Download - Curs 1 Clasificare Anemii
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Catedra de Hematologie Catedra de Hematologie Universitatea de Medicina si Farmacie “Carol Davila”Universitatea de Medicina si Farmacie “Carol Davila”
Centrul de Hematologie si Transplant Medular Centrul de Hematologie si Transplant Medular “ Stefan Berceanu”, Institutul Clinic “ Stefan Berceanu”, Institutul Clinic Fundeni Fundeni
Sindroamele anemice: Clasificare
Conf. Dr. Daniel CORIU
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Obiective:Obiective:
Hematopoieza / Fiziologia seriei eritroideHematopoieza / Fiziologia seriei eritroide
Definitia anemieiDefinitia anemiei
Elementele importante in anamneza, Elementele importante in anamneza, examen obiectiv si evaluarea de laboratorexamen obiectiv si evaluarea de laborator
Clasificarea anemiilorClasificarea anemiilor
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Obiective:Obiective:
Hematopoieza / Fiziologia seriei eritroideHematopoieza / Fiziologia seriei eritroide
Definitia anemieiDefinitia anemiei
Elementele importante in anamneza, Elementele importante in anamneza, examen obiectiv si evaluarea de laboratorexamen obiectiv si evaluarea de laborator
Clasificarea anemiilorClasificarea anemiilor
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SangeleSangele
Eritrocite (RBCs)Eritrocite (RBCs) Leucocite (WBCs)Leucocite (WBCs) Trombocite ( PLT)Trombocite ( PLT) PlasmaPlasma Adult ~ 5L de sangeAdult ~ 5L de sange
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Frotiu de sange periferic – aspect normal
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Frotiu de sange periferic – aspect normal
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Celula stem multipotenta: - autoreplicare - migrare in sange periferic - diferentiere ( angajare )
CFU = Unitati Formatoare de Colonii( CS angajate )
Linia mieloida Linia limfoida
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HEMATOPOIEZA NORMALAHEMATOPOIEZA NORMALA
lipocit
lipocit
megakariocit
sinus
Vena centrala
Celule endoteliale
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rearanjament genic => diferentiereCFU = Unitati Formatoare de
Colonii( CS angajate )
Linia mieloida Linia limfoida
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HEMATOPOIEZA NORMALAHEMATOPOIEZA NORMALA
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Maturatie: - reducerea dim. nucleare - hemoglobinizarea citoplasmei
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Durata: 7-8 zile etapa de reticulocit: - 2 zile in maduva - 1 zi in sangele periferic
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Reticulocitele = indicator de erotropoieza
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Reticulocite – coloratie supravitala (fara fixare) cu albastru cresyl
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Durata de viata a reticulocitelor = 3 zileDurata de viata a reticulocitelor = 3 zile
- 2 zile in maduva- 2 zile in maduva
- 1 zi in sange- 1 zi in sange
Numar de reti. = 0,5 – 1,5 % ( 90 000 / Numar de reti. = 0,5 – 1,5 % ( 90 000 / μl )μl )
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Reticulocyte Production Index - Correction for left shift
TIMPUL DE MATURATIE CORELAT CU HEMATOCRITUL
Hematocrit (%)
45
35
25
15
2 1.0
1,5 1.5
1 2.0
0.5 2.5
Factorul de corectie
“SHIFT”
Reticulocitele
din sangele periferic
(zile)
Reticulocitele
M.O
(zile)
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Durata de viata a reticulocitelor = 3 zileDurata de viata a reticulocitelor = 3 zile
- 2 zile in maduva- 2 zile in maduva
- 1 zi in sange- 1 zi in sange
Numar de reti. = 0,5 – 1,5 % ( 90 000 / Numar de reti. = 0,5 – 1,5 % ( 90 000 / μl )μl )
TIMPUL DE MATURATIE CORELAT CU HEMATOCRITULTIMPUL DE MATURATIE CORELAT CU HEMATOCRITUL
Hematocrit (%)Hematocrit (%) maturatie ( zile)maturatie ( zile)
4545 11
3535 1,51,5
2525 22
1515 2,52,5
Indice de productie ( Ip) = % Rt x Ht real x 1 Ht ideal timp de maturatie Ip > 3 = An. Hiperregenerativa Ip < 2 = An. Hiporegenerativa
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Indice de productie ( Ip) = % Rt x Ht real x 1 Ht ideal 2
Ip > 3 = An. Hiperregenerativa Ip < 2 = An. Hiporegenerativa
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Red Blood Cells (Eritrocitele)
Productie in maduva hematogena
