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Hematology 2 - Hemostasis Vol. II

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omarys's version from 2017-10-03 17:18

Section 1: Hypercoagulability (m section 2 in htlgy 1)

Question Answer
Acquired risk factors for VTEage (coag' factors more active) ; "acute" diseases (COPD, stroke) ; trauma ; surgery ; neoplasia (itself + tx) ; AI diseases (SLE, RA, IBD, ITP) & nephrotic synd. ; hematologies (MPD, PNH) ; the HOAAAAARmones is pregnant (estrogen, HRT) ; immobilization ; varicose veins ; pregnancy & obesity
Protein C and S def' isAD (heterozygotes are asymp' till 3rd-4th decade)
Purpura fulminans (as in homozygous protein C/S def')small thrombi in blood vessels just below skin > skin necrosis and bleeding (can necessitate amputation)
Warfarin/coumadin can lead to _______ similar to in protein C/S def' (purpura fulminans) bc protein C is affected BEFORE clotting factors are.skin necrosis (=hypercoagulability....whereas warfarin is meant to PREVENT coagulation...but at later stages)
Other than (1) protein C/S def', another reason for congenital thrombophilia is (2)aPCR / factor V Leiden
In aPCR, the mutation is apoint mutation (Arg >> Gln) - leads to chronic hyperactivity of coagulation system - also to higher thrombin-AT complexes (indicate activation of system) and higher prothrombin residues (same)
Factor V Leiden is a pretty ____ ____ and not ____ _____ a _____.common variant ; universally considered ; disease (but rather- polymorphism)
(3) Prothrombin mutationpoint mutation in the 3'UTR of prothrombin gene >> higher expression of gene (not different AA sequence!)
(4) isAT3 mutation (AT3 needs GAGs, deactivates thrombin, 9 and 10)(AT3 mutation is much rarer but the added risk is higher than in FVL or prothrombin mutation)
Threshold concept brahuwot m8
memorize

Section 2: Assessment of bleeding patients & 2 bleeding disorders

Question Answer
(1) is to assess whether the bleeding has caused ahematoma vs. contusion
(2) iswhere the bleeding is. If local then there's no systemic problem. Bleeding in multiple places or in the trunk is a sign of something wrong w the coag' system as a whole.
Relates to (2)- Among mucosal bleedings, epistaxis alone (unless bilateral/severe/unexplained by anyfin else) is-a WEAK sign of a coagulation problem.
Relates to (2)- If there's GIT/GUT/Lung bleeding,first eliminate other, more dangerous causes of this then move to check coagulation system
(3) is trigger: ___ bleeding is a sign of a coagulation system disorder.spontaneous
The fibrinoLYTIC 'system' is more active innasal cavity, mouth, urinary stem (+bladder & prostate), endometrium
(4) is timing- if bleeding occurs immediately after trigger, the problem is most likely in ____.platelets (whereas if it takes time to VISIBLY bleed, it'll probs be the coag' cascade- but the cascade is compromised 'microscopically' just as early as in platelet damage)
(5) other factors to consider: bad wound healing factor XIII def
Multiple abortions might be due todef in factor XIII or def in fibrinogen (hypofibrinogenemia)
What's usually def in the elderly and leads to compromised wound healing&coagulation?Vitamins K and C
Coagulation disorders might also emanate fromcirrhosis or other liver damage
Some ____ ____ are also blood thinners and must be considered.diet supplements
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Section 3: Bleeding disorders: Hemophilia

Question Answer
A:B8:9
Hemoph A incidