Download - Semiologia afectiunilor ganglionare
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Semiologia afectiunilor ganglionare
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Sistemul limfatic –sistem vascular +statii gg, care comunica cu sistemul venos
Circulatia imfatica –lenta- contractia muschilor scheletici- respiratie- musculatura neteda a veselor limfatice- efectul de suctiune al presiunii negative din vasele mari- pulsatiile arterelor invecinate- peristaltismul intestinalGanglionii:organe periferice limfoide conectate la circulatie
prin limfatice aferente si eferente si venule post capilare inalt endotelizate
Functii-epurare,depozit, raspuns imun
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Structura gg
• Zona pericapsulara( paracorticala) +e sinusurile medulare celule B
• Zona corticala cu foliculi primari (limfTCD4+ si CD8+) + centri germinativi ( stimulare ag)
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Caractere clinice ale gg normali ( dimensiuni, mobilitate, sensibilitate, consistenta, localizare)
• GG –normali-dimensiuni reduse, (nepalpabili) ,mobili ,nedurerosi, consistenta elastica,
• Gg patologici - dimensiuni crescute, frecvent poliadenopatii sau conglomerari gg, durerosi, aderenti de plan profund /superficial, duri, fluctuenti,fistulizati,tegumente modificate,treneu limfangitic +/-
• +/- splenomegalie,hepatomegalie,(hiperplazia sistemului limfoid)
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Afectiuni gg- etape diagnostice
• Anamneza• Examen fizic local • Examen fizic sistemic• Teste uzuale• Hemograma frotiu periferic/maduva • Teste imunologice (autoanticorpi,
ab.antibacterieni ,antivirali,..)• Punctie / biopsie gg• Imagistica pt evidentierea grupelor gg
(ecografie, limfografie,scintigrafie-Ga,CT,RMN)
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Semne si simptome- determinate de localizarea adenopatiilor
Sindroame compresive: v,cava superioara,inf.Tromboze, dispnee , wheezing tuse- traheepareza de diafragm - n.frenic, disfonie - n.laringianEdem limfatic/obstructie limfatica - Edem de culoare normala, consistenta crescuta,
temperatura normala,+infiltrarea partilor moi,+/-leziuni cutanate
secundare, - pleurezie, ascita, pericardita, icter, insuficienta
renala,sindroame hemoragipare,
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clasificare
• Acute,cronice• Adenopatii generalizate• Adenopatii localizate ( regionale)• Adenopatii dureroase : inflamatorii, BH,• Adenopatii nedureroase:mm,sifilis• Adenopatii nesupurate• Adenopatii supurate: tbc,tularemie,
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Afectiuni asociate cu marirea gg
.Boli infectioase:
1 virale: hepatite virale,mononucleoza inf.AIDS,rubeola,varicela,herpes zoster,..
2.bacteriene: streptococi,stafilococi,brucella,..
3.fungice: histoplasmoza, coccidiomycosis
4.Clamydia trahomul,limfogranulomatoza benigna
5.Mycobacterii (tbc…..,lepra)
6.paraziti: toxoplasmoza
7.Spirochete (sifilis, leptospiroza)
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Afectiuni asociate cu marirea gg
• Afectiuni imunologice• -AR,LES,dermatomiozita,reactii la
medicamente,s.Sjogren,limfadenopatia angioimunoblastica,ciroza biliara primitiva, sarcoidoza, B.Kawasaki,b Still
• Afectiuni maligne• Hematologice: limfoame maligne,leucemii acute ,
cronice,histiocitoza
• Metastaze: melanom,s.Kaposi,…• Endocrine: hipertiroidie
• Afectiuni de depozit:Gaucher,
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limfangita
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Sindroame histiocitare - grup larg de afectiuni caracterizate de proliferarea anormala a macrofagelor si celulelor histiocitare
• 1.histiocitoza Langerhans- rara, predominant la copii,B>F,simptomatologia depinde de organul infiltrat( os, piele,dinti,gg,gl.endocrine,ficat, splina,
• -granulomul eozinofil: leziuni osoase+ tumefiere locala- radiologic- leziune similara osteosarcomului
• -boala Hand - Schuller-Christian -granulom eozinofil multifocal- defecte osoase, exoftalmie,infiltrat gingival cu tulburari ale dentitiei, afectare pulmonara
• Biopsie: celula Langerhans- histiocit dentritic multinucleat situat in zonele B ale gg + eozinofile, macrofage,limfocite
• Prognostic- variabil ( insuficiente de organ, malignizare)
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Pacient :NB 61ani- stare febrila, dispnee,palpitatii- Fa paroxistica
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Mononucleoza infectioasa: infectie cu VEB( virus herpetic B limfotrofic- cu receptori CD21- post infectie lif.B exprima ag.de membrana –tinte de
raspuns imun celular; virusul exprima o gena similara cu IL-10-( blocare INF-γ,NK
-stimulate producerea de anticorpi policlonali( IgM,IgG)
• Febra, limfopenie ,faringita ,limfocitoza atipica
• Adenopatii, splm ,hepatita fulminanta, pericardita miocardita,encefalite,mielita transversa, aa.hemolitica
• Hipergamaglobulinemie policlonala + anticori heterofili
• Anemie,leucopenie,ab.antiVEB• Evolutie: • - cronicizare• -reactivare din v.cantonat in
celulee epiteliale din orofaringe• - inf.latenta-limfoame,carcinom
anaplazic nazofaringian
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Citomegalovirus - v.ADN herpetic –replicarea virala este insotita de aparitia unor incluzii intranucleare mari
- transmitere prin saliva,urina,perenteral, sexual,transplant
- purtator
• Inhiba raspunsul cel.TCD4+
• Sindrom mononucleozic
• Afectare marcata SNC
• Infectie invaziva la pacientii HIV+
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SIDA-HIV prin revers transcriptaza este integrat in AND celulei gazda- limf.T CD4+,macrofage, celule
microgliale, epiteliale, endoteliale
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Toxoplasmoza-
• Transmitere : ingestie, transfuzii,transplant, transplacentar
• -invadeaza tesuturile – proliferare –necroza+ inflamatie
• Febra, artralgii,mialgii, rush • Adenopatii generalizate• Avorturi recurente,afectare SNC
cutanata,hepatosplenmgalie, leziuni oculare
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Sarcoidoza- af.inflamatorie caracterizata de formarea de granuloame necazeante in tesuturile afectate
• 20-40 ani, F>B• Gramulomul sarcoid: -acumulare locala de celule
inflamatorii-cel.epitelioide,cel gigante L ,cel.TCD4+ ( crescute in lavajul br.alv)
• Activitate lif.T de tipTh2 (IL-4,il-10)sistemic crescuta • Etiologie : trigger infectios (My) –proteina micobacteriana
rezistenta la degradare • Astenie fizica, eritem nodos, artralgii, adenopatii,
af.oculara (+sin.sicca), lez.pulmonare( adenopatii,infiltrate,alveolita,interstitiala, pleurezie),miocardita, CMH,coronarita, hepatomegalie, splenomegalie…
• Angiotensin convertaza crescuta, hipergamaglobulinemie, hipercalciuurie…
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Adenopatie tbc
-My bovisPrimoinfectieTbc secundarCaractere: - Adenopatie cervicala- Gg fluctuenti- Aderenta plan superficial- Fistulizeaza- Tuberculide - Vindecare cicatricialaSimptome generale de impregnare bacilara
(subfebra,scadere ponderala,sindrom inflamator prezent)
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tuberculide
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Boala Hodgkin• Incidenta crescuta• Adultii tineri B>F• VEB?• Celula Sternberg-Reed- cel.BClasificare histologica• - predominanta limfocitara• -scleroza nodulara• - celularitate mixta• - depletie limfocitara• Investigatii: …LDH crescut,F alc.crescuta,biopsie