Durata de viata ~ 120 zile Rol – transport gaze Nu are nucleu Paloare centrala (1/3) Culoare roz (Hb Diametru 7- 8 µ - capill. 2 µ
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RBC- RBC-
HemoglobinaHemoglobina– Legare reversibila si transport 0Legare reversibila si transport 022 de la de la
plamani spre tesut plamani spre tesut – 4 lanturi de globina si fier4 lanturi de globina si fier
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RBC- distructie RBC- distructie
Hemoliza fiziologica = indepartarea eritrocitelor Hemoliza fiziologica = indepartarea eritrocitelor imbatranite (cu varsta peste 120 de zile)imbatranite (cu varsta peste 120 de zile)
Eritrocitele imbatranite :Eritrocitele imbatranite :
=> hemoliza extravasculara (intracelulara) => hemoliza extravasculara (intracelulara) indeparteaza aproximativ 90% din eritrocitele indeparteaza aproximativ 90% din eritrocitele senescente senescente
=> hemoliza intravasculara 5 – 10 %=> hemoliza intravasculara 5 – 10 %
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Formarea si distructia eritrocitelorFormarea si distructia eritrocitelor
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Metabolismul hemoglobinei intravasculareMetabolismul hemoglobinei intravasculare Hb libera
tetrameri)
Hbdimeri
haptoglobin
complexehaptoglobin-hemoglobin
rinichi ficat
metHb
Legare la Hemopexin si albumin
ferriheme (Fe3+)globin
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Obiective:Obiective:
Hematopoieza / Fiziologia seriei eritroideHematopoieza / Fiziologia seriei eritroide
Definitia anemieiDefinitia anemiei
Elementele importante in anamneza, examen Elementele importante in anamneza, examen obiectiv si evaluarea de laboratorobiectiv si evaluarea de laborator
Clasificarea anemiilorClasificarea anemiilor
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ANEMIA – definitieANEMIA – definitie
ANEMIE – scaderea cant. de sangeANEMIE – scaderea cant. de sangeFIZIOPATOLOGIE: → tulburare fundamentala: incapacitatea sangelui de a-si FIZIOPATOLOGIE: → tulburare fundamentala: incapacitatea sangelui de a-si
indeplini functia de transport a 0 indeplini functia de transport a 022
LABORATOR: - scaderea RBC, Hb, Ht, sub valoarile normaleLABORATOR: - scaderea RBC, Hb, Ht, sub valoarile normale pentru varsta si sexul respectiv pentru varsta si sexul respectiv
CLINIC: - sindrom cu simptomatologie diversa care are la baza hipoxiaCLINIC: - sindrom cu simptomatologie diversa care are la baza hipoxia
Tabloul clinic depinde de:Tabloul clinic depinde de:
- modul de producere rapid sau lent al anemiei- modul de producere rapid sau lent al anemiei
- capacitatea de compensare a mecanismelor homeostaziei- capacitatea de compensare a mecanismelor homeostaziei
- gradul anemiei- gradul anemiei
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Obiective:Obiective:
Hematopoieza / Fiziologia seriei eritroideHematopoieza / Fiziologia seriei eritroide
Definitia anemieiDefinitia anemiei
Elementele importante in anamneza, examen Elementele importante in anamneza, examen obiectiv si evaluarea de laboratorobiectiv si evaluarea de laborator
Clasificarea anemiilorClasificarea anemiilor
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ANEMIA: Etape de diagnosticANEMIA: Etape de diagnostic
- Anamneza: AHC, APF, APP, CVM
- Ex. Obiectiv
- Hemograma
- Determinare: sideremie, CTLF, feritina,
- Examenul Maduvei Osoase
- Teste speciale: dozare cobalaminemie, folatemie, test Coombs, …….
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ANEMIA: Etape de diagnostic - ANEMIA: Etape de diagnostic - anamnezaanamneza
Anamneza:
- AHC : anemie, icter, crize biliare
- APF: menstra, sarcini, avorturi, alaptare
- APP: boli cronice, pierderi de sange; malabsortie
- CVM: obiceiuri alimentare (vegetarieni, alcool), expunere profesionala ( ex. Plumb, benzen..), medicamente
!!! Vechimea anemiei
-
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ANEMIA: Etape de diagnostic ANEMIA: Etape de diagnostic - examen obiectiv- examen obiectiv
Tegumente / mucoase: paloareTegumente / mucoase: paloare Limba depapilata – glosita Icter, purpura Adenopatii Hepato-splenomegalie Astenie / Dispnee de efort Tahicardie / Insuficienta Cardiaca Sangerari
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
- Automata – analizor de hematologie (Coulter ….)
- Manuala - camera de numarat
- Determinarea reticulocitelor: analizor automat sau metoda clasica
- Examinare a la microscop a frotiului de sange periferic (Col. MGG)
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
PARAMETRII ERITROCITARI:
1. Cantitatea de Er. existente in circulatie RBC
2. Volumul Er procentual HCT
3. Hb dintr-un decilitru de sange HGB
4. Volumul Er. mediu VEM
5. Incarcarea cu Hgb a Er. HEM = hemoglobina Er medie CHEM = conc.medie a Hgb din Er
6. Productia de Er a M.O. Reticulocitele
7. Variatia dimensiunilor Er: RDW = largimea distributiei Er
-
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PARAMETRIIERITROCITARI
BARBATI FEMEI
HEMOGLOBINA g/dL 13,5 - 17,5 12,0 - 16,0
ERITROCITE x 106/μL 4,5 - 6,0 4,0 - 5,4
HEMATOCRIT (%) 40 - 52 36 - 48
RDW < 14,6% < 14,6%
PARAMETRII ERITROCITARI: Valori normale (analizor – Coulter)
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
INDICII ERITROCITARI:
VEM (MCV) = Ht% x 10 N = 80 – 94 fl
RBC (x 106/μL)
HEM (MCH) = Hb (g/dL) x 10 N = 25 – 33 pg
RBC (x 106/μL)
CHEM (MCHC) = Hb (g/dL)x 100 N = 32 – 36 g/dL
Ht
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
Analizarea frotiului de sange periferic (Col. MGG)
- Leucocite
- Trombocite
- Eritrocite
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
Analizarea frotiului de sange periferic (Col. MGG)
Leucocite:
- formula leucocitara
- modificari morfologice: - hipogranulare
- hipersegmentare nucleara
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
Analizarea frotiului de sange periferic (Col. MGG)
Trombocite: - aspect / marimeTrombocite: - aspect / marime
- grupe - grupe
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
Analizarea frotiului de sange periferic (Col. MGG)
Eritrocite : normocromie / normocitozaEritrocite : normocromie / normocitoza
Modificari de culoare (anizocromie) Modificari de culoare (anizocromie)
Modificari de marime (anizocitoza )Modificari de marime (anizocitoza )
Modificari de forma ( poikilocitoza)Modificari de forma ( poikilocitoza)
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
Analizarea frotiului de sange periferic (Col. MGG)
Eritrocite : normocromie / normocitoza
Modificari de culoare (anizocromie) Modificari de culoare (anizocromie)
- hipocromie - hipocromie
- anulocite- anulocite
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
Analizarea frotiului de sange periferic (Col. MGG)
Eritrocite : normocromie / normocitoza
Modificari de culoare (anizocromie)
Modificari de marime (anizocitoza )Modificari de marime (anizocitoza )
- microcite- microcite
- macrocite- macrocite
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA
Analizarea frotiului de sange periferic (Col. MGG)
Eritrocite : normocromie / normocitoza
Modificari de culoare (anizocromie)
Modificari de marime (anizocitoza )
Modificari de forma ( poikilocitoza)Modificari de forma ( poikilocitoza)
- microsferocit, schizocit- microsferocit, schizocit
- Er. in semn ”de tras la tinta”- Er. in semn ”de tras la tinta”
- Ovalocit , Dacrocit ( “in lacrima”)- Ovalocit , Dacrocit ( “in lacrima”)
- drepanocit- drepanocit
Incluziuni Eritrocitare:Incluziuni Eritrocitare:
- punctatii bazofile- punctatii bazofile
- corpi Jolly- corpi Jolly
- inele Cabot- inele Cabot
- Corpi Heinz- Corpi Heinz
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic
(Col. MGG)NormalNormal
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
MacrociteMacrocite
Hipersegmentarea Hipersegmentarea
granulocitaragranulocitara
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
HipocromieHipocromie
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
Punctatii bazofile:Punctatii bazofile:
- talasemie- talasemie
- intoxicatie cu Pb- intoxicatie cu Pb
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
Eliptocite / ovalocitEliptocite / ovalocitee
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
Corpi Howell Jolly = resturi Corpi Howell Jolly = resturi nucleare:body:nucleare:body:
- anemii hemolitice- anemii hemolitice
- hiposlenism- hiposlenism
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
Schizocite / Eritrocite Schizocite / Eritrocite fragmentatefragmentate
- Fragmented (mechanical - Fragmented (mechanical or phagocytosis) or phagocytosis)
- DIC- DIC
- PTT- PTT
- valve cardiace mecanice- valve cardiace mecanice
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
drepanocitedrepanocite
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
Eritroblasti:Eritroblasti:
- nou nascut- nou nascut
- hemoliza - hemoliza
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
Sferocite:Sferocite:
- AHAI- AHAI
- sferocitoza ereditara- sferocitoza ereditara
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
Eritrocite in semn de tras la Eritrocite in semn de tras la tinta: tinta:
- hemoglobinopatii- hemoglobinopatii
- talasemie- talasemie
- SMPC- SMPC
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Etape de diagnostic:Etape de diagnostic: HEMOGRAMA - frotiu de sange periferic (Col. MGG)
Eritrocite in picatura:Eritrocite in picatura:
- myelofibrosis- myelofibrosis
- talasemie- talasemie
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Etape de diagnostic:Etape de diagnostic: STUDIUL MADUVEI STUDIUL MADUVEI HEMATOGENE HEMATOGENE
RECOLTARE: RECOLTARE:
- aspiratie (medulograma) = ex. Citologic- aspiratie (medulograma) = ex. Citologic
- biopsie osteomedulara = ex. Histologic- biopsie osteomedulara = ex. Histologic
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Etape de diagnostic:Etape de diagnostic: STUDIUL MADUVEI STUDIUL MADUVEI HEMATOGENE HEMATOGENE
MEDULOGRAMA:MEDULOGRAMA:
Ex. cu obiectivul mic ( 20 / 40x)Ex. cu obiectivul mic ( 20 / 40x)
– celularitatea medularacelularitatea medulara
– nr / aspect megacariocitenr / aspect megacariocite
– prezenta celulelor straineprezenta celulelor straine
– se cauta o zona de imersiese cauta o zona de imersie
Ex. cu obiectivul mare( 100x)Ex. cu obiectivul mare( 100x)
– identificare diverselor tipuri celulareidentificare diverselor tipuri celulare
– maturatia celularamaturatia celulara
– modificari morfologice: - marime / - nucleu / - citoplasmamodificari morfologice: - marime / - nucleu / - citoplasma
- raport Granulo / Eritrocitar ( N= 3:1)- raport Granulo / Eritrocitar ( N= 3:1)
Concluzii: Concluzii:
Coloratie speciala: ex. Col. Perls pt. Hemosiderina medulara.Coloratie speciala: ex. Col. Perls pt. Hemosiderina medulara.
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STUDIUL MADUVEI HEMATOGENE:STUDIUL MADUVEI HEMATOGENE: MEDULOGRAMAMEDULOGRAMA
Ex. cu obiectivul mic ( 20 / 40x)Ex. cu obiectivul mic ( 20 / 40x)
– celularitatea medularacelularitatea medulara
– nr / aspect megacariocitenr / aspect megacariocite
– prezenta celulelor straineprezenta celulelor straine
– se cauta o zona de imersiese cauta o zona de imersie
75% din MO pentru seria granulocitara (G)
25% din MO pentru seria eritroida (E)
Raport E / G = 1:3
Grasimea – zone intinse din maduva
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Etape de diagnostic:Etape de diagnostic: STUDIUL MADUVEI STUDIUL MADUVEI HEMATOGENE HEMATOGENE
MEDULOGRAMA:MEDULOGRAMA:
Ex. cu obiectivul mic ( 20 / 40x)
Ex. cu obiectivul mare( 100x)Ex. cu obiectivul mare( 100x)
– identificare diverselor tipuri celulareidentificare diverselor tipuri celulare
– maturatia celularamaturatia celulara
– modificari morfologice: - marime / - nucleu / - citoplasmamodificari morfologice: - marime / - nucleu / - citoplasma
- raport Granulo / Eritrocitar ( N= 3:1)- raport Granulo / Eritrocitar ( N= 3:1)
Concluzii:Concluzii:
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STUDIUL MADUVEI HEMATOGENE:STUDIUL MADUVEI HEMATOGENE: maduva normala maduva normala
