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Therapy of patients with severe hemophilia (including hemophilia with inhibitors) and other severe bleeding disorders could be monitored and guided based upon special clotting assays , eg thrombin generation and thromboelastography. In this study blood sampled from patients with bleeding disorders will be evaluated applying ex- vivo spiking assays with various coagulation concentrates to potentially address the feasibility of replacement /bypass agents/ combined therapy for future bleeding episodes. Patients that will be further treated by any regimen potentially suggested (as standard care- not within trial) will be thereafter followed , including repeated lab studies to assess the impact of therapy upon hemostasis.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Hemophilia | Patients with Hemophilia A OR B | ||
| inhibitor patients | Hemophilia or FXI def with inhibitors | ||
| anticoagulants | patients under therapy with various anticoagulant drugs | ||
| thrombocytopenia | patients with ITP, chronic thrombocytopenia, chemotherapy induced thrombocytopenia | ||
| platelet function disorders | Glanzmann, Bernard Soulier, antiplatelet therapy | ||
| Fibrinogen disordsers | hyperfibrinogenemia, hypofibrinogenemia, dysfibrinogenemia | ||
| acquired hemophilia | patients with autoimmune Ab's to FVIII | ||
| RBD |
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| Measure | Description | Time Frame |
|---|---|---|
| Thrombin Generation Parameters pre and post therapy / spiking assays | Thrombin Generation Parameters (Peak height, ETP) before and after spiking assays with procoagulant drugs will be compared for each patient separately and within each group of patients diagnosed with similar coagulation disorder. | 1 year from enrollment |
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Inclusion Criteria:
Exclusion Criteria:
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patients with coagulopathies (see brief description)
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Gili Kenet, MD | Contact | +972526666385 | gili.kenet@sheba.health.gov.il |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Sheba Mediacl Center | Recruiting | Ramat Gan | 52621 | Israel |
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| ID | Term |
|---|---|
| D006467 | Hemophilia A |
| ID | Term |
|---|---|
| D025861 | Blood Coagulation Disorders, Inherited |
| D001778 | Blood Coagulation Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
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FXIII DEF, FV+FVIII DEF, FVII DEF, FV DEF |
| THROMBOLYTICS | Patients treated by thrombolytic agents |
| Hypercoag | thrombophilias, thrombosis- inc under therapy, acquired high thrombotic risk (eg: cancer) |
| D020147 | Coagulation Protein Disorders |
| D006474 | Hemorrhagic Disorders |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |