Download - Curs 26 - Diateze Hemoragice
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Diateze hemoragice
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Sdr hemoragice
Def= stari patologice caract prin sangerari - spontane - provocate
• Clasificare:1. Sd purpurice
- Deficitul homeostaziei provizorii la care participa patul vascular, endoteliul vascular si trombocitul
- sdr purpurice de cauza trombocitara - sdr purpurice de cauza vasculara
ca expresie a alterarii procesului normal al homeostaziei
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1. Sd purpurice:
Expresivitate clinica:- tabl clinic suficient de sugestiv, permite incadrarea- Caracter spontan al accidentelor hemoragice- Prez lez petesiale- hemoragii spontane sau posibil de
provocare a acestora prin testul garoului- Pred manif hemoragice superficiale (teg= aspect de
echimoze, mai rar hematoame diseminate multiple)- Absenta hemoragiilor profunde (intraarticulare,
intramusculare,viscerale, parenchimatoase- Tendinta hemoragiilor precoce la niv lez de continuitate cu
sistarea ulterioara tardiva a acestor hemoragii
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1. Sd purpurice:
Biologic:
- Din explorarile de hemostaza este alterat:
- Timpul de sangerare
- Testul garoului
= teste de evaluare a functionalitatii a etapei vasculoplachetare a hemostazei
! Testele ce exploreaza coagularea = nealterate
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2. Sd coagulopatice: caracterul provocat al accid hemoragice (ele nu
sunt spontane ci conditionate de traumatism) lez petesiale nu vor aparea (atat cele spontane cat si cele provocate de testul garoului)- uneori pot apare in coagulopatii severe –provocare minima, neobservabila
Manifestarila niv teg si mucoaselor= echimoze si hematoame masive, mici /multiple la niv traumatismului
Hemoragii profunde intraarticulare, musculare si a org parenchimatoase (rinichi, ficat, etc)
Cronologia aparitiei hemor este obisnuit tardiva (odata exteriorizate ramane o hem incoercibila)
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2. Sd coagulopatice: Biologic:
- Timp sangerare - Testul garoului
- Timp de coagulare = afectat hipocoagulabilitate- situatii in care deficitul nu este major, timpul de coagulare
sa nu fie afectat (hemofilii cu timp de coagulare N)- Etapa formarii de tromboplastina endogena cu alterarea
timpului de tromboplastina, protrombina si tromboplastinogen
def fact protrombinic (II,VII,X) alt timpului Quick, timp izolat de proconvertina, proaccelerina
la niv f VIII alterarea timpului de trombina
Normale (pot fi in CID, afibrinogenemii)
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Purpura trombocitopenica autoimuna a copilului
= stare patologica definita prin hiperdistructia necompensata, de cauza autoimuna a trombocitelor circulante + (facultativ in f severe) o distructie a precursorilor trombocitari)
Epidemiologie: -2-3% din totalul boln spitalizati, afect de 3-5X mai frecv decat leucemia
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Purpura trombocitopenica autoimuna a copilului Etiopatogenie:- De nat autoimuna (PT idiopatica)- s- a dovedit
aparitia Ac antitrombocitari, avand ca substart IgG, mai rar IgE- au specificitate pt Ag proprii trombocitare, fixandu-se pe trombocit cu legare de complement lez Tr
duratei de viata liza TrHiperdistructia periferica (deobicei la niv splinei,
mai rar ficat-cand au ca substrat IgG)-declanseaza o hipertrombocitopoeza medulara cu scopul compensarii deficitului din compartimentul periferic
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Purpura trombocitopenica autoimuna a copilului Clinic:- 70%- inf de cai resp sup (deobicei), alteori rubeola,
rujeola, varicela si rareori vaccinari- preced cu 1-4 sapt simpt caract
- Dupa infect –per de latenta=1-4 sapt- Apoi manif cl de boala: - ac si zgomotos - rareori debut insidios(se leaga de progn prost evol
spre cronicizare)- Extravazatele hemoragice sang spontane purpurice cu
aspect de petesii, echimoze,vezicule si bule hemoragice cu sediu difuz, numeroase si diseminate “copil ciomagit”
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Purpura trombocitopenica autoimuna a copilului
Clinic:
- La niv mucoaselor petesii si bule hemoragice(sever), gingivoragii, epistaxis, metroragii, hemoragii dig si meningocerebrale
- Rar- usoara splenomegalie (acrosabila), usor palpabila la 1 cm sub rebord
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Purpura trombocitopenica autoimuna a copilului Biologic:- Tr-penie (Tr<100 000/mm3)- Indici de megatrombocite (similar cu testul reticulocitelor pt anemie)
caracter recuperativ- Ts prelungit- Deficit / iretractibilitatea cheagului- Alterarea timpului de consum de protromb (sever)- Timp de coagulare si celelalte =N- HLC: - anemie posthemoragica - Le - anemie±leucopenie (autoagresiune hematologica complexa)- medulograma: populatie megacariocitara N sau excesiva cu dev la
stg a formulei megacariocitare- Imunologic: Ac antitrombocitari- Izotopi radioactivi (Tr cu Cr marcat) - duratei de viata - sediul de sechestrare (deobicei splina)
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Purpura trombocitopenica autoimuna a copilului F clinice:- Usoare Tr>50 000/mm3- Medii Tr 10 000-50 000/mm3- Severe Tr<10 000/mm3Evolutiv: - ac: cu restabilire < 1 luna - subacute :1-3 luni - cr >3 luniDg+- Asp clinic- Tr-penie- Caracter megacariocitar al medulogramei natura
periferica de hiperdistructie Tr
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Purpura trombocitopenica autoimuna a copilului Dg#:A. Alte trombocitopenii de cauza periferica:- tr-penii izoimune- Tr-penii-trombocitolitice din inf severe (mononucleoza
infect, rubeola, rujeoal) - de sechestrare din hipersplenism - de consum (CID) - mecanice (proteze valvulare)B. Tr-penii de cauza medulara centrala amegacariocitara - f congenitala - f dobandita prin substitutie (leucemii, limfoame,
metastaze ggl) - din aplazii medulare
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Purpura trombocitopenica autoimuna a copilului
Dg#:
C. Cu trombocitopatii:
- Clinic se manifesta la fel
• Trombastenia Glanzman
• Trombocitopatia dobandita sau medicamentoasa (aspirina, fenilbutazona, clorpromazina)
- nr Tr in discordanta cu Ts
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Purpura trombocitopenica autoimuna a copilului Tratament:- spitalizare-risc de evol severa in f ac sau spre cra. Igiena -evitarea traumatismelor, masurilor terap sangerande :i.m.,
punctii C.I. aspirinab. etiopatogenic:
• -corticoterapia: Prednison (prednisolon): - 2mg/kgC/zi cura de atac 10-14 zile - se poate creste doza la 3 mg/kgC/zi daca nu raspunde - cu raspuns bun- trat de intretinere: Prednison:5-10mg/sapt
sub control biol al tr
• splenectomia- indicata in f corticorezistente corticointolerante (ulcer,DZ,HTA)
corticodependente• Temporizarea splenectomiei timp de 1 an, exceptie:- Tr-penia severa cu risc de hem cerebrala- Bolnavi <3 ani
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Purpura trombocitopenica autoimuna a copilului
• splenectomia:- Intraoperator –pregatirea adecv de masa tr - controlul splinelor supranumerare- Postoperator – monitorizare pt - - accidente trombotice - risc infectios (pneumococ, haemophilus influenzae) - Forme fudroiante cu evol letala rapida (24-48 h)Profilaxie cu - ATB – Penicilina retard 2 ani - vacc antipneumococice, anti haemophilus influenzae
• Imunosupresoare-de exceptie
• Ig polivalente in doza mare1-2 g/kgc/zi 4-5 zile Rol de blocare a monocitelor
• Plasmafereza
• Tratament substitutiv in f severe- MT 1U/10kg
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Purpura trombocitopenica autoimuna a copilului Evolutie:- PT autoimuna la copil-evol fav- Risc de cr: 10-15% (cele cu debut insidios)- Risc de letalitate : 1%- Cauze de deces : accid hemoragice
meningocerebrale, mai rar dig, intraperitoneale- Sechele- legate de accidente ale analizatorului