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Obiective:Obiective:
Hematopoieza / Fiziologia seriei eritroideHematopoieza / Fiziologia seriei eritroide
Definitia anemieiDefinitia anemiei
Elementele importante in anamneza, Elementele importante in anamneza, examen obiectiv si evaluarea de laboratorexamen obiectiv si evaluarea de laborator
Clasificarea anemiilorClasificarea anemiilor
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ANEMIE – ClasificareANEMIE – Clasificare
Clasificarea morfologica: - An. Hipocrome- MicrocitareClasificarea morfologica: - An. Hipocrome- Microcitare
- An. Normocrome- - An. Normocrome- NormocitareNormocitare
- An. Macrocitare- An. Macrocitare
Clasificarea fiziopatologica:Clasificarea fiziopatologica:– An. Hiporegenerative (cauza centrala) I.P < 2An. Hiporegenerative (cauza centrala) I.P < 2– An. Hiperregenerative ( cauza periferica) I.P >3An. Hiperregenerative ( cauza periferica) I.P >3
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ANEMIE – ClasificareANEMIE – Clasificare
Clasificarea morfologica (indicii eritrocitari): Clasificarea morfologica (indicii eritrocitari):
- An. Normocrome- Normocitare- An. Normocrome- Normocitare
- An. Hipocrome- Microcitare- An. Hipocrome- Microcitare
- An. Macrocitare- An. Macrocitare
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I. Anemie microcitara (VEM < 80 fl) hipocroma (HEM < 27 pg)
1) Anemie prin deficit de fier (Fe)2) Anemia cronica simpla (inflamatii cronice, infectii cronice, neoplasme, insuficienta renala),3) Talasemiile (α si β)4. anemii sideroblastice
II. Anemie normocitara (VEM 80 – 100 fl) normocroma
(HEM > 27 pg)
1) Sangerare recenta2) Insuficienta medulara (aplazia medulara, infiltrare
medulara cu celule anormale / fibroza…)3) Anemia Hemolitica4 ) Anemia cronica simpla (inflamatii cronice, infectii cronice, neoplasme, insuficienta renala),
III. Anemie macrocitara (VEM > 100 fl)
1) Anemiile Megaloblastice (deficit de folati / cobalamina …..)
ANEMIA - Clasificarea morfologica (indicii eritrocitari)
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ANEMIE – Clasificare fiziopatologicaANEMIE – Clasificare fiziopatologica
– Anemia Hipo-regenerative (cauza centrala) I.P < 2Anemia Hipo-regenerative (cauza centrala) I.P < 2
– Anemia Hiper-regenerative ( cauza periferica) I.P >3Anemia Hiper-regenerative ( cauza periferica) I.P >3
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ANEMIE – Clasificare fiziopatologicaANEMIE – Clasificare fiziopatologica
CAUZA CENTRALA:CAUZA CENTRALA:
– aplazia medularaaplazia medulara
– dislocare medulara (LA, LM, BH, Metastaze, MM, mielofobroza)dislocare medulara (LA, LM, BH, Metastaze, MM, mielofobroza)
– hipoproductie prin carenta de fier / biocatalizatorihipoproductie prin carenta de fier / biocatalizatori
– MielodisplazieMielodisplazie
CAUZA PERIFERICA:CAUZA PERIFERICA:
– Hemoragii acuteHemoragii acute
– An. Hemolitice – cauza intra corpusculara (ereditare)An. Hemolitice – cauza intra corpusculara (ereditare)
- cauza extra corpusculara ( auto-imuna , iso-imuna, - cauza extra corpusculara ( auto-imuna , iso-imuna, infectioasa, hipersplenism, CID, PTT)infectioasa, hipersplenism, CID, PTT)
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Ce este Anemia ?Ce este Anemia ?
Important de retinut:Important de retinut:
- anemia este semnalul clinic pentru o boala / mecanism - anemia este semnalul clinic pentru o boala / mecanism de boalade boala
- anemia ca atare nu este o singura boala - anemia ca atare nu este o singura boala
- este nevoie sa cautam cauza de baza- este nevoie sa cautam cauza de baza
- diagnosticul anemie nu este simplu !! - diagnosticul anemie nu este simplu !!
- anemia este foarte importanta si frecventa in practica - anemia este foarte importanta si frecventa in practica medicalamedicala
- tratamentul depinde de cauza- tratamentul depinde de cauza