttp://content.nejm.org/content/vol350/issue23/images/large/10f1.jpeghttp://content.nejm.org/content/vol350/issue23/images/large/10f1.jpeghttp://content.nejm.org/content/vol350/issue23/images/large/10f1.jpeg


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Rolul fierului inproducereahemoglobinei

normocromie

hipocromie


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Unghii friabileUnghii friabile


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Glosita atroficaGlosita atrofica


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Anemiapernicioasa

si feripriva


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ANEMIAANEMIA

HEMOLITICAHEMOLITICA


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Circuitul hematiilorCircuitul hematiilor1.formare

2.Indeplinirea functiilor

3.fagocitoza

4.reciclarea componentelor

5.producerea de noi hematiiprin folosirea elementelor reciclate

METBOLISMUMETBOLISMU


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METBOLISMUMETBOLISMU

LL

BILIRUBINEIBILIRUBINEI


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HEMOGLOBINA

HEMGLOBINA

PROTOPORFIRINA

BILIVERDINA

BILIRUBINA NECONJUGATA

AMINOACIZI

HEM OXIGENAZA

BILIVERDIN REDUCTAZA

FIER


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BILIRUBINA LEGATA DE ALBUMINABILIRUBINA NECONJUGATA LIBERA


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BILIRUBINA LEGATA DE ALBUMINA

DETASAREA DE ALBUMINA

RETICULUL ENDOPLASMATICNETED

BILIRUBINA CONJUGATA

LIGAND Y&Z PROTEIN

URIDINE DIPHOSPHATEGLUCORYL TRANSFERAZA E

URINAINTESTIN


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BILIRUBINA CONJUGATA

STERCOBILINOGEN

FLORA BACTERIANA INTESTINALA E-COLI

OXIDARE

B-GLUCURONIDAZA E

CIRCULATIA ENTEROHEPATICA


STERCOBILIN


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[deficienta de G6PD][deficienta de G6PD]


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Boabe de fasoleBoabe de fasole


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SICLEMIA


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PATOFIZIOLOGI

E

Infarct osos (necroza de capInfarct osos (necroza de cap


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Infarct osos (necroza de capInfarct osos (necroza de caphumeral)humeral)


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TALASEMIILETALASEMIILE


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Distributie geograficaDistributie geografica


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MOD DE TRANSMITEREMOD DE TRANSMITERE

PATOFIZIOLOGIA PATOFIZIOLOGIA


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PATOFIZIOLOGIA -PATOFIZIOLOGIA -

TALASEMIEI MAJORETALASEMIEI MAJORE

MANIFESTARILE CLINICE ALEMANIFESTARILE CLINICE ALE


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MANIFESTARILE CLINICE ALEMANIFESTARILE CLINICE ALE

- TALASEMIEI MAJORE - TALASEMIEI MAJORE


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Celulele in tintaCelulele in tinta

RADIOLOGICRADIOLOGIC


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RADIOLOGICRADIOLOGIC

INVESTIGATIONINVESTIGATION

Plain x ray skullPlain x ray skull

There is maxillaryThere is maxillary

over growth,over growth,prominentprominentwidening of thewidening of thediploic spaces, withdiploic spaces, with

hair standing onhair standing onend appearanceend appearance..


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TESTE DE LABORATORTESTE DE LABORATOR

De rutin:De rutin:Numr trombocite 150-300,000/ mmcNumr trombocite 150-300,000/ mmcTimp de sngerare 4-6 minuteTimp de sngerare 4-6 minuteTimp de coagulare 6-10 minuteTimp de coagulare 6-10 minuteTimp de protrombin (PT) 10-12 secTimp de protrombin (PT) 10-12 sec

Timp de tromboplastin parial activat (PTT) 25-36 secTimp de tromboplastin parial activat (PTT) 25-36 secTimp de trombin (TT) 9-35 sec (15-18 sec)Timp de trombin (TT) 9-35 sec (15-18 sec)Fibrinogen 200-400 mg/dlFibrinogen 200-400 mg/dlFragmente de fibrinogen/fibrin


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Funcia vascularFuncia vascular

Testul Rumple Leed normal (5 minute)Testul Rumple Leed normal (5 minute)max 5 peteiimax 5 peteii

Testul cupa de suciune (5 minute)Testul cupa de suciune (5 minute)

>5 peteii=fragilitate vascular>5 peteii=fragilitate vascularcrescutcrescut


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Teste hemostaza primarTeste hemostaza primar

Timp de sngerare (TS metoda Ivy)Timp de sngerare (TS metoda Ivy)detecteaz defectele funcieidetecteaz defectele funcieitrombocitelor.trombocitelor.

Relaie direct ntre numrul deRelaie direct ntre numrul detrombocite i TS:


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TS valoros n detectarea defectelorTS valoros n detectarea defectelor

funcionale ale trombocitelor:funcionale ale trombocitelor:1. Uremie, ingestie aspirin : TS crete1. Uremie, ingestie aspirin : TS crete

(8-15 min)(8-15 min)

2.2. vWDvWD (defect congenital n sinteza(defect congenital n sintezaVWF); TS>30 minute.VWF); TS>30 minute.

Limit: nu difereniaz anomaliileLimit: nu difereniaz anomaliile

cantitative trombocitare de celecantitative trombocitare de celecalitative .calitative .


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Daca un vas deDaca un vas de

sangesange

este lezateste lezat

apare un raspunsapare un raspunslocallocal

rapid in vederea opririirapid in vederea opririihemoragieihemoragiei


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Imposibilitatea coagularii conduce laImposibilitatea coagularii conduce la

afectiuni hemoragiceafectiuni hemoragice

MECANISMELE HEMOSTAZEIMECANISMELE HEMOSTAZEI


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MECANISMELE HEMOSTAZEIMECANISMELE HEMOSTAZEI

Formarea cheagului hemostatic
http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2


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Formarea cheagului hemostatic

Hemostazie primara

adeziune

activare

Agregare

fibrinogen

GPIIB,IIIA

Hemostazie secundara

FORMAREACHEAGULUI DE

FIBRINA

eliberare
http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=%5Cwebsites%5Cemedicine%5Cmed%5Cimages%5CLarge%5C15371738EMEDFIG1.JPG&template=izoom2


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Nivelul activitatii &Nivelul activitatii &


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Nivelul activitatii &Nivelul activitatii &severitatiiseveritatiiTip Activitate

%

Tipul hemoragiei

sever


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Terapie geneticaTerapie genetica


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PURPURELEPURPURELE


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PatogenezaPatogeneza

Mecanism autoimuneMecanism autoimune

i d


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Timp de sangerareTimp de sangerare

4-6minute

O OC O


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TROMBOCITOPENIATROMBOCITOPENIA

LEZIUNI PURPURICELEZIUNI PURPURICE


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LEZIUNI PURPURICELEZIUNI PURPURICE

PURPURA HENOCH PURPURA HENOCH


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PURPURA HENOCH PURPURA HENOCH

SCHOENLEINSCHOENLEIN


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TRANSFUZIILETRANSFUZIILE


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Pl fPl f


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PlasmaferezaPlasmafereza


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