2013-11-01 Coagulopathies

Question 1

vW Dz

• inheritance pattern
• classification
• presentation
• labs
• tx

Answer 1

—autosomal dominant, most common bleedg d/o
CLASSIFICATION
—[1] quant 80%
—[2A,B,M,N] qual ~20%
—[3] absent vWF RARE
—acquired vWF def
PRESENTATION
—menorrhagia, cuts long bleed, mucocutaneous
LABS
—run gel, aPTT/VIII levels may be low b/o shepherd, incr PFA-100 time, abnl ristocetin plt aggr
TX
—DDAVP, plasma-deriv VIII, vWF+F8 (cyroprecip), OCPs, new antifibrinolytics

Question 2

Type O blood and vWF

Answer 2

lower levels; not true def but may have bleeding diathesis

Question 3

Hemophilia A & B
—deficiency?
—present?
—labs?
—tx?
—complications?

Answer 3

—A = Factor VIII def, x-linked recess
—B = Factor IX def, “Xmas dz”
—Both: severity depends on amt decr
—present w/ hemarthrosis, intramuscular, CNS, heamturia
—Labs: low aPTT corrects w/ mix, low Factors
—Tx:
MILD A: DDVAP
BOTH: recombinant or plasma-derived factors; prophylax now
—complications: HIV/HCV, arthritis, develop inhibs (bypass w/ FVII)

Question 4

Hemophilia C

Answer 4

autosomal recessive; heteros bleed

Question 5

parahemophilia

Answer 5

F5 def; heteros don’t bleed

Question 6

Acquired Hemophilia
—pathophys
—labs
—tx

Answer 6

—Abs to F-VIII
—aPTT prolonged does NOT correct w/ mix, Ab titer (low to high levels)
—Tx (low Ab titer): rF-VIII
—Tx (high Ab titer): FEIBA (F-8 Inhib Bypass Activ)
——rituxin + pred; cyclophosphamide

Question 7

Fibrinolytic bleeding
—presentation
—inheritance
—subtypes?
—Labs?
—Tx?

Answer 7

—delayed bleed
—usu acq

HEREDITARY D/O (RARE):
—Québec plt d/o: rare, AD, excess tPA/uPA.etc
—A.R. inhib defs: α2-antiplasmin, PAI1, F11

ACQUIRED: cancer, liver dz, DIC, release of PAs from prostate surg

LABS
—prolong PT/aPTT, sky-high D-dimer (r/o DIC)

TX
—plasma infuse

Question 8

Vitamin K deficiency

• needed for?
• causes

Answer 8

NEEDED FOR
Fs II, VII, IX, X, APC, APS

CAUSES
decr intake, malabs, EtOH; abx, salicylates, herbals, Vit A/E

LABS
PT/aPTT both prolong; corrects w/ mix
All those factors down

TX
P.O. or IV Vit K, FFP, prothrombin complex conc. (II, VII, IX, X)

Question 9

Coag in Liver Dz

Answer 9

-both bleed and clot, but bleeds usu dominate

BLEED B/C: all F’s low ‘cept VIII, low fibrinolytic factor inhibs, abnl fibrinogen

CLOT B/C: low APC/S, anti-thromb, plasminogen
—clearance of clotting factors
—low synth of anti-coags

LABS
—prolong PT/aPTT, low F’s ‘cept 8

TX: fix liver/transplant

Question 10

D.I.C.
—cause
—qu'est-ce qui se passe?
—Present
—Labs
—Tx

Answer 10

CAUSE: usu 2° infx or malig or OB or rattle snake or APL or adenocarcinoma

CE QUI SE PASSE: Pathological activation of the coag cascade—> widespread microthrombi—>

PRESENT
ischemia/infarction PLUS bleeding from mucosal sites like IV site e.g. b/c plts consumed

LABS
prolonged PT/aPTT, low plts, low fibrinogen, HIGH D-DIMER!, see Schitocytes

TX
address cause, cryoprecip, heparin