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ANEMII
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Defnitie:
Reducerea semnifcativa a masei de hematii,cu scaderea consecutiva a capacitatii sangelde a transporta de oxigen
Valorile normale ale Hb:
- barbati: > 13 g dl !Htc > 3"#$
- %emei: > 1& gdl !Htc >3'#$
- nou-nascut: 1"gdl (- )g
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*+./.*R0 02..+R:
14 *+..*:• .n %unctie de rapiditatea instalarii
acute cronice
• .n %unctie de severitate
usoare - Hb 5 11-6gdl, Htc 5 3"-37#,
medii - Hb 5 6-8 gdl, Htc 37-&,
severe - Hb 9 8gdl, Htc 9 &7#
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&4 0.;
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4 Defcienta substprecursorilor necesari
eritropoie=ei:
.prod4 De Hb !globina, sinte=a H02, /e$
defcite en=imatice ( defcit de vitamina 8
defcit de substante ce conditionea=amaturarea normala a hematiilor: vit 1&,acid %olic
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4 dr de defcienta medulara:• cuparea spatiului medular
!+e,+i$, metasta=e medulare, infltratii
lipidice !
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*4 dr de insufcienta medulara:
Defcit de %actori necesari si maturarii
seriei 0- defcit de eritropoietina
-defcite hormonale ! tiroida, testosteron,
glucagon$ ce repre=inta %actori anaboli=antinecesari sinte=ei 0
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D4 oli cronice:
- in%ectii cronice
- in?amatii cronice din colageno=e
- neopla=ii
- a%ectiuni renale si hepatice cronice
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0R.R;.0@:
*elula tem 2ultipotenta
/A
*/A
;roeritroblast eritroblast ba=ofl 0bl4;olicromatofl
0bl oxifl reticulocit 0ritrocit matur
citoBine
eritropoietina
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inte=a hemoglobinei:
uccinil *o ( glicina
D+
por%obilinogen
uroporfrinogen ...
coproporfrinigen ...
protoporfrina .C
(/e H02
H02 ( globina 5 H02
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*.R*A.A+ /.0RA+A. . R
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.V0.
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;R0*.0R0 D0;@.0+R D0 /.0R
14 Determinarea hemosiderinei medulare!1(-3($
&4 Do=area %eritinei circulante - R.!8"µgmlbarbati, 3µgdl %emei$
34 estul de absorbtie intestinala a /e
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02. /0R.;R.V
Defnitie: nemie hipocroma in care defcitulde sinte=a al Hb are drept cau=a scadereacantitatii totale a /e din organism
.ncidenta:
De%4 De /e 5 cea mai raspandita cau=a deanemie din lume
2: a%ectea=a 37# din populatia globului
)3# dintre prescolari
3'# dintre scolari
1 # %emei gravide
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D..+0 D0/.*.A+A. D0 /.0R
stadiul Depozite
De fier
Fe
plasmatic
Anemie Hipocromie
microcitoza
normal normale normal absenta absente
Deficienta
Pre-latenta
reduse normal absenta Absente
abs Fe
Deficienta
latenta
absente redus absenta Absente
PLE
A.feripriva
St. initial
absente redus Usoara/
medie
in unele E
indici E ! "
A. feripriva
St. avansat
absente reduse severa in #$$% E
&E'
(
)HE'
'odif.epit.
