Curs Anemii
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![Page 1: Curs Anemii](https://reader033.fdocuments.net/reader033/viewer/2022061511/5571fb5b497959916994a689/html5/thumbnails/1.jpg)
Semiologia sangelui si Semiologia sangelui si organelororganelor hematopoietice hematopoietice
1.Sd.anemice
2.Sd.leucemiilor acute
3.Sd.leucemiilor cronice
4.Sd.Mieloproliferative
5.Limfoamele
6. Gamapatiile monoclonale
7.Sd. hemoragipare (patologia hemostazei)
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Functiile sangelulFunctiile sangelul
a, Transport
-Oxigen
-Subst. Nutritive
-Produsi de catabolism
-Hormoni
b. Reglare hidroelectrolitica si acidobazica
c. Aparare nespecifica sau specifica
d. Functia de coagulare
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Elementele cheieElementele cheie
Maduva osoasa
Splina
Ganglionii limfatici
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Sindroamele anemiceSindroamele anemice
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Definitia anemieiDefinitia anemiei
Incapacitatea sangelui de a furniza un nivel adecvat de oxigen tesuturilor pentru realizarea unui metabolism normal
HGB<13.5 g/dL (Barbati) <12 (Femei)
HCT<41% (Barbati) <36 (Femei)
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Maduva osoasaMaduva osoasa
Celula stem pluripotenta
Mecanismele de reglare:
-Citokine
-EGF
-Eritropoietina
Eritrocite – 120Z
Reticulocite – 4 Z
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Factorii principaliFactorii principali
1. Hemoglobina
2. Fierul
3. Transferina
4. Feritina
5. Vitamina B12
6. Ac.folic
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Hemoglobina
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Componentele HbComponentele Hb
Hb – F
Hb – A (2 alfa – 2 beta)
Hb – A1c (glicosilata) 3-6%
Fe+++
Hemul (Fe-Protoporfirina)
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Metabolismul fieruluiMetabolismul fierului
Fe:- Hemoglobina 2200mg- Feritina si Hemosiderina 1000mg- Mioglobina 300mg- Citocromi (enzime) 100mg
TOTAL 3600mg
Aport zilnic: 15mgAbsorbtie: 1mg.
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Metabolismul fierului IIMetabolismul fierului IIStomac
Fe2+ (feros)HCl
Fe3+++ (feric)
Duoden – Jejun Absorbtie
FeritinaCelule intestinaleTransferina (apoferitina)
DepozitDepozit
FeritinaFeritina HemosiderinaHemosiderinaR I
HematopoiezaHematopoieza HEMHEM
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Metabolismul vitaminei B12Metabolismul vitaminei B12
Stomac: Factor Intrinsec + Vit. B12
Ileonul terminal
Transcobalamina (protein – binding - globulin)
Ficat (depozit – 5 ani)
Maduva osoasa Hematopoieza
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Metabolismul ac folicMetabolismul ac folic
Alimente Duoden si Jejun
Hematopoieza
Vitamina B12:1. Sinteza metioninei;2. Metabolismul acetil CoA;3. Conversia ribonucleotidelor in dezoxiribonucleotide;
Ac. Folic:-Sinteza acizilor nucleici.
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Fe
Vitamina B12
Acidul folic
HEM
ANEMIE
Aminoacizi
Hemoragii
Aplazie medulara
Distructie intravasculara
A. AutoimuneB. Microangiopatice
Distructie splenica
HemoglobinopatiiA. Corpusculare
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Clasificarea anemiilorClasificarea anemiilor (I) (I)
1. Clinica (pe grade)-Usoara-Medie-Severa
2. Clinica dupa debut-Acuta (post hemoragica)-Cronica
3. Morfologica (dimensiuni, cromie)4. Eritrokinetica (regenerativa – non – regenerativa5. Etiopatogenica
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Clasificarea anemiilorClasificarea anemiilor (II) (II)Dupa severitate
a. Cu pierdere rapida de sange:
20% - Tahicardie
30 - 40% - Soc
50% - Moarte iminenta
b. Cronica
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Clasificarea dupa VEMClasificarea dupa VEM
Microcitara
Normocitara
Macrocitara
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Dupa CHEMDupa CHEM
Hipocroma
Normocroma
Hipercroma
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Dupa capacitatea de Dupa capacitatea de regenerare a MOregenerare a MO
Regenerativa
Aregenerativa
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Clasificarea etiologicaClasificarea etiologica
a. a. Anemii prin pierderi de sange
b. b. Anemii prin distructie eritrocitaraIntrinseca
Extrinseca
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Diagnosticul anemiilorDiagnosticul anemiilor
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EV
ALU
AR
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EM
NE S
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IMP
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SIN
DR
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ME
Simptome generale
a. Neuropsihice
- Cefalee
- Ameteli
- Astenie
- Irascibilitate
- Somnolenta
- Tulburari de vedere (ceata)
- Ataxie (Biermer)
- Senzatie de frig
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EV
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Simptome generale
b. Digestive
- Anorexie
-Greata
-Dureri abdominale:
-Intoxicatie cu Pb
-Crize de deglobulinizare
-Purpura Henoch
-Disfagie (sideropenica)
-Glosodinie
-Tulburari de tranzit (Biermer, a. feriprive)
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Paloare
Tegumente uscate
Par friabil
Cheilita
Glosita.....
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EV
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c. Cardiovasculare:
- Palpitatii
- Dispnee
- Dureri precordiale
d. Sindrom hemoragic
- Petesii
- Echimoze
- Purpura
- Hematoame
- Hemartroza (hemofilie)
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EV
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e. Dureri osoase
- Mielom multiplu
- Leucemii acute si cronice
f. Prurit
- B. Hodgkin
- Leucemii cronice
g. Febra
- B. Hodgkin (ondulanta)
- Agranulocitoza
- Leucemii acute
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EV
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Semne – examen obiectiv
1. Faciesul si aspectul somatic
- “Faciesul mongoloid” – Talasemia major (b. Cooley)
- “Craniul in turn” si frunte olimpiana – Sferocitoza
- “Omul rosu” - Leucemia cronica limfatica
- Infiltrarea glandelor lacrimale
- Faciesul leonin
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EV
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Semne – examen obiectiv2. Tegumentele si mucoasele
a. Paloarea cu diverse nuante:-”Ca varul”-”Ca ceara”-”Café au lait”
b. Subicterul p- anemia Biermerc. Icterul – anemii hemoliticed. Roseata
-Leucemiile limfatice cr.-Poliglobuliile
e. Purpura (Trombocitopenii etc)f. Teleangiectazia (Rendu Osler)g. Leucemidele (brune, viololacee, dureroase)h. Fanerele (coilonichie)
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EV
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Semne – examen obiectiv
3. Cavitatea bucala:
- Glosita Hunter (Biermer)
- Gingivita hemoragica (agranulocitoza)
- Gingivita hipertrofica (leucemii)
- Gingivoragii (diateze hemoragice)
4. Sd. ganglionar
- B. Hodgkin
- Limfoamele non – hodkiniene
- LLC
- Leucemiile acute
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EV
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Semne – examen obiectiv
5. Splenomegalia
