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board review 6

CLOTTINGTESTS. MKSAP-Q1. . 80 yr man3 week h/o massive bruisingon no meds; FH negativeLabs: platelets 350,000 PT 12 sec; aPTT 78 sec; Bleeding time: 6 min fibrinogen 390 g/dL; D-dimer: 1000 ng/mL aPTT 1:1 mix: 71 sec LFTs normal. Q: Dx?. acquired F VIII inhibitor DIC LA Factor XII deficiency chronic liver disease.

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board review 6

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    1. Board Review

    3. MKSAP-Q1 80 yr man 3 week h/o massive bruising on no meds; FH negative Labs: platelets 350,000 PT 12 sec; aPTT 78 sec; Bleeding time: 6 min fibrinogen 390 g/dL; D-dimer: 1000 ng/mL aPTT 1:1 mix: 71 sec LFTs normal

    4. Coag tests – MKSAP-Q2 32 yr man; hematemesis x 2 h Strict vegetarian diet x 4 weeks for weight control and abstained from alcohol for same period of time PE: pale, spleen 3 cm enlarged; liver not palpable Hbg 8.0; MCV 85; WBC 10; plts 75,000 PT 28 sec; aPTT 50 sec; BT 7.5 min; fibrinogen 165 mg/dL Albumin 2.0; ASAT 75; ALAT 45 PT 1:1 mix: 12 sec; aPTT 1:1 mix: 30 sec

    5. MKSA-Q3 46 yr man post CABG On heparin DVT prophylaxis 5000 U q 12 Day 4 post-op right calf swelling and pain – Doppler-US: DVT plts 75,000

    6. Heparin induced thrombocytopenia HIT (HIT-1, HIT-2, HAT) Platelet ? of > 50 % from baselineafter = 5 days of heparin (earlier if heparin given within last 3 months)PLUS PT and aPTT normal Lab demonstration of heparin-dependent antibodies- HIT ELISA (PF4) or- HIPA test (heparin induced platelet aggregation test)

    7. LMWH and HIT HIT incidence: 2.7 % standard heparin vs. < 1 % LMWH but: 90-95 % cross-reactivity!

    9. Bleeding – MKSAP-Q4 55 yr woman Planned cholecystectomy Hx: easy bruising, frequent prolonged nosebleeds Bleeding after nasal surgery Blood count normal PT normal; aPTT 64 sec; aPTT 1:1 mix: complete correction XII 110 %; XI 16%; IX 98 %; VIII 112 %

    10. Factor concentrates PCCs: II, VII, IX, X Bebulin®, FEIBA®, Autoplex®, Cryo: fibrinogen, von Willebrand factor (factor VIII) FFP: all other factors (little fibrinogen and von Willebrand factor) half-life of factor VII: 4 h Humate P: von Willebrand factor, factor VIII

    11. Bleeding – MKSAP-Q5 30 yr man Lifelong epistaxis + easy bruising Tooth extraction – bleeding for several days Adopted Hbg 13.0; MCV 78; plts. 250,000 BT 13.5 min; PT 12 sec; aPTT 40 sec; TCT normal Platelet aggregation study normal

    12. von Willeband’s disease Prevalence: 1 % of population Bruising – mucosal (nose, gums, menstrual, tonsillectomy, tooth extraction)

    13. MKSAP-Q6

    14. MKSAP-Q7 27 yr woman SLE 2 DVTs, now 3rd PT 13.6 sec; aPTT 43 sec; LA Standard heparin 5000 U bolus, then 1200 U/h. F/u aPTT 120 sec

    15. Antiphospholipid antibodies

    16. Lupus anticoagulant 1) Screening test (aPTT, dRVVT, KCT, Silica clot time) – prolonged 2) Normal plasma mixing study – does not correct 3) Confirm (mix with excess of phospholipids) – corrects- hexagonal phospholipid test- platelet neutralization procedure (PNP)

    17. MKSAP-Q8 20 yr woman, bleeds after dental extraction SLE – steroids. No h/o bleeding; plts 160,000; PT 17.5 sec; corrects with 1:1 mix to 11.4 sec aPTT 43.3 sec; does not correct with 1:1 mix Fibrin (ogen) degradation products normal

    18. APLA syndrome 1) thrombosis or 2) recurrent abortions 3) pos. APLA test (repeat) (+ thrombopenia, derm. or neurol. symptoms)

    19. MKSAP-Q9 Homocysteine Factor V Leiden Prothrombin 20210 mutation Antiphospholipid antibodies

    20. MKSAP-Q10 Factor V Leiden and factor II 20210 gene studies Protein C, protein S, and antithrombin tests Screening tests for cancer, incl. Chest and abdo CT A lupus anticoagulant panel

    21. Thrombophilia

    22. Factor V Leiden prevalence : 2 - 15 % (western world) RR for 1st DVT/PE: heterozygotes: 3 - 8 homozygotes: 80 heterozygotes + pill: 30-50 Diagnosis: coagulation test (APC resistance) or genetic test Not associated with arterial clots (except for selected patients)

    23. Prothrombin 20210 polymorphism prevalence: 2.3 % (normal population) mild risk factor for 1st DVT/PE: RR 2.8 risk for recurrence of DVT/PE: not increased associated with elevated prothrombin levels not associated with arterial thrombosis

    24. www.fvleiden.org

    25. MKSAP-Q11 47 year-old man DVT after 1 h airplane flight FH: uncle with DVT after hip arthroplasty, grandfather stroke age 68 Thrombophilia w/u negative

    26. DVT/PE: Anticoagulation – how long? after transient risk factors short (6 weeks - 3 mo) Idiopathic DVT/PE: at least 3 months- Factor V Leiden, hetero at least 3 months- Prothrombin 20210 mutation: at least 3 months- Protein C or protein S deficiency ???

    27. MKSAP-Q12 56 yr healthy man DVT calf and popliteal vein Adamantly refuses hospitalization

    28. MKSAP-Q13 75 yr man Calf and popliteal DVT after 10 h car ride Smoking. Otherwise healthy Phys. exam nl. CBC, PT, PTT, routine serum chemistry nl

    29. MKSAP-Q14 28 yr woman ACA three 1st trimester pregnancy losses Now 6 weeks pregnant

    30. MKSAP-Q14 28 yr woman ACA three 1st trimester pregnancy losses Now 6 weeks pregnant

    31. MKSAP-Q15 35 yr woman epistaxis and bruising Plt: 5,000 Refractory ITP (fails steroids)

    32. ITP 1:20,000 Antibodies against platelets, often against GPIIb/IIIa Dx: negative history, r/o all other causes: - iron studies (deficiency?)- vitamin B12, folate- blood smear (clumping?)- TSH (hypo?)- HIV(- ANA)- PT, PTT- bone marrow aspirate and biopsy: controversial

    33. ITP - Therapy Guided by patient’s bleeding symptoms Treat when platelets < approx. 20 – 50,000 or when bleeding Prednisone 1 mg/kg Slow taper when platelet count > 50,000

    34. Questions?

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