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Diagnosi dei Difetti dell’Emostasi Primaria

Diagnosi dei Difetti dell’Emostasi Primaria. Marco Cattaneo Unità di Ematologia e Trombosi Ospedale San Paolo DMCO, Università di Milano. EMOSTASI. Fase vasopiastrinica (Emostasi Primaria) Fase della coagulazione Fase della fibrinolisi. EMOSTASI. Fase vasopiastrinica (Emostasi Primaria)

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Diagnosi dei Difetti dell’Emostasi Primaria

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  1. Diagnosi dei Difetti dell’Emostasi Primaria Marco Cattaneo Unità di Ematologia e Trombosi Ospedale San Paolo DMCO, Università di Milano

  2. EMOSTASI • Fase vasopiastrinica (Emostasi Primaria) • Fase della coagulazione • Fase della fibrinolisi

  3. EMOSTASI • Fase vasopiastrinica (Emostasi Primaria) • Fase della coagulazione • Fase della fibrinolisi

  4. EMOSTASI • Meccanismo di difesa che arresta il sanguinamento • da soluzioni di continuo dell’albero vascolare • Dipende da una complessa interazione tra flusso ematico, • parete vascolare e sangue (cellule e proteine del plasma) TROMBOSI Forma malregolata o inappropriata di emostasi

  5. Tutti i test di laboratorio che esplorano l’emostasi primaria sono utili solamente per la diagnosi dei difetti dell’emostasiNessuno di essi e’ utile per la definizione del rischio trombotico o per il monitoraggio della terapia antitrombotica

  6. FASE VASO-PIASTRINICA vaso sottoendotelio Lesione di continuo w w w w w sottoendotelio

  7. FASE VASO-PIASTRINICA w w w w w w w w

  8. Difetti dell’Emostasi Primaria • Piastrinopenie • Piastrinopatie • Difetti di proteine adesive (afibrinogenemia, malattia di von Willebrand) • Anemia (es: uremia)

  9. Difetti dell’Emostasi Primaria • Piastrinopenie • Piastrinopatie • Difetti di proteine adesive (afibrinogenemia, malattia di von Willebrand) • Anemia (es: uremia)

  10. PIASTRINOPENIAClassificazione • PIASTRINOPENIE EREDITARIE • PIASTRINOPENIE ACQUISITE: - Da ridotta o difettosa produzione midollare - Da aumentata distruzione/consumo periferico su base immune su base non immune - Da sequestro od anomalo pooling - Da emodiluizione • PSEUDOPIASTRINOPENIA (riduzione in vitro della conta piastrinica, per agglutinazione EDTA-dipendente)

  11. PIASTRINOPENIA Pseudopiastrinopenia?

  12. PIASTRINOPENIA • Pseudopiastrinopenia? • Striscio di sangue periferico • in EDTA

  13. Normale – Sangue in EDTA

  14. Pseudopiastrinopenia – Sangue in EDTA

  15. Satellitismo piastrinico

  16. PIASTRINOPENIA • Pseudopiastrinopenia? • Striscio di sangue periferico • in EDTA Sì No STOP • Ereditaria o Acquisita? • Conta piastrinica normale in passato? • Familiarità? • Anomalie morfologiche e/o funzionali? Ereditaria Acquisita

  17. Inherited Thrombocytopenias: a Proposed Diagnostic Algorithm from the Italian Gruppo di Studio delle Piastrine CL Balduini, M Cattaneo, F Fabris, P Gresele, A Iolascon, FM Pulcinelli, A Savoia, on behalf of the Italian Gruppo di Studio delle Piastrine Haematologica 2003, 88: 582-592

  18. PIASTRINOPENIA ACQUISITA • [Emocromo, MPV] • [Striscio di sangue periferico] • Anamnesi farmacologica e trasfusionale • Valutazione splenomegalia • Markers virus epatite, Herpes, HIV • Elettroforesi sieroproteica • Analisi aspirato midollare • (obbligatorio se >60 anni e se anomalie sangue periferico) • Ricerca ANA Aumetata distruzione/consumo Sequestro o pooling anomalo Ridotta/difettosa Produzione di MK/plts

  19. Difetti dell’Emostasi Primaria • Piastrinopenie • Piastrinopatie • Difetti di proteine adesive (afibrinogenemia, malattia di von Willebrand) • Anemia (es: uremia)

  20. Patients with bleeding diathesis, no thrombocytopenia:

  21. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100)

  22. Punteggio normali (0→3)basso (4→7)intermedio (8→11)alto (12→19) p Numero 41 53 22 12 TE (min) 5.3 (3,0-10,0) 5.0 (2.3-14.3) 5.0 (2.0-20.0) 4.8 (3.3-20.0) 0.870 PCE (s) 130 (85-244) 142 (88-300) 152 (74-300) 153 (95-300)0.004 PCA (s) 85 (67-179) 91 (57-182) 89 (59-300) 95 (73-207) 0.457 Valori mediani (range) Kruskall-Wallis test

  23. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P

  24. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P N

  25. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P PT aPTT PT, aPTT PT, aPTT, BT PT, BT N aPTT, BT PT, BT BT - aPTT

  26. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P PT aPTT PT, aPTT PT, aPTT, BT PT, BT N aPTT, BT PT, BT BT - aPTT Work-up for hemophilia

  27. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P PT aPTT PT, aPTT PT, aPTT, BT PT, BT N aPTT, BT PT, BT BT - aPTT Work-up for hemophilia FVII FXI, FIX FV, FX, FII FV FVII FVIII Fibrinogen Fibrinogen

  28. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P PT aPTT PT, aPTT PT, aPTT, BT PT, BT PTT, BT BT - N aPTT, BT PT, BT BT - aPTT PT PT, aPTT PT, aPTT, BT Work-up for hemophilia FVII FXI, FIX FV, FX, FII FV FVII FVIII Fibrinogen Fibrinogen