medulara,gg,stadializare CT, monitorizare ecografica• -
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Depletie limfocitara
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Stadializare Ann Arbor
• St I un singur grup gg sau structura limfoida ( splina, timus, inel Waldeyer)
• St II 2-n grupe gg de aceeasi parte a diafragmului
• St III 2-n gr.gg de ambele parti ale diafragmului (a+b)
• St IV afectare de structuri extralimfatice
• A,B( +simptomatologie sistemica)
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Boala Hodgkin- adenopatii
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Infiltrate hepatice in BH
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Limfoamele ne-H- cuprind toate tumorile primitive limfoide care sunt in afara BH
• Clasificare Kiel 1988( dupa tipul de celula)• Limfoame B ( grad scazut de malignitate-celule diferentiate)-
limfomul malign limfocitar,LLC,leucemia ‘’cel.paroase’’• Limfoame limfoplasmocitoid – limfocite mici + numar crescut de
celule plasmocitoide- poate evolua ca o gamopatie monoclonala- B.Waldenstrom
• Limfomul plasmocitar –• Limfomul centroblastic /centrocitic – tumora creste folicular, gg mari,
generalizati• Limfomul Burkitt – celule mari , intens bazofile, + celule
stelate( histiocite mari, cu fagocitoza crescuta)• Limfomul limfoblastic-LLA• Limfoame T- grad crescut de malignitate
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Limfom folicular :expansiunea nodulara a celulelor maligne( contin limfocite mici cu nuclei clivati)
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Limfom difuz cu celule B
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Leucemiile – grup de afectiuni maligne ale tesutului heatopieticasociate caracteristic cu numar de leucocite
crescut in maduva si/sau periferie • Incidenta crescuta B>F• Etiologie:radiatiiionizante,medicatie
citotoxica,expunere la benzen,retrovirusuri,genetic,deficite imunologice
• Acut/cronic- aprecierea formei de boala
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clasificare
• LLA• Tipul comun- preB• Cel.T, cel.B, nediferentiata• LMA• M0-nediferentiata M1-diferentiere miniala• M2diferentiata M3promielocitara• M4mielomonocitara M5monocitara• M6eritroleucemia M7 megacariocitara• LLC: B comuna, T rara• LMC :Ph+,Ph-,leucemia eozinofilica
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Punctie iliaca
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Frotiu periferic LMC
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LMC pm
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LMC
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Corpi Auer
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Hairy cell leukaemia
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L.Eritroblastica fp
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LLC- neoplasm caracterizat prin acumularea de limfocite monoclonale (B in 95%)
• Celule cu apoptoza scazuta ( bcl2+), oprite in diferentiere , cu un continut crescut de IgM intracelular si IgM de suprafata scazute ( un singur tip de lanturi λ,κ)
• Cel.B ag panB-Cd21,20,24,23,receptori pt.Fc• Cel.T CD5, CD25(receptor de IL-2)
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Stadializare Ray
• I limfocitoza
• II limfocitoza + adenopatii
• III limfocitoza+ adenopatii+ hepatosplenomegalie +Hb<11g
• IV III+ Hb<11g+trombocite <100,000/mm3
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stadializare
• Stadiul clinic A- sub trei grupe gg afectate, fara anemie, fara trombopenie
• B – peste trei gr.gg ,fara anemie, fara trombopenie
• C anemie/trombopenie + n grupe gg
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Semne si simptome
• Generale: scadere ponderala, astenie, febra,prurit, cefalee
• Gg +/-compresii (icter, tromboze ,disfagie, disfonie,c.med,)obstructii (pleurezie,ascita-chilotorax,pericardita..)
• Infiltrate limfoide ( ficat- icter, i.hep. ,medulare, SNC)• Sindrom de hiperviscozitate : tulburari circulatorii• Hiperuricemie • Anemie,trombocitopenie• Patologie asociata : ICC,SCA,infectii • Prognostic : depinde de tipul celular, virsta ,comorbiditati
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LLA
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LLC
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LLA LLC
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crioglobulinemia
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Metastaze splenice
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Metastaze gg
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