- Policitemia esentiala
- Leucemia granulocitara cronica
- Metaplazia mieloida
- PTI
- Anemiile hemolitice
N. B. Hipersplenismul (ciroze)
- Distructie splenica
- Inhibitia citodiabazei
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EV
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Semne – examen obiectiv
6. Alte aparate
a. Pulmonar
-Dispnee inspiratorie (adenopatie mediastinala)
-Hemoptizie in diatezele hemoragice
b. Cardiovascular
-Tahicardie ( anemii)
-Sufluri sistolice pluriorificiale (anemii)
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Investigatii complementareInvestigatii complementare1. Hemograma
a. Numaratoarea
eritrocitelor:
- 4-5milioane/mmc
- Crestere: poliglobulii
- Scadere: anemii b. Dozarea hemoglobinei - Valoare normala 13 – 15 g%
- Patologic la fel
c. Hematocritul (volumul eritrocitelor la 100/ ml/ sange
- Normal:
-B=45
-F=40
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Investigatii complementareInvestigatii complementare2. Constantele eritrocitare
a. Valoarea globulara (indicele de culoare)
-continutul de hemoglobina
-Normal: 0.9-1.1
-Anemii normocrome
-Anemii hemolitice
-Boli cronice
-Hipercrome
-Biermer
-Hipocrome
-Feriprive
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Investigatii complementareInvestigatii complementare
3. Constantele eritrocitare
b. Volumul eritrocitar mediu (VEM)
-Normal : 80 – 95µ³
-Anemii normocrome normocitare (cronice
simple)
-Anemii hipocrome microcitare (feriprive)
-Anemii hipercrome macrocitare (ac folic,
megaloblastice)
c. Concentratia medie a hemoglobinei (CHEM)
d. Hemoglobina eritrocitara medie (27 -31 µg)
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Investigatii complementareInvestigatii complementare
5. Examenul frotiului
a. Modificarile de forma:
- Normal : disc
- Poilokitoza (modificate)
- Ovalocitoza
- Secera (drepanocitoza)
- Schizocite (fragmente)
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Investigatii complementareInvestigatii complementare
5. Examenul frotiului
b. Modificarile de volum (anizocitoza):
-Microcitoza
-Macrocitoza
-Megalocitoza
-Normocitoza
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Investigatii complementareInvestigatii complementare
5. Examenul frotiului
c. Modificarile de culoare (anizocromie):
-Anulocite (anemie feripriva)
-In “tras la tinta” – talasemie (palarie
mexicana)
-Hipercromie ( megaloblastica)
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Eritrocite in semn de ~tras la tinta~
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Investigatii complementareInvestigatii complementare
5. Examenul frotiului
d. Alte modificari
- Corpusculii Heinz ( granulatii mici) –
hemoglobinopatii
- Granulatii bazofile – Pb
-Resturi nucleare (Howell – Jolly, inele Cabot):
-Anemii severe
- Dupa splenectomii
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Corpii Heinz
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Corpi Howell Jolly
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Investigatii complementareInvestigatii complementare5. Examenul frotiului
e. Numarul de reticulocite
-Normal : 0.5-2%
-Crestere: anemie regenerativa
-Scade: aplazii
6. Altele : VSH
-Normal: 7-10/1h – 8 – 15/2h
-Creste:
-Infectii
-Neoplazii
-Scade in poliglobulii
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Investigatii complementareInvestigatii complementare6. Altele
a. Rezistenta globulara (rezistenta osmotica) fata de
actiunea
hemolizanta a solutiilor hipertone (NaCl):
-Hemoliza este totala la 3.4gNaCl%o
-Anemii hemolitice (sferocitoza)
b. T. Coombs – anticorpii anti eritrocitari – anemii
autoimune
c. Durata medie de viata – anemii hemolitice (hematii
marcate)
d. Sideremia:
-Scade in anemii feriprive
-Creste in hemoliza
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Investigatii complementareInvestigatii complementare6. Altele
e. Dozarea bilirubinei
f. Teste enzimatice
g. Teste citochimice
h. Testul Schilling
I. Anaclorhidia histaminorezitenta
j. Endoscopia
k. Biopsia gastrica
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Abordarea practica a unui bolnav Abordarea practica a unui bolnav cu anemiecu anemie
Anemie
Anamneza – examen clinic
Posthemoragica
Exteriorizata Nexteriorizata
HematemezaMelenaHemoptizieEpistaxisMetroragieHematurie etc.