  29. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P PT aPTT PT, aPTT PT, aPTT, BT PT, BT PTT, BT BT - N aPTT, BT PT, BT BT - aPTT PT PT, aPTT PT, aPTT, BT Work-up for hemophilia Work-up for vWD FVII FXI, FIX FV, FX, FII FV FVII FVIII Fibrinogen Fibrinogen

  30. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P PT aPTT PT, aPTT PT, aPTT, BT PT, BT PTT, BT BT - N aPTT, BT PT, BT BT - aPTT PT PT, aPTT PT, aPTT, BT Work-up for hemophilia Work-up for vWD FVII FXI, FIX FV, FX, FII FV FVII vWD FVIII Fibrinogen Fibrinogen

  31. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P PT aPTT PT, aPTT PT, aPTT, BT PT, BT PTT, BT BT - N aPTT, BT PT, BT BT - aPTT PT PT, aPTT PT, aPTT, BT Work-up for hemophilia Work-up for vWD FVII FXI, FIX FV, FX, FII FV FVII vWD negative FVIII Fibrinogen Fibrinogen

  32. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P PT aPTT PT, aPTT PT, aPTT, BT PT, BT PTT, BT BT - N aPTT, BT PT, BT BT - aPTT PT PT, aPTT PT, aPTT, BT Work-up for hemophilia Work-up for vWD FVII FXI, FIX FV, FX, FII FV FVII vWD negative FVIII Fibrinogen Fibrinogen Work-up for Platelet Function Disorders

  33. Diagnosi di malattia divon Willebrand

  34. Diagnostic Flow Chart of VWD Types plasma VWF:Ag Absent Present Adapted from Castaman et al, 2003

  35. Diagnostic Flow Chart of VWD Types Type 3 plasma VWF:Ag Absent Present Adapted from Castaman et al, 2003

  36. Diagnostic Flow Chart of VWD Types Type 3 plasma VWF:Ag Absent Present plasma VWF:RCo Adapted from Castaman et al, 2003

  37. plasma VWF:RCo Rco:Ag Diagnostic Flow Chart of VWD Types Type 3 plasma VWF:Ag Absent Proportionate (0.7 – 1.2) Present Type 1 Discrepant (<0.7) Type 2 Adapted from Castaman et al, 2003

  38. plasma VWF:RCo Rco:Ag Diagnostic Flow Chart of VWD Types Type 3 plasma VWF:Ag Absent Proportionate (0.7 – 1.2) Present Type 1 Discrepant (<0.7) Type 2 Increased (0.2-0.8) R.I.P.A (mg/mL) Decreased (>1.2) Adapted from Castaman et al, 2003

  39. plasma VWF:RCo Rco:Ag Diagnostic Flow Chart of VWD Types Type 3 plasma VWF:Ag Absent Proportionate (0.7 – 1.2) Present Type 1 Discrepant (<0.7) Type 2 Increased (0.2-0.8) R.I.P.A (mg/mL) Decreased (>1.2) Type 2B Adapted from Castaman et al, 2003

  40. plasma VWF:RCo Rco:Ag Diagnostic Flow Chart of VWD Types Type 3 plasma VWF:Ag Absent Proportionate (0.7 – 1.2) Present Type 1 Discrepant (<0.7) Type 2 Increased (0.2-0.8) R.I.P.A (mg/mL) Decreased (>1.2) Plasma High Multimers Type 2B Adapted from Castaman et al, 2003

  41. plasma VWF:RCo Rco:Ag Diagnostic Flow Chart of VWD Types Type 3 plasma VWF:Ag Absent Proportionate (0.7 – 1.2) Present Type 1 Discrepant (<0.7) Type 2 Increased (0.2-0.8) R.I.P.A (mg/mL) Type 2A Absent Decreased (>1.2) Plasma High Multimers Type 2B Type 2M Present Adapted from Castaman et al, 2003

  42. plasma VWF:RCo Rco:Ag Diagnostic Flow Chart of VWD Types Type 3 plasma VWF:Ag Absent Platelet VWF Proportionate (0.7 – 1.2) Present Proportionate Type 1 Plasma FVIII:C/vWF:Ag Discrepant Discrepant (<0.7) Type 2 Type 2N FVIII binding assay Increased (0.2-0.8) R.I.P.A (mg/mL) Type 2A Absent Decreased (>1.2) Plasma High Multimers Type 2B Type 2M Present Adapted from Castaman et al, 2003

  43. Patients with bleeding diathesis, no thrombocytopenia: Screening tests: PT, aPTT, BT (or PFA-100) P PT aPTT PT, aPTT PT, aPTT, BT PT, BT PTT, BT BT - N aPTT, BT PT, BT BT - aPTT PT PT, aPTT PT, aPTT, BT Work-up for hemophilia Work-up for vWD FVII FXI, FIX FV, FX, FII FV FVII vWD negative FVIII Fibrinogen Fibrinogen Work-up for Platelet Function Disorders

  44. Aggregazione piastrinica

  45. OUT IN

  46. OUT IN OUT IN GPIIb/IIIa

  47. Epinephrine Thrombin TxA2 ADP 5HT Shear OUT IN OUT IN GPIIb/IIIa

  48. Epinephrine Thrombin TxA2 ADP 5HT Shear OUT IN OUT IN GPIIb/IIIa

  49. Epinephrine Thrombin TxA2 ADP 5HT Shear OUT IN OUT IN GPIIb/IIIa OUT IN

  50. Epinephrine Thrombin TxA2 ADP 5HT Shear OUT IN OUT IN OUT IN GPIIb/IIIa Adesive protein

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