Post on 14-Apr-2018
7/30/2019 -Hematologie
1/87
7/30/2019 -Hematologie
2/87
PARTILE COMPONENTE ALEPARTILE COMPONENTE ALE
SANGELUISANGELUI
7/30/2019 -Hematologie
3/87
Din ce este compus SANGELE?
plasma
Elemente figurate
7/30/2019 -Hematologie
4/87
ELEMENTELE FIGURATEELEMENTELE FIGURATE
7/30/2019 -Hematologie
5/87
GLOBULELE ROSIIGLOBULELE ROSII
7/30/2019 -Hematologie
6/87
7/30/2019 -Hematologie
7/87
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.jpg7/30/2019 -Hematologie
8/87
7/30/2019 -Hematologie
9/87
7/30/2019 -Hematologie
10/87
7/30/2019 -Hematologie
11/87
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.jpg7/30/2019 -Hematologie
12/87
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.jpg7/30/2019 -Hematologie
13/87
Producerea plachetelorProducerea plachetelor
(trombocitelor)(trombocitelor)
7/30/2019 -Hematologie
14/87
7/30/2019 -Hematologie
15/87
ema opo ezaema opo eza(locurile in care se formeaza elementele(locurile in care se formeaza elementele
figurate)figurate)
7/30/2019 -Hematologie
16/87
Diferentierea celulelor inDiferentierea celulelor in
cursul hematopoiezeicursul hematopoiezei
INTRAMEDULAR
EXTRAMEDULAR
7/30/2019 -Hematologie
17/87
)mastocite(
7/30/2019 -Hematologie
18/87
7/30/2019 -Hematologie
19/87
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.jpg7/30/2019 -Hematologie
20/87
HematocritulHematocritul
7/30/2019 -Hematologie
21/87
HEMOGLOBINA ( Hb )HEMOGLOBINA ( Hb )
7/30/2019 -Hematologie
22/87
7/30/2019 -Hematologie
23/87
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%
7/30/2019 -Hematologie
24/87
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
7/30/2019 -Hematologie
25/87
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
7/30/2019 -Hematologie
26/87
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.
7/30/2019 -Hematologie
27/87
Schema de investigatie a anemieiSchema de investigatie a anemiei
7/30/2019 -Hematologie
28/87
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)
7/30/2019 -Hematologie
29/87
7/30/2019 -Hematologie
30/87
ANEMIA FERIPRIVAANEMIA FERIPRIVA
7/30/2019 -Hematologie
31/87
Metabolismul fieruluiMetabolismul fierului
7/30/2019 -Hematologie
32/87
AbsorbtiaAbsorbtia
Tf = transferina,implicata intransportul fierului
Impiedica absorbtia fierului
7/30/2019 -Hematologie
33/87
Utilizarea fieruluiUtilizarea fierului
http://content.nejm.org/content/vol350/issue23/images/large/10f1.jpeg7/30/2019 -Hematologie
34/87
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.jpeg7/30/2019 -Hematologie
35/87
Rolul fierului inproducereahemoglobinei
normocromie
hipocromie
7/30/2019 -Hematologie
36/87
Unghii friabileUnghii friabile
7/30/2019 -Hematologie
37/87
Glosita atroficaGlosita atrofica
7/30/2019 -Hematologie
38/87
Anemiapernicioasa
si feripriva
7/30/2019 -Hematologie
39/87
7/30/2019 -Hematologie
40/87
ANEMIAANEMIA
HEMOLITICAHEMOLITICA
7/30/2019 -Hematologie
41/87
Circuitul hematiilorCircuitul hematiilor1.formare
2.Indeplinirea functiilor
3.fagocitoza
4.reciclarea componentelor
5.producerea de noi hematiiprin folosirea elementelor reciclate
METBOLISMUMETBOLISMU
7/30/2019 -Hematologie
42/87
METBOLISMUMETBOLISMU
LL
BILIRUBINEIBILIRUBINEI
7/30/2019 -Hematologie
43/87
HEMOGLOBINA
HEMGLOBINA
PROTOPORFIRINA
BILIVERDINA
BILIRUBINA NECONJUGATA
AMINOACIZI
HEM OXIGENAZA
BILIVERDIN REDUCTAZA
FIER
7/30/2019 -Hematologie
44/87
BILIRUBINA NECONJUGATA
BILIRUBINA LEGATA DE ALBUMINABILIRUBINA NECONJUGATA LIBERA
7/30/2019 -Hematologie
45/87
BILIRUBINA LEGATA DE ALBUMINA
DETASAREA DE ALBUMINA
RETICULUL ENDOPLASMATICNETED
BILIRUBINA CONJUGATA
LIGAND Y&Z PROTEIN
URIDINE DIPHOSPHATEGLUCORYL TRANSFERAZA E
URINAINTESTIN
7/30/2019 -Hematologie
46/87
BILIRUBINA CONJUGATA
STERCOBILINOGEN
FLORA BACTERIANA INTESTINALA E-COLI
OXIDARE
B-GLUCURONIDAZA E
CIRCULATIA ENTEROHEPATICA
BILIRUBINA NECONJUGATA
STERCOBILIN
7/30/2019 -Hematologie
47/87
[deficienta de G6PD][deficienta de G6PD]
7/30/2019 -Hematologie
48/87
Boabe de fasoleBoabe de fasole
7/30/2019 -Hematologie
49/87
SICLEMIA
7/30/2019 -Hematologie
50/87
7/30/2019 -Hematologie
51/87
PATOFIZIOLOGI
E
Infarct osos (necroza de capInfarct osos (necroza de cap
7/30/2019 -Hematologie
52/87
Infarct osos (necroza de capInfarct osos (necroza de caphumeral)humeral)
7/30/2019 -Hematologie
53/87
TALASEMIILETALASEMIILE
7/30/2019 -Hematologie
54/87
Distributie geograficaDistributie geografica
7/30/2019 -Hematologie
55/87
7/30/2019 -Hematologie
56/87
MOD DE TRANSMITEREMOD DE TRANSMITERE
PATOFIZIOLOGIA PATOFIZIOLOGIA
7/30/2019 -Hematologie
57/87
PATOFIZIOLOGIA -PATOFIZIOLOGIA -
TALASEMIEI MAJORETALASEMIEI MAJORE
MANIFESTARILE CLINICE ALEMANIFESTARILE CLINICE ALE
7/30/2019 -Hematologie
58/87
MANIFESTARILE CLINICE ALEMANIFESTARILE CLINICE ALE
- TALASEMIEI MAJORE - TALASEMIEI MAJORE
7/30/2019 -Hematologie
59/87
Celulele in tintaCelulele in tinta
RADIOLOGICRADIOLOGIC
7/30/2019 -Hematologie
60/87
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..
7/30/2019 -Hematologie
61/87
7/30/2019 -Hematologie
62/87
7/30/2019 -Hematologie
63/87
7/30/2019 -Hematologie
64/87
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
7/30/2019 -Hematologie
65/87
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
7/30/2019 -Hematologie
66/87
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:
7/30/2019 -Hematologie
67/87
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 .
7/30/2019 -Hematologie
68/87
Daca un vas deDaca un vas de
sangesange
este lezateste lezat
apare un raspunsapare un raspunslocallocal
rapid in vederea opririirapid in vederea opririihemoragieihemoragiei
7/30/2019 -Hematologie
69/87
Imposibilitatea coagularii conduce laImposibilitatea coagularii conduce la
afectiuni hemoragiceafectiuni hemoragice
MECANISMELE HEMOSTAZEIMECANISMELE HEMOSTAZEI
7/30/2019 -Hematologie
70/87
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=izoom27/30/2019 -Hematologie
71/87
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=izoom27/30/2019 -Hematologie
72/87
Nivelul activitatii &Nivelul activitatii &
7/30/2019 -Hematologie
73/87
Nivelul activitatii &Nivelul activitatii &severitatiiseveritatiiTip Activitate
%
Tipul hemoragiei
sever
7/30/2019 -Hematologie
74/87
Terapie geneticaTerapie genetica
7/30/2019 -Hematologie
75/87
PURPURELEPURPURELE
7/30/2019 -Hematologie
76/87
PatogenezaPatogeneza
Mecanism autoimuneMecanism autoimune
i d
7/30/2019 -Hematologie
77/87
Timp de sangerareTimp de sangerare
4-6minute
O OC O
7/30/2019 -Hematologie
78/87
TROMBOCITOPENIATROMBOCITOPENIA
LEZIUNI PURPURICELEZIUNI PURPURICE
7/30/2019 -Hematologie
79/87
LEZIUNI PURPURICELEZIUNI PURPURICE
PURPURA HENOCH PURPURA HENOCH
7/30/2019 -Hematologie
80/87
PURPURA HENOCH PURPURA HENOCH
SCHOENLEINSCHOENLEIN
7/30/2019 -Hematologie
81/87
7/30/2019 -Hematologie
82/87
TRANSFUZIILETRANSFUZIILE
7/30/2019 -Hematologie
83/87
7/30/2019 -Hematologie
84/87
Pl fPl f
7/30/2019 -Hematologie
85/87
PlasmaferezaPlasmafereza
7/30/2019 -Hematologie
86/87
7/30/2019 -Hematologie
87/87