Hemoperitoneu
Sarcina ectopica rupta
Cronica
Pasul I
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Abordarea practica a unui bolnav Abordarea practica a unui bolnav cu anemiecu anemie
Frotiul
Microcitara Macro - megalocitaraNormocitara
Pasul II
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Pasul III aMicrocitara
Microcitara hipercromica
Sferocitoza
Microcitara hipocromica
Sideremia Fe
Scazut Crescut
PlumbSideroblasticeHINB6
Aport saczutAbsorptie scazuta (M)Cresterea necesitatilor (S)Pierderi
-GI (oculte)-Menoragii
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Pasul III bNormocitara
Scazute
Productie medulara deficitara
Reticulocite
Examenul maduvei osoase
Anemie aplastica
Maduva normala Boli cronice-TBC-PCE
Neoplazii
Crescute
Anemii hemolitice
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Pasul III bMacrocitara
B 12 scazut
V. B12 metabolism
Dozarea Viaminei B12/ Ac folic
Ac folic scazute
v. A. folic (metabolism)
SarcinaCopiiAlcoolMalabsorbtieCitostatice
T. Schiling……
MalabsorptieGastrite atroficeGastrectomii etc
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Cazul 1Cazul 1
Bolnava in varsta de 72 de ani prezinta de 4 luni slabire ponderala si scaune diareice fara rectoragii. In ultima luna acuza ameteli si dispnee de efort. Tegumente si mucoase palide la examenul fizic general ?
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Cazul 2Cazul 2
Bolnava supraponderala acuza de mai multi arsuri retrosternale. Apetitul este pastrat dar la investigatiile uzuale se constata prezenta unei Hb de 8g%. Nu mai prezinta alta simptomatologie.
?
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Cazul 3Cazul 3
Bolnav in varsta de 69 de ani operat pentru cancer de prostata in urma cu un an. Acuza dureri osoase localizate la nivelul bazinului si coloanei vertebrale si poliadenpatii. Mai acuza palpitatii, ameteli si dispnee de efort. Exaemenele de laborator uzuale pun in evidenta o Hb. De 6g/% cu absenta reticulocitelor pe frotiu.
?
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Anemiile hemoliticeAnemiile hemolitice
a. Intracorpusculare Tulb. Membranare Tulb.metabolice Hemoglobinopatii
b. Extracorpusculare Imune Non - imune
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TalasemiaTalasemia
Afectiune genetica caracterizata printr-un defect de sinteza al lanturilor globinice din Hb
Se clasifica in functie de lantul globinic afectat – beta sau alfa talasemia
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TalasemiaTalasemia
Dezechilibrul de sinteza al lanturilor globinice are urmatoarele consecinte
- reducerea sintezei de Hb
- eritropoieza ineficienta
- hemoliza
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Beta talasemiaBeta talasemia
Talasemia majora sau Anemia Cooley - anemie severa - splenomegalie - deformari osoase - insuficienta cardiaca in prima decada de
viata electroforeza Hb - 90% Hb F
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Defecte membranareDefecte membranare
Sferocitoza
Stomatocitoza (Defect de permeabilitate M)
Cresterea sensibilitatii la complement (HPN)
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Talasemia minoraTalasemia minora
Beta talasemia minora
- cresterea compensatorie in sinteza lanturilor non beta
- anemie usoara
- microcitoza
- sideremie – normala
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DrepanocitozaDrepanocitoza
Hemoglobinopatie caracterizata printr-o Hb anormala : Hb S
Anemie hemolitica
Ocluzia microcirculatiei
Autosplenectomie
Poikilocitoza
Anizocitoza
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SferocitozaSferocitoza
Anemie hemolitica ereditara , datorata unui defect al membranei eritrocitare
Crize hemolitice – dureri abdominale
Eritrocite cu fragilitate osmotica crescuta
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Anemiile autoimuneAnemiile autoimune
Medicamentoase
Postransfuzionale
Cu anticorpi la rece
Cu anticorpi la cald
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MedicamenteMedicamente
Izoniazida
Chinidina
Penicilina
Metildopa
Procainamida