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*au=ele defcitului de fer
14 ;ierderi de sange: digestive, genitale,pulmonare, urinare
1# - %ara cau=a decelabila clinic, Rx,endoscopic
teleangiecta=ii
para=ito=e intestinale
sdr hemoragipare !sangerari pe mucoase$
&4 port sca=ut: vegetarieni, sugari hraniticu lapte de capra
34 dr4 de malabsorbtie - aclorhidrie, re=ectiigastrice, EeEunite
F
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2ani%estari clinice ale defcitului de fer
14 Defcit asimptomatic de /e !stocaE,erotropoie=a$
dg4 De laborator
&4 Diagnostic clinic - apare sdr4 nemic! Hb96gdl$
oboseala, palpitatii, dispnee
dis%agie, le=4 le cavitatii bucale!stomatita angulara, atrofa papilelorlingule, o=ena, modi% unghii-%riabile,lenticulare$
pervertirea gustului
17# splenomegalie
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i i l d l b
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Diagnosticul de laborator
14 /rotiu de sange
initial indici 0 normali, ulteriorani=ocito=a, poiBilocito=a, hipocromie
&4 ange peri%eric
Hb
, re=istenta osmotica
, reticulocite5
ideremia: 97µgdl
rans%erina pl - *+/
!n5&7-)77 µgdl$aturatia trans%erinei: 9 1#
/eritina circulanta: 917ngdl !5 1&-3&
ngdl$
Di i l d l b
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Diagnosticul de laborator
34 0xaminarea maduvei osoase:
Hiperpla=ia seriei rosii
0ritroblasti %eriprivi grupati in insule de -17 elem4 *u citoplasma caracteristica cuprelungiri !0 cu %ranEuri$
*oloratie ;earls - absenta granulelor de/e din macro%agele medulare
Di ti l di% ti l
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Diagnosticul di%erential
14 nemiile din bolile cronice: inf. cronice:TBC, abcese pulmonare, pielonefrite cronice,
colagenoze, cancere (san, plaman)
2ecanism:
R4 in?am .+1 hiperactivitatea 2a ce
captea=a /e din hematii si il stochea=a sideremia - pseudo%eriprivie
A feripriva A.
boli croniceµcito=a(hcromie, Ret µcito=a(hcromie, Ret
*+/ , %eritina *+/ ,
t t
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ratament
14 *orectia cau=ei care a generat anemia
&4 &4 ratamentul martialdministrare orala de /e do=a 5177 mg/e=i ad &77mg in anemii severe
( vitamina *imp de administrare 5 pana la corectiaanemiei ( 8 luni dupa pt4 re%acereadepo=itelor
*ri=a reticulocitara apare la 17 =ile
F ;rudenta in administrare - reactiianaflactice severe
02.. .D0R+.*0
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02.. .D0R+.*0
Defnitie:
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02.. 20
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- alte etiologii: alcoolism, htiroidism, hepatopatiicronice, sarcina, %enitoina
Anemii prin decit de B12
- necesar 1& 5 1-3 µg=i, absorbit in 1& distalaa . sub actiunea /. *astle- 1& legat plasmatic de transcobalamine(I,II,III)- depo=itare in fcat - 3- mg
- pierderi: A, , , ex%oliere - 7,7-7,1#=i & anidefcit pana la ap4tulb4
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*A@0+0 D0/.*.A+A. D0 1&:14 port insufcient - egetarieni&4 ecretie inadecvata de /.*- A. Biermer ! atc anti!"IC#cel parietala gastrica
! $e%cit genetic $e sinteza a "IC- rezectia gastrica34 *onsum G de 1&: supracrestere bacteriana !&ip'lobotrium latum
)4 2alabsorbtie: rezectii ileale, C'ron,malabsorbtie selectia B* ! IMME+-N&
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Anemia Biermer
- secretie /.* nein?4 de H.- asociata cu : D@ ,
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+aborator:- Hb , V02 G, +e, r cuani=ocito=a si r gigante
- 2o hiperpla=ica 0>>
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02. 20
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ratament:- 1& 177µg=i & sapt, apoi 177µg=i
de &xsapt )-8 sapt, apoi177µgluna toata viata- In formele c neropatie ! 1777µg
la &-3 =ile cateva sapt, ulterior177µg=i de &-3xsapt4 8-1& luni- hidroxicobalamina 77 µg la 8-6
sapt4- acid %olic concomitent cu 1&- fer- trans%u=ii 20 -anemie severa
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nemii megaloblastice prin defcit deac4 /olic
- rolul / - in metabolismul ac4 nucleici!sub %orma de coen=ima /H)$- nu se sint4 in organism
- aport alimentar - poliglutamati - 877µg=i, necesarul 5 7 µg=i, abs4 .ndd(EeEun ca monoglutamati depo=it in
fcat- transport plasmatic liberlegat deglobuline- pierderi: piele, ,A,saliva
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*au=ele defcitului de acid %olic:
14 port varstnici,alcoolici,malnutritie, sugari, re=ectii intestinale,&4 ulburari de absorbtie: sprue,celiachie
34 evoi G: sarcina, lactatia)4 ;ierderi G: psoria=is
ratament: acid %olic mg=i p4o4
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02.. H02+..*0
haima"san#e$ lsis"distr#ere! durata de viata a eritrocitelor 91-&7 =ile
- hemoli=a f=iologica - predominentin 2 > 1&7 =ile
- hemoli=a patologica: extravasculara: fcat, splina
intravasculara
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H02+.@ 0CRV*A+R:
%&,%AHematii----------i. ( albumina plasm4
.-----------/icat !conEugare$
. 17-&7# . iD
.
