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Board Review

6/17/2009

CLOTTING TESTS

XII VIII IX PL X V II Fibrinogen PL

VII TF PL

Fibrin

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 acquired F VIII inhibitor DIC LA Factor XII deficiency chronic liver disease

Q: Dx?

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

Q: Dx?
A: chronic liver disease (vitamin K deficiency; DIC; acquired f V inhibitor)

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 What do you do? Begin full-dose heparin Change to LMWH D/c heparin and observe D/c heparin and start warfarin D/c heparin, start danaproid or lepirudin

Heparin induced thrombocytopenia


HIT (HIT-1, HIT-2, HAT) Platelet of > 50 % from baseline after 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)

Thromboses (arterial and venous)

LMWH and HIT

HIT incidence: 2.7 % standard heparin vs. < 1 % LMWH

but: 90-95 % cross-reactivity!

HIT
XII VII

thrombin
[ASH 2001, Ortel]

Arixtra = Fondaparinux
clot

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 %

Q: How will you treat?


A: FFP (Cryo; PCC; Amicar; nothing)

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

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

Q: Dx? A: von Willebrands disease (Glanzmann, mild hemophilia A or B, dysfibrinogenemia)

von Willebands disease


Prevalence: 1 % of population Bruising mucosal (nose, gums, menstrual, tonsillectomy, tooth extraction) Dx: von Willebrand factor activity factor VIII bleeding time, PFA100 von Willebrand factor multimers type 1: quantitative. 90 % of cases (all multimers present, but decreased) type 2: qualitative (missing large multimers). 2A, 2B, 2N, platelet type type 3: severe 1 (all multimers missing) type 1: DDAVP (0.3 g/kg) i.v., s.c., or intranasal type 2: Humate P (= factor VIII concentrate with vWf)

Rx:

Cryo

MKSAP-Q6

A 86

B 87

C 89

D 90

E 88

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 Q: How to best give and monitor heparin rx? heparin Use anti-Xa level, not aPTT LMWH and follow aPTT IVC filter

Antiphospholipid antibodies
I) antibody test (ELISA)

APL A
L A ACA

anticardiolipin anti-2-glycoprotein I

II) functional test


lupus anticoagulant (inhibitor)

2-GP I

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)
aPTT

XII

VII TF
PL PT

PL

X
DRVVT PL

Fibrinogen

Fibrin

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 Q: Cause of the prolonged prothrombin time?

DIC
Congenital XII deficiency F VIII inhibitor

LA-hypoprothrombinemia syndrome
Vitamin K deficiency

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

Patient bleeds: Hypoprothrombinemia Low platelets

MKSAP-Q9
33 yr man with CP

Coro: extensive 3 vessel disease


Non-smoker, normotensive, lipids including lipoprotein(a) normal

Strong FH of premature CAD and stroke

Q: Which thrombophilia test is most likely going to be abnormal?


Homocysteine
Factor V Leiden Prothrombin 20210 mutation Antiphospholipid antibodies

MKSAP-Q10
21 yr woman from Nigeria SOB, CP post airline flight

On OCPs
Mother: thrombophlebitis x 2 postpartum. Sister: sudden death pstpartum

VQ: PE
Q: Which diagnostic study should be done next? 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

Thrombophilia
venous Protein S deficiency protein C def. AT III def. factor V Leiden prothrombin 20210 homocysteinemia MTHFR polymorphism antiphospholipid antibodies yes yes yes yes yes yes no yes arterial yes yes yes no no yes no yes

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)

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

www.fvleiden.org

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

Q: How long to anticoagulate? A: 3 months (INR 2.0 3.0)

DVT/PE: Anticoagulation how long?


1. after transient risk factors 2. Idiopathic DVT/PE: short (6 weeks - 3 mo) at least 3 months

- Factor V Leiden, hetero


- Prothrombin 20210 mutation: - Protein C or protein S deficiency - ATIII deficiency: - homozygote factor V Leiden: - Factor V Leiden + prothrombin 20210 mutation:

at least 3 months
at least 3 months ??? indefinite indefinite indefinite

- APLA syndrome:
- Tumor 3. Recurrent DVT/PE

indefinite
indefinite (LMWH) indefinite

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

Q: Rx options?

A: LMWH s.c. in therapeutic doses + warfarin 5-10 mg qd (ASA, prophylactic LMWH, daily LMWH monitoring, 15 mg warfarin)

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

Q: What should be included in evaluation?


1. Thorough PE and p.a. and lat. CXR
2. Thorough PE, p.a. and lat. CXR, and chest/abdo MRI 3. Thorough PE and p.a. and lat. CXR plus EGD/colonoscopy 4. Thorough PE and CEA

MKSAP-Q14
28 yr woman

ACA
three 1st trimester pregnancy losses Now 6 weeks pregnant

Q: What is the most appropriate treatment?

MKSAP-Q14
28 yr woman

ACA
three 1st trimester pregnancy losses Now 6 weeks pregnant

Q: What is the most appropriate treatment?


Careful observation only
Prednisone 40 mg qd Aspirin IvIg infusions Heparin and ASA

MKSAP-Q15
35 yr woman
epistaxis and bruising Plt: 5,000

Refractory ITP (fails steroids)

Q: What therapy is best for long-term response? A: splenectomy (?Rituxan = Rituximab?)

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

ITP - Therapy
Guided by patients bleeding symptoms

Treat when platelets < approx. 20 50,000 or when bleeding


Prednisone 1 mg/kg Slow taper when platelet count > 50,000

Iv Ig 1 g/kg q d x 2 d
Anti-D antibody (WinRho) single dose. Response in 2-3 d. Duration of response: 1 month Relapse: splenectomy. Good response in 60-70%. Rituximab. If splenectomy fails: cyclophosphamide, azathioprine, danazol, multiagent chemotherapy

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