Clotting Factors
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Hemophilia A & B
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Path
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X-recessive
· A = Aight (A= Factor 8)
· B = 9
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Clx
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Prolonged bleeding after mild trauma (deep)
· Hemarthrosis, intramuscular hematomas
· GI/GU tract bleeding
· Intracranial hemorrhage
Complications: hemophilic arthropathy
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Dx
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· ⤴️ PTT/ ⓝ PT
· ⓝ PLT
· ❌ or ⤵️ Factor 8/9
· DDx: vWB has mucosal bleeding (not deep bleeding)
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Tx
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· Factor replacement
· Desmopressin for mild hemophilia A
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PLT:
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Immune Thrombocytopenic Purpura (ITP)
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Features
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· Commonly acquired form of thrombocytopenia / PLT Autoantibody
· May show ⊕ Hx of recent viral infection or comorbidity (HIV, HCV, CLL)
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S/S
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· Frequently Asx
· Mucocutaneous bleeding (Menorrhagia, epistaxis)
· Skin: Ecchymoses, petechiae, purpura
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Dx
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· Diagnosis of exclusion
· Isolated ⤵️ PLT
· ⓝ coagulation tests, platelet morphology (peripheral smear)
· HIV, HCV testing (commonly induces ITP)
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Tx
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Children 🧒🏻
· if cutaneous sx only → Observe
· if bleeding → Glucocorticoids, IVIG, or anti-D
Adults 🤵🏻
· if PLT >30k → Observe
· if cutaneous sx only → Observe
· if bleeding → Glucocorticoids, IVIG, or anti-D
· if PLT <30 k→ Glucocorticoids, IVIG, or anti-D
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Thrombotic thrombocytopenic purpura (TTP)
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Path
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· ⤵️ ADAMTS13 level → uncleaved vWF multimers →PLT trapping & activation
· Acquired (autoantibody) or hereditary
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Clx
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· Hemolytic anemia (⤴️ LDH, ⤵️ haptoglobin) w/ schistocytes
· ⤵️ PLT (⤴️ BT, ⓝ PT/PTT)
· Sometimes with: Renal failure / Neurologic sx / Fevere
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Tx
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· Plasma exchange
· Glucocorticoids
· Rituximab
· NEVER TRANSFUSE PLTs
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Hemolytic uremic syndrome (HUS)
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Path
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Infection w/ bacteria that has Shiga toxin (most commonly Escherichia coli [0157:H7]) → Vascular damage & microthrombi formation
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Clx
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· Pt reports having diarrheal illness (often bloody) some time in the past
· Microangiopathic hemolytic anemia (fatigue, pallor, schistocytes)
· AKI (Oliguria / Edema)
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Antiphospholipid Syndrome
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Dx
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Requires 1 clinical & 1 laboratory criterion must be met
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Clx
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· Vascular (Arerial / venous) thrombosis ✳️
· Pregnancy
· ≥3 consecutive fetal losses <10 weeks
· ≥1 unexplained fetal loss >10 weeks
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Labs
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⊕ Lupus anticoagulant
⊕ Anticardiolipin antibody
⊕ Anti-beta-2 glycoprotein I antibody
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Side Effects of Drugs
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Drugs that affect warfarin metabolism (affects CytP450)
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CytP450
Inhibitors
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· Acetaminophen, NSAlDs
· Metronidazole
· SSRls (Fluoxetine)
· Cimetidine
· Omeprazole
· Thyroid hormone
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CytP450 Inducers
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⤵️ Warfarin efficacy
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· Carbamazepine, phenytoin
· St. John’s wort
· OCP
· Phenobarbital
· Rifampin
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Warfarin Toxicity Tx
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INR
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Bleeding
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Tx
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<5
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X
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lower dose
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>5
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X
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Skip next 2 doses
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>9
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X
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· Stop warfarin
· Give Vit K
· Resume Warfarin on a lower dose
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>20
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· Stop warfarin
· Give Vit K
· Give FFP
· Resume Warfarin on a lower dose
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Pt is BLEEDING
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Emergency 🚨
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Same as ⤴️
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HIT Type 2
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Clx
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Pt on Heparin >5 days + any of these findings:
· PLT # ⤵️ >50% from baseline
· Thrombosis: Arterial or venous
· Necrotic skin lesions (at injection sites)
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Dx
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· Clx Dx (Start Tx right away before confirmatory tests)
· Gold standard test: Serotonin release assay
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Tx
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1˚ STOP ALL HEPARIN ✳️
2˚ Start argatroban or fondaparinux
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Fast HY Review 