Hematologie
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![Page 1: Hematologie](https://reader030.fdocumente.com/reader030/viewer/2022012400/5571f39949795947648e4caa/html5/thumbnails/1.jpg)
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PARTILE COMPONENTE ALE PARTILE 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
eritroblasteritroblast ortocromatofilortocromatofil
reticulocitreticulocit
eritroblasteritroblast policromatofipolicromatofi
ll
hematie adultahematie adulta
proeritroblastproeritroblast eritroblasteritroblastbazofilbazofilcelula celula
stemstem
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Formarea monocitelorFormarea monocitelor
celula stemcelula stem monocitmonocitmonoblastmonoblast promonocitpromonocit
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Formarea limfocitelorFormarea limfocitelor
limfocitlimfocitprolimfocitprolimfocitlimfoblastlimfoblastcelula stemcelula stem
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Producerea plachetelor Producerea plachetelor (trombocitelor)(trombocitelor)
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Hematopoieza Hematopoieza (locurile in care se formeaza elementele(locurile in care se formeaza elementele
figurate)figurate)
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Diferentierea celulelor in Diferentierea celulelor in cursul hematopoiezeicursul hematopoiezei
INTRAMEDULAR
EXTRAMEDULAR
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(mastocite)
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EritropoiezaEritropoieza
eritroblasteritroblast ortocromatofilortocromatofil
reticulocitreticulocit
eritroblasteritroblast policromatofipolicromatofi
ll
hematie adultahematie adulta
proeritroblastproeritroblasteritroblasteritroblast
bazofilbazofilcelula celula stemstem
5 zile
2 zile + 1 ziPierdere nucleu + maturatie
120 zile
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HematocritulHematocritul
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HEMOGLOBINA ( Hb )HEMOGLOBINA ( Hb )
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Parametrii globulelor rosiiParametri Abreviere Valori normale Hemoglobina Hb ♂ 14–18 g/dl, ♀ 12–16 g/dl
Hematocritul Ht ♂ 40–52%, ♀ 37–48%
Numarul eritrocitelor GR ♂ 4.3–5.7 × 106/μl, ♀ 3.9–5.3 × 106/μl (RBC)
Volumul eritrocitar mediu VEM )MCV( 85–98 fl
Hemoglobina eritrocitara HEM )MCH( 28–34 pgmedie
Concentratia medie a Hb CHEM )MCHC( 32–37 g/dldin eritrocit
Diametrul eritrocitului 6.8–7.3 μm
Reticulocite Ret 0.3–1.5%
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CLASIFICAREA ANEMIILORCLASIFICAREA ANEMIILORAfectarea celulelor stem
Anemia aplazica
Mielodisplazia
Hemoglobinuria paroxistica nocturna )HPN(
Anemia hipocroma
Anemia hemolitica corpusculara
Anemia posthemoragica
Anemia feriprivaTalasemiaAnemia din boli cronice
TalasemiileSiclemia
Sfero-eliptocitozaDeficitul de G6PDH
HPN
Hemoragia cronicaHemoragia acuta
Infiltratie medulara
Anemia megaloblastica
Anemia hemolitica extracorpusculara Hipersplenism
LeucemieLimfom
Tumori solide
Deficit devitamina B12
/acid folic
Hemoliza imunaTrauma
MicroangiopatiileNoxe/infectii
Perturbari metabolice
Splenomegalia
Deficit de eritropoietina
Anemia renala
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Clasificarea anemiei dupa VEM si HEMClasificarea anemiei dupa VEM si HEM
Anemie microcitaraAnemie microcitara (VEM < 80 fl)(VEM < 80 fl)
hipocromahipocroma (HEM < 27 pg)(HEM < 27 pg)
1) Anemie prin deficit de fier (Fe)1) Anemie prin deficit de fier (Fe)2) Talasemiile (2) Talasemiile (α α sisi β β))
3) Anemia din bolile cronice3) Anemia din bolile cronice
Anemie normocitara Anemie normocitara (VEM 80 – 100 fl) (VEM 80 – 100 fl)
normocromanormocroma(HEM > 27 pg)(HEM > 27 pg)
1) Sangerare recenta1) Sangerare recenta2) Anemia din bolile cronice2) Anemia din bolile cronice
3) Cele mai multe din deficitele nonhematinice3) Cele mai multe din deficitele nonhematinice4) Deficit combinat (Fe + B12 /folati)4) Deficit combinat (Fe + B12 /folati)
1) Deficit de folati sau de B1) Deficit de folati sau de B1212
2) Anemie hemolitica2) Anemie hemolitica
Anemie macrocitara Anemie macrocitara (VEM > 100 (VEM > 100
fl)fl)3) Boala hepatica3) Boala hepatica
4) Displazie medulara si insuficienta medulara 4) Displazie medulara si insuficienta medulara inclusiv anemia aplasticainclusiv anemia aplastica
5) Anemia secundara unor antimetabolite (e.g. : 5) Anemia secundara unor antimetabolite (e.g. : hidroxiuree)hidroxiuree)
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Primele sugestiiPrimele sugestii
Ret. Ret. ↓↓
ANEMIEANEMIE
Nr. Er. ↓; Hb ↓ Nr. Er. ↓; Hb ↓
Ht ↓Ht ↓
• Inspectia • Inspectia
frotiului de frotiului de
sange sange
• Ret. • Ret.
• VEM; CHEM • VEM; CHEM
Analize Analize aditionaleaditionale
VEM ↓ CHEM VEM ↓ CHEM ↓ ↓
• • feriprivaferipriva• cronica • cronica simplasimpla• • sideroblasticasideroblastica• tara • tara talasemicatalasemica
VEM ↑ CHEM = VEM ↑ CHEM = NN
• • megaloblasticamegaloblastica - carentiala - carentiala - noncarentiala - noncarentiala• nonmegaloblastica• nonmegaloblastica - boala cronica de ficat - boala cronica de ficat - hipotiroidie - hipotiroidie
VEM = N CHEM = VEM = N CHEM = NN
• • aplazie aplazie medularamedulara• dislocare • dislocare medularamedulara(neoplazie, (neoplazie, fibroza,fibroza,scleroza)scleroza)
VEM, CHEM = VEM, CHEM = variabilevariabile
• • hemolizehemolize• sechestrare• sechestraresplenicasplenica• stare post-• stare post-hemoragie acutahemoragie acuta
1. Bilantul fierului1. Bilantul fierului - compartimentul circulant: sideremie, CTLF - compartimentul circulant: sideremie, CTLF - compartimentul de utilizare: feritinemia, - compartimentul de utilizare: feritinemia, hemosiderina medulara, sideroblastii inelari hemosiderina medulara, sideroblastii inelari2. Electroforeza hematoglobinei2. Electroforeza hematoglobinei
1. Examenul maduvei osoase1. Examenul maduvei osoase2. Bilantul catalizatorilor: cobalaminemia, 2. Bilantul catalizatorilor: cobalaminemia, folatemia + folatul eritrocitar folatemia + folatul eritrocitar3. Testul Schiling, aciditatea gastrica dupa 3. Testul Schiling, aciditatea gastrica dupa stimulare chimica stimulare chimica
1. Punctie-biopsie medulara1. Punctie-biopsie medulara2. Bilantul fierului2. Bilantul fierului3. Dozarea eritropoietinei circulante3. Dozarea eritropoietinei circulante4. Culturi medulare4. Culturi medulare5. Examen citogenetic5. Examen citogenetic
1. Teste de hemoliza1. Teste de hemoliza2. Frotiul medular2. Frotiul medular3. Teste pentru hemolize imune: test Coombs, 3. Teste pentru hemolize imune: test Coombs, aglutenine la rece, hemolizine bifazice aglutenine la rece, hemolizine bifazice4. Fragilitatea osmotica; autohemoliza4. Fragilitatea osmotica; autohemoliza5. Testul Ham5. Testul Ham6. Testul de siclizare6. Testul de siclizare7. Electroforeza hemoglobinei7. Electroforeza hemoglobinei
Ret. 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 de Morfologia eritrocitelor la normali si in diferite tipuri de anemieanemieMorfologia eritrocitelor la normali si in diferite tipuri de Morfologia eritrocitelor la normali si in diferite tipuri de anemieanemie
Morfologia eritrocitelorMorfologia eritrocitelorLa normali si in diferite tipuri de La normali si in diferite tipuri de anemieanemie
Disc biconcavDisc biconcavNormaliNormali
Microcit hipocromMicrocit hipocromDeficit de fierDeficit de fierTalasemieTalasemie
MacroovalocitMacroovalocitAnemie megaloblasticaAnemie megaloblastica
MicrosferocitMicrosferocitSferocitoza ereditaraSferocitoza ereditaraHemoliza autoimunaHemoliza autoimuna
EliptocitEliptocitEliptocitoza ereditaraEliptocitoza ereditara
Eritrocit in tintaEritrocit in tintaHemoglobina C, SCHemoglobina C, SCTalasemie, hepatopatieTalasemie, hepatopatie
Eritrocit in seceraEritrocit in seceraHemoglobina SS, SCHemoglobina SS, SCS – talasemieS – talasemie
Eritrocit in picaturaEritrocit in picaturaMielofibrozaMielofibrozaTalasemie majoraTalasemie majora
Eritrocit fragmentatEritrocit fragmentatHemoliza microangiopatica / mecanicaHemoliza microangiopatica / mecanica
StomatocitStomatocitStomatocitoza ereditaraStomatocitoza ereditara
AcantocitAcantocitHepatopatieHepatopatieAbetalipoproteinemieAbetalipoproteinemie
EchinocitEchinocitInsuficienta renala (uremie)Insuficienta renala (uremie)
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ANEMIA FERIPRIVAANEMIA FERIPRIVA
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Metabolismul fieruluiMetabolismul fierului
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Absorbtia Absorbtia
Tf = transferina, implicata in transportul fierului
Impiedica absorbtia fierului
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Utilizarea fieruluiUtilizarea fierului
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Fierul este stocat in ficat
prin intermediul feritinei
Circuitul fierului
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Rolul fierului in producerea hemoglobinei
normocromie
hipocromie
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Unghii friabileUnghii friabile
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Glosita atroficaGlosita atrofica
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Anemia pernicioasa si feripriva
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ANEMIA ANEMIA HEMOLITICAHEMOLITICA
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Circuitul hematiilorCircuitul hematiilor1 .formare
2 .Indeplinirea functiilor
3 .fagocitoza
4 .reciclarea componentelor
5 .producerea de noi hematii prin folosirea elementelor reciclate
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METBOLISMUMETBOLISMU
L L BILIRUBINEIBILIRUBINEI
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HEMOGLOBINA
HEMGLOBINA
PROTOPORFIRINA
BILIVERDINA
BILIRUBINA NECONJUGATA
AMINOACIZI
HEM OXIGENAZA
BILIVERDIN REDUCTAZA
FIER
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BILIRUBINA NECONJUGATA
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
BILIRUBINA NECONJUGATA
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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PA
TO
FIZ
IOLO
GIE
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Infarct osos (necroza de cap Infarct osos (necroza de cap humeral)humeral)
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TALASEMIILETALASEMIILE
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Distributie geograficaDistributie geografica
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MOD DE TRANSMITEREMOD DE TRANSMITERE
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PATOFIZIOLOGIA Β-PATOFIZIOLOGIA Β-TALASEMIEI MAJORETALASEMIEI MAJORE
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MANIFESTARILE CLINICE ALEMANIFESTARILE CLINICE ALE Β - TALASEMIEI MAJORE Β - TALASEMIEI MAJORE
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Celulele “in tinta”Celulele “in tinta”
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RADIOLOGIC RADIOLOGIC INVESTIGATIONINVESTIGATION
Plain x ray skullPlain x ray skull There is maxillary There is maxillary
over growth, over growth, prominent prominent widening of the widening of the diploic spaces, with diploic spaces, with hair standing on hair standing on end appearanceend appearance..
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TESTE DE LABORATORTESTE DE LABORATOR
De rutină:De rutină:Număr trombocite 150-300,000/ mmcNumăr trombocite 150-300,000/ mmcTimp de sângerare 4-6 minuteTimp de sângerare 4-6 minuteTimp de coagulare 6-10 minuteTimp de coagulare 6-10 minuteTimp de protrombină (PT) 10-12 secTimp de protrombină (PT) 10-12 secTimp de tromboplastină parţială activată (PTT) 25-36 Timp de tromboplastină parţială activată (PTT) 25-36
secsecTimp de trombină (TT) 9-35 sec (15-18 sec)Timp de trombină (TT) 9-35 sec (15-18 sec)Fibrinogen 200-400 mg/dlFibrinogen 200-400 mg/dlFragmente de fibrinogen/fibrină <10micrograme/mlFragmente de fibrinogen/fibrină <10micrograme/mlD-dimeri< 500 ng/mlD-dimeri< 500 ng/mlATIII 80-120%ATIII 80-120%Alfa2 antiplasmina 80-120%Alfa2 antiplasmina 80-120%
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Funcţia vascularăFuncţia vasculară
Testul Rumple Leed normal (5 minute) Testul Rumple Leed normal (5 minute) max 5 peteşiimax 5 peteşii
Testul cupa de sucţiune (5 minute)Testul cupa de sucţiune (5 minute)
>5 peteşii=fragilitate vasculară >5 peteşii=fragilitate vasculară crescutăcrescută
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Teste hemostaza primarăTeste hemostaza primară
Timp de sângerare (TS metoda Ivy) Timp de sângerare (TS metoda Ivy) detectează defectele funcţiei detectează defectele funcţiei trombocitelor. trombocitelor.
Relaţie directă între numărul de Relaţie directă între numărul de trombocite şi TS: <100,000, TS trombocite şi TS: <100,000, TS creşte, Tr =10,000, TS=20-30 creşte, Tr =10,000, TS=20-30 minute.minute.
Relaţie directă nr trombocite-TS. Relaţie directă nr trombocite-TS.
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TS valoros în detectarea defectelor TS valoros în detectarea defectelor funcţionale ale trombocitelor:funcţionale ale trombocitelor:
1. Uremie, ingestie aspirină : TS creşte 1. Uremie, ingestie aspirină : TS creşte (8-15 min)(8-15 min)
2. 2. vWD vWD (defect congenital în sinteza (defect congenital în sinteza VWF); TS>30 minute.VWF); TS>30 minute.
Limită: nu diferenţiază anomaliile Limită: nu diferenţiază anomaliile cantitative trombocitare de cele cantitative trombocitare de cele calitative . calitative .
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Daca un vas de Daca un vas de sangesange
este lezateste lezat
apare un raspuns apare un raspuns local local
rapid in vederea opririi rapid in vederea opririi hemoragiei hemoragiei
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Imposibilitatea coagularii conduce Imposibilitatea coagularii conduce la afectiuni hemoragicela afectiuni hemoragice
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MECANISMELE HEMOSTAZEIMECANISMELE HEMOSTAZEI
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Formarea cheagului hemostatic
Hemostazie primara
adeziune activare
Agregare
fibrinogen
GPIIB,IIIA
Hemostazie secundara
FORMAREA CHEAGULUI DE
FIBRINA
eliberare
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Nivelul activitatii & Nivelul activitatii & severitatiiseveritatii TipTipActivitatActivitat
e e
%%
Tipul hemoragieiTipul hemoragiei
seversever<1%<1%Spontana; hemartroza; Spontana; hemartroza; hemoragie interstitiala hemoragie interstitiala profundaprofunda
moderatmoderat1 -5%1 -5%Sangerare ca urmare unui Sangerare ca urmare unui traumatism mic spre moderattraumatism mic spre moderat
usorusor5–25%5–25%Sangerare ca urmare unui Sangerare ca urmare unui traumatism moderat spre traumatism moderat spre seversever
Femeie Femeie purtatorpurtator cu cu risc ridicatrisc ridicat
30-50%30-50%Hemoragii ginecologice & Hemoragii ginecologice & obestetricale obestetricale
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Terapie geneticaTerapie genetica
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PURPURELEPURPURELE
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PatogenezaPatogeneza
Mecanism autoimuneMecanism autoimune
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Timp de sangerareTimp de sangerare
4-6 minute
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TROMBOCITOPENIATROMBOCITOPENIA
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LEZIUNI PURPURICELEZIUNI PURPURICE
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PURPURA HENOCH – PURPURA HENOCH – SCHOENLEINSCHOENLEIN
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TRANSFUZIILETRANSFUZIILE
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Plasmafereza Plasmafereza
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