Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.

Ttp shu

521 views

Published on

TTP-HUS
Thrombotic microangiopathy is marker for TTP/HUS as well as for DIC/DIC-like (secondary thrombotic microangiopathy-TMA), this giving us a first overlapping area.
ADAMTS 13 (ADAMTS 13 Ab/ADAMTS 13 relative or absolute deficiency) - a recent marker for TTP, regulatory complement factors flaws (CFH, MCP-CD46, IF and CD46 Ab, CFH Ab as well)-pathogenetic elements in D- HUS, increased PAI 1-recently proved for TTP, all of this are nowadays valid pathogenetic lego bricks in that wall we call secondary TMA, this giving us our second overlapping area.
Plasma exchange, grade IA recommendation for “true” TTP, has been gaining a place in the last decade in the supportive basket for secondary TMA(e.g., sepsis ), this giving us a third overlapping area.
At least three overlapping areas and the lack of certain particular cases (malignant hypertension, HCT related TMA, D+ HUS early years) deliver us a syndrome (TMA) likely to be highly responsive to plasma exchange and, in certain situations, to tailored corticotherapy, monoclonal CD20 Ab, C5 Ab.

Published in: Health & Medicine
  • Be the first to comment

Ttp shu

  1. 1. PREZENTARE DE CAZ PURPURA TROMBOTICA TROMBOCITOPENICA –SINDROMUL HEMOLITIC UREMIC
  2. 2. Pacienta 74 ani, hipertensiva (tratata) si diabetica, este internata la Spitalul Clinic Caritas in vederea interventiei chirurgicale pentru ruptura de perineu. De retinut bacteriuria simptomatica preoperator diagnosticata – germene identificat E.Coli.
  3. 3.  Evolutie imediat postoperator favorabila.  Ulterior, in cursul aceleiasi internari, la aproximativ 2-3 zile statusul neurologic al pacientei se altereaza – confuza, ulterior neresponsiva la stimuli verbali dar fara semne de focar.  Stabila hemodinamic si in respiratie spontana.  Penseaza diureza.  HLG – trombocitopenica, anemica (normocroma si normocitara), fara ͟ŵisĐarea͟ testelor de coagulare.  Sindrom inflamator – neimpresionant.
  4. 4. Este transferata la SUUB pentru investigatii suplimentare si tratament. Admitem in STI o pacienta varstnica cu disfunctie neurologica, renala si ͞heŵatologiĐa͟. Este intubata si ventilata mecanic .
  5. 5. Neurologic – nu raspunde la stimuli verbali si nociceptivi, manifesta convulsii generalizate ce cedeaza la benzodiazepine si examenul CT cerebral nu releva modificari TD recente, in acord cu examenul clinic neurologic ce nu a relevat semne de focar. Disfunctie renala – necesita terapie de epurare extrarenala – CVVHDF. La montarea CVC – PVC≈0 cm H2O(?) Mecanism prerenal ≥12h asociat unui rinichi in context diabetic si hiperteŶsiv→ATN(si PTT-SHU)
  6. 6. HEMATOLOGIC... Trombocitopenie. Anemie normocroma normocitara. LDH, bilirubina indirecta crescute. Haptoglobina - lipsa kituri. PT, APTT cvasinormale. FDP prezenti (context postoperator!) Fibrinogen normal spre crescut. Frotiu sg. periferic – schizocite si coifuri (ER), policromatofilie, trombociti mari.Rar sferocite. Test Coombs negativ.
  7. 7. Disfunctie renala(context DZ /HTA/hvol) Disfunctie neurologica Interventie chir. Si bacteriurie cu E.Coli MAHA si Trombocitopenie
  8. 8. Anemie hemolitica microangiopatica + trombocitopenie + teste coagulare ͞Ŷeŵiscate͟
  9. 9. TTP(thrombotic thrombocytopenic purpura) Congenital TTP(Upshaw-Schulman syndrome) •Inherited ADAMTS 13 deficiency Idiopathic TTP •Aquired ADAMTS13 deficiency •Without acquired ADAMTS13 deficiency  Dupa Williams, 7th Ed
  10. 10. HUS(hemolytic uremic syndrome) Diarrhea positive(infectious, Shiga toxin associated) • Sporadic • Epidemic Diarrhea negative • Inherited complement regulatory protein deficiencies(factor H, membrane cofactor protein, factor I)  Dupa Williams, 7th Ed
  11. 11. Secondary thrombotic microangiopathy CID de diverse cauze poate poza drept TTP-SHU in conditiile in care raportul consum factori coagulare-trombocite este mic. Schizocitele, anemia hemolitica si trombocitopenia sunt multifactoriale si ...relative(vezi pac. Normal, valve protetice, HTA maligna) 1-18% pare a fi un interval larg si... cuprinzator Unitate heterogena in patogeneza si raspuns la PLEX  Dupa Williams, 7th Ed
  12. 12. Secondary thrombotic microangiopathy 1  Dupa Williams, 7th Ed
  13. 13. Secondary thrombotic microangiopathy 2  Dupa Williams, 7th Ed
  14. 14. Secondary thrombotic microangiopathy 3 Bacterii
  15. 15. Secondary thrombotic microangiopathy 4 Medicamente
  16. 16. Secondary thrombotic microangiopathy 5 Diverse
  17. 17. Patogeneza Usor de retinut cand e vorba de PTT Si mai simplu la SHU D+ Un pic mai greu daca e SHU D-Devastator la cele secundare
  18. 18. Patogeneza - TTP Dupa Wintrobe,2009
  19. 19. Patogeneza - TTP
  20. 20. Patogeneza - TTP
  21. 21. Patogeneza – TTP DefeĐte iŶ aĐtivitatea fiďriŶolitiĐa;tPA ↓ sau PAI-1 ↑Ϳ Nivel crescut de trombomodulina Complexe imune altele decat ADAMTS13-IG Anti CD36(ligand al ADAMTS13) Injurie endoteliala(second hit) Absenta HLA DR 53(protectiva) Dupa Wintrobe,2009 Dupa Williams 7th
  22. 22. Patogeneza SHU D+
  23. 23. Care pe care?
  24. 24. • Tetrahymena (left) is approximately fifty times the size of the bacteria it's trying to capture but it's entirely vulnerable to the Shiga toxin the bacteria carry in their DNA(E.Coli O157:H7)
  25. 25. Patogeneza SHU D+ (Shiga toxin) Proteoliza Injurie endoteliala Activare plachete Fav. Rinichi prin asoc.Gb3
  26. 26. Patogeneza SHU D+ • Injurie vasculara colonica. • Stim. direct agregarea plch. • Stim. Expresia factorului tisular in celulele tubului proximal. • Senzitiveaza cel. epiteliale tubulare la toxicitatea hemului. • Expresie Gb3 diferita adult-copil. • Creste expresia TNF renal
  27. 27. Patogeneza SHU D- • Mutatii in cele 3 proteine reglatoare de complement(CFH, MCP sau CD46, CFI sau IF) • Autoanticorpi –anti MCP, anti CFH, anti IF C5bC6C7C8C9-MAC
  28. 28. Pentade,triade si diade
  29. 29. Pentade,triade si diade LDH Schyzo Trpenie
  30. 30. Pentade,triade si diade Trpenie HA MA
  31. 31. TTP-HUS
  32. 32. Terapie
  33. 33. Terapie
  34. 34. Terapie - PLEX TTP congenital TTP idiopatic HUS D – ? HUS D + ?(adult) • HUS D + copil • Chimioterapie • Posttransplant • HUS D – ? • HUS D + ?(adult)
  35. 35. Terapie - PLEX • Zilnic 1 -2/zi • 3-145 sedinte • Oprire gradata • LDH,Brb 0,08×G×(1-Ht)
  36. 36. Terapie Corticoterapie(TTP idiopatic, SHU D -) Rituximab(Mabther a) Cyclosporine Vincristine Imunoglobuline Infuzie PPC
  37. 37. Terapie - inutile • Splenectomia • Heparina • Antiagregante plachetare
  38. 38. Succes
  39. 39. Bibliografie Williams 2007 Wintrobe 2009 Uptodate 2011 Harrison 17th Hillman Emedicine Oklahoma TTP-HUS registry

×