vizual - Pareze, paralizii, sd epileptice - Rar cecitate, surditate
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Purpura anafilactoida (sdr purpuric vascular)- Purpura Henoch Schonlein
stare patologica indusa de vasculita imunologica a corionului superficial sau diverselor viscre (renal)
Epidemiologie:- frecv intalnita la copil
- varsta de prescolar cu precadere mai ales la sex masc
- incid sezoniera cu frecv primavara si toamna
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Etiopatogenie:
mecanism imun de hipersensibilizare sau autoagresiune
Organe tinta: peretele vasc :declansarea proceselor se realizeaza predilect prin inf (strepto, rar staf, vacc, alergeni alim, seroterapie, intepaturi de insecte)
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Tablou clinic: precesiune prin inf Per de latenta 1-2-3 sapt Debut manifest clinic al perioadei de stare:- purpura: -particularitati: expresie maculopapuloasa pseudourticariana –nu este cu
prurit, cu caracter hemoragic manif petesiale buloase, echimotice sau necrotice-fiind mai
rare distributie topografica -f sugestiva: - simetrica - pred distala, paraarticulara (picioare, gambe) si fesier - f rar fata, trunchi – nu sunt afectate - ± edeme hemoragice ciudat localizate (buza, scalp,
carp, tars) tend evolutiva in pusee succesive de accentuare la frig si
ortostatism
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Tablou clinic:
Manif articulare :60-80% din cazuri cu aspect artritic sau artralgic ca in RAA (purpura reumatoida)
Manif digestive (60-80%) –nespecifice: - varsaturi, dureri abd, constipatie - specifice: - de tip hemoragic (hemoragii dig) 10-15% au simpt abd f zgomotoasa:• tablou ac de pseudoabd chir (pseudoapendicita, ileita,
stenoza dd) exista riscul declansarii unui abd ac chir si aparitia
invaginarii si a perforarii intestinale
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Tablou clinic:
manif renale:- 30-40% d.p.d.v biologica- Mai rar clinic 70-80% din cazuri G.N-este o GN focala
oligosimptomatica (nu cu edeme, rar cu HTA) frecv hematurie macroscopica
! Explorare renala de 2-3X/sapt
- Alte manif rare :pulm, cardiace, nervoase convulsivante, testiculare
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Laborator:
modif nespecifice fara val dg hemostaza indemna, uneori scurtarea Tc testul garoului – (+ in teritoriul unde exista manif
bolii) leucocitoza, neutrofilie, eozinofilie=nespecifice teste renaleVSH, reactanti de faza acIgA , CIC
-
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Dg +:
argumentatie clinica si normalitatea hemostazei
Dg#:- Cu alte vasculite imunologice - colagenoze - LED - artrita reumatoida infantila - dermatomiozita- Cu alte vasculite infectioase: - septicemii - ricketsioze- Exanteme : - eritem multiform - rash din septicemii- Alte afect : - fragilitate vasc excesiva - hipovit C
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Tratament:
repaus absolut la pat dietetic: -evitarea alim energizante ±
restrictii impuse de corticoterapie etiologic- daca streptococ: penicilina – 7
zile penicilina retard pe perioada activa a bolii
patogenic – corticoterapie in forma cu manif articulare si abd : Prednison 1-2 mg/kgC/zi
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Tratament: in functie de forma clinica• F cutanata –antihistaminice - vasculotrope - vit E, C• F cu GN – corticoterapie - anticoagulant cu- heparina 500-700 Ui/kgC/zi-
reducand doza in fctie de val Tc - heparina retard sc - antiagregant – aspirina 4-10 mg/kgC/zi - ± indometacin - apoi imunosupresoare: - cu risc si rar - ciclofosfamida 2-3
mg/kgC/zi - imunran 2-3 mg/kgC/zi - exceptie : plasmafereza
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Evolutie.Prognostic
Evol:- f ac cu evol autolimitata in general
Progn: - imediat – poate fi nefavorabil prin accid chirurgicale si GN rapid progresive
- letalitate 1-2%
- tardiv – agravat de GN cu tend de evol persistenta 25-30%