------------A< urina stercobilinogen
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H02+.@ .RV*A+R:2o,2a
H ( hapto#lobina
H libera H02 oxidare
Hematina ( hemope'ina ( albmina 2etHb
Dimeri Hb hemoglobinurie depunere renala hemosiderina
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*lasifcarea H
.4 A intracorpsclare
- de%ecte de membrana: s%erocito=a,
eliptocito=a, H;- en=imopatii: defcit de ;J,
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&4 A e'tracorpsclare- prin atc: H.
- prin ag4 in%ectiosi- prin medicamente- prin ag4 chimici: ;b, *u, @n, s
- prin ag4 f=ici: arsuri intinse,radiatii- prin %actori traumatici: prote=e,*.D, sdr4hemolitic-uremic,; hemolitica, hemoglobinuria demars- hipersplenism
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Diagnosticul H:
*+..*:
- simpt4 nespecifca
- ex4 obiectiv: paloare,sbicter icter , de%ormari osoase,splenomegalie, litia=a biliara- crize aplastice ! zile :Re, 0bldeclansate de in%4 Viralebact4 dgdi%erential cu +FFF
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;R*+..*:
- Anemie* Hb, Htc4 - emolitica* Ret , i.,iD ,
Haptoglobina , hemopexina ,
stercobilinogen %ecal(((- semne indirecte* +e , r , 2
hiperpla=ica, H2
- hipercatabolism eritrocitar* +DH,
/e, /e in R0 din 2
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/0R*.@ 0R0D.R
Defcit de anchirina *ontinutul de spectrina din mb4 eritrocitara si Vtransmitere D in '# ca=uri, &# R
- *linic: paloare, icter, 2, ulcere cr4 alegambelor, litia=a biliara-+aborator: anemie moderatanormocroma,reticulocito=a 6-&# cri=e aplastice,
/e moderat - %rotiu: µs%erocite &-"7#, policromatoflie, corpiHoKel-LollM
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- 2ielograma:
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*omplicatii:
- colecistita calcloasa, angiocolita- crizele de de#lobnizare: J, ce%alee,%en dispeptice, %risoane biologic:i,
2((, hiperpla=ie medulara- crizele aplastice: rau, %ebra )7,colaps, coma, Hb,Htc disparitia 0bl,
i si icterul - revin in 17 =ile -hiperreticulocito=a, hiperpla=ia serieirosii sunt produse de parvovirus 1"- +rizele me#aloblastice* aport %olati
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V+*.@- transmitere D- defcit de spectrina si prot )41
D0/.*.A+ D0
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/orme clinice:
14 0pisoade acute de hemoli=a&4 .cter neo-natal34 H congenitala nes%erocitara
)4 /avismul
declansate de: medicamente* antimalarice, sul%amide,
analge=ice, aspirina, %enacetina virsri : gripal,HV vicia fava
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ratament:
- s%erocito=a,ovalocito=a- plenectomia:%orme severe de H
splina mare cu %enomenecompresive
in%arcte splenice multiple
Defcit de
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Hemoglobinuria paroxistica nocturna 0tiopatogenie:- boala clonala ce a%ectea=a cel4 tem din2 -gena ce codifca glico=il-%os%oino=itolul din structmb N vulnerabilitate la li=a de catrecomplementul activat N hemoli=a
intravasculara continua
*linic:- debut insidios cu paloare, icter, 2, Hm
peste care se suprapun episoadele deH; ! in %orme grave: %rison %ebra,mialgii, lombalgii, colaps, anurie$
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+aborator:
- pancitopenie +e, r in 87#ca=uri in special la cei cu 2((- /+ 5 7
- 2: hipercelulara, hemosiderinaabs4- plasma: i., Hb libera- urina hipercroma-teste specifce:
Ham - plasma pH 8,-',7 sucro=aapa-=ahar
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*omplicatii:
- in%ectii- cri=e aplastice
- %en4 trombotice !venoase$ Accidente minore* cri=e ce%alalgice,dureri lombare
Accidente maore* terit cerebral,brahial, portal, splenic, suprahep,crural, art 2 si renala
ratament
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ratament- trans%u=ii cu eritrocite spalate- androgeni: /luoximesteron - scad
nevoia de trans%u=ii- %eroterapia - F ;oate declansacri=e hemolitice
- corticoi=i - la *oombs(rebeli latrat- hidratare per os Dextran- anticoagulare- antiin%ectios- splenectomie daca 2"((sihipersplenism
- llo 2
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H02
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.*+02. - anomalii in lant O-pre=enta Hb care in stare redusa polimerice de%ormea=a hematia sludge
*linic:- Ht=ig 5 asimptomaticdoar in cond4 de
hipoxie ((- homo=ig4 H grava cu debut in . an de viatapaloare, astenie, defcit staturo-ponderal
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4sdr hemolitic: icter, 2, insu%4
cardiaca, litia=a biliara,osteoporo=a, craniu in perie, sdrmana-picior
4rombo=ele vasculare:- in%arcte osoase: . copilarie- in%arctul pulmonar: adult- cri=ele abdominale- tulburari neurologice- mani%estari renale
- ulcere cronice maleolare
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+aborator:-anemie, reticulocito=a
-%rotiu: hematii in tinta, corpi LollM,drepanociteest de sicli=are 5 po=itiv
0lectro%ore=a Hb 5 Hb anormala
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*omplicatii:- cri=e hemolitice - uneori mortale
- in%ectii intercurente- compl4 soase: %racturinecro=easeptice- trombo=e *- colecistita cr, cri=e aplastice,hemosidero=a
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ratament:
- simptomatic: trans%u=ie 20 la Hb9'- cri=e vasooclu=ive: .- exanguinotrans%u=ie in cri=elevasooclu=ive grave
- ag4antisicli=anti: *-ra HA- incal=ire, hidratare, alcalini=are,analge=ie,
- ransplant de 2 - mortalitate:17-7#
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Diagnostic prenatal:
- 16-&7: anali=a reticulocitelor- D din amniocitele choriale: "-11
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+02..
+02..
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+02..
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+02..
ransmitere auto=omal codominant
γ γ δ
------ ---- ----------- ------ ---- ----------- cr418 cr411
dult: Hb 1 5 & & Hb & 5 & &δ Hb / 5 & &γ
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alasemii
(5 exista lanturi dar - 5 lanturi absente
Hb precipita
distrugerea 0bl in2o hematii defciente
hemoli=a splenica anemie 0po hematopoie=a
2(extra2
/!TA-AEMII
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/ TA-AEMIIma*ora interm minora 'in
Ef Hb F e+ces F ,-#% F #-% F #%
S' /$ $
cter /$ $
0s /$ /$ $
Hb 1 -#$ 2#$ #-#3
Hipocr
H tinta /-
4et /-
"bl. /$ /$ $
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talasemii - homo=igoti
14 2aEora-anemia *ooleM- anemie severa > & sapt
- Hb
, H2
- modifcari scheletale- hipersplenism- /e - hemosidero=a, *+/, sat trans%erinei , Hb/ >7#
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talasemii - homo=igoti
&4 talasemia intermedia- anemie moderata dupa 1-& ani- exces de + in 0bl - distructie
medulara(splenica-modifcari scheletale- splenomegalie
- hemosidero=a- necesita trans%u=ii in ca= dein%ectii
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talasemii - hetero=igoti
talasemia minora(minima
- asimptomatici- Hb usor in minora si in minima- Hb &: 3,-'# Hb /: 1-#- sideremia, %eritina, *+/ 5
- %rotiu: µcite, H in tinta,0(granulatii ba=ofle
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ratament:
- 20 pt mentinerea Htc>37#
- Des%eral- llo2 - la pac cu compl4evere- H-uree - stim4 int de +γ- terapie genica pt gena +
+/ +02..
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+/-+02..
/
Hbno=a
minora minima H
l i i !& $
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alasemia minora !& gene$:- homo=ig (- hetero=igot 7
- %ara anemie sau hemoli=asemnifcativa Hemoglobino=a H !3 gene$
- +
, +
tetrameri )5corpiHein=- tablou clinic variabil, Hb5'-11gdl,2 Hidrops %etal cu Hb art- homo=igot 7 γ γ)- incompatibila cu viata FFF
* li ii
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*omplicatii:
- colecistita calcloasa, angiocolita- crizele de de#lobnizare: J,ce%alee, %en dispeptice, %risoane
biologic:
i, 2((, hiperpla=iemedulara- crizele aplastice: rau, %ebra )7,colaps, coma, Hb,Htc disparitia0bl, i si icterul - revin in 17 =ile -hiperreticulocito=a, hiperpla=iaseriei rosii sunt produse de
parvovirus 1"
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.n H., dt viata a H estemediata imun prin autoatc !.g
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H. cu atc la cald
- &3 idiopatica- 13 sec:
b autoimune:+0,;R, ;.,
miastenia g, hepatita imuna b maligne:+H, +H, ++*, H*+ tu solide: teratoame ov4 post medicamentoase
- temperatura optima la care are lochemoli=a 5 3' grade *
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- tc 5 I#- cu tropism pt4 tg Rh,nefxatori de complement- varsta 5)7 ani, %b5141- hemoli=a extravasculara
*linic - variabil: hemoli=a minima adhemoli=a acuta severa- paloare, icter, 2, urini
hipercrome- H. ( r-penie imuna5sdr40vans
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+aborator:
- anemie croma, citara, Re,s%erocite, ani=ocito=a, 0bl peri%erici- %ragilitate osmotica a 0
- 2: hiperpla=ia seriei rosii (-semne de hematopoie=a inefcienta- uneori cri=e aplastice !legarea atcde mb 0bl$
- i. , +DH , /e n - urina: A
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.munologic:- test *oombs direct ( pt .g<- test *oombs indirect (- det atg: Rh: *c, D si /eratament:14bstittiv* 20 in hemoli=e masive
&4+orticoterapia* ;rednison 1mgBg -re=utate in 7# ca=uri34 plenectomia* raspuns in 67# c)4 *iclo%os%amida, a=athioprina - nu ca .
linie alcaloi=i de Vinca4 ;lasma%ere=a84 .g .v 1- gBg timp de =ile
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H. cu atc la rece:
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- idiopatice- secundare: +H, 2P, b4in%ectioase- mediate de atc4 .g2 monoclonali
sau policlonali!aglutinine la rece$care se leaga de struct4 specifce depe supra%4 0 la temp49 37-3& grade
*4 tc fxea=a ulterior *o li=aintravasculara- exc Hb-uria parox4 +a rece -.g%
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- varsta: 7-67 ani b>% - atg 5 .i din mb H
- .diopatica 5 HR - b cronica cuhemoli=a continua ( %en RaMnaud,acrociano=a- in %ormele in%4 - hemoli=a acuta
intensa autolimitata+aborator:- normocr, reticulocito=a- auto-aglutinarea H dupa recoltare siracire, reversibila la incal=irea sangelui- .g 2 monoclonal
.munologie
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g - test *oombs direct cu ser anti-complement 5 (, cu ser anti .g< 5 -- titrul agluininelor la rece!13&$- atg eritrocitari: atg ., iratament:
- evitarea expunerii la %rig- prod atc: agenti alchilanti
! ciclo%os%amida,
clorambucilul$, ./- plasma%ere=a- inefcient: corticoterapie,splenectomie
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Hemoglobinuria paroxistica la rece:
- atc4 .g< cu specifcitate pt atg ; dinmb 0- atc ! Donnath-+andsteiner$ leaga
*1,*),*& la temp4 sca=ute!91 grd$,dar hemoli=a apare doar la 3& grd- episoade acute de H intravasculara
cu Hb-urie la expunerea la %rig- t *oombs ( cu ser anti .g< la temp si cu ser anti-*o la temp4
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- incompatibilitate de grup mama-copil:i=oatc %ormati de mama strabatbariera placentara si produc hemoli=ala %at
- cea mai %recv imuni=are - Rh- H, Hpla=ie 0bl in 2, /,, icter cui., convulsii in . &)-)6 ore de viata
- t *oombs direct (- *omplicatia cea mai grava:ence%alopatia bilirubinica !icterulnuclear$
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ratament:
- proflaxia icterului hemolitic - .gspecifce anti-D dupa primul contact
- Hb913, gl sau i. > &7mg#exanguinotrans%u=ia care:inloc. an#ele /h0 al copilli c
/h!
previne icterl ncl prin scadereaBi2 m#3dl
H 20*.*0:
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H 20*.*0:14 Hb-uria de mars - de ob4 /ara
anemie&4 H mecanica asociata cu anomaliiale inimii si vaselor:
- le= valvulare cord stgprote=e34 H µangiopatice asociate unora%ectiuni caracteri=ate prin depunerede trombi in µcirculatie
*.D ; hemangoame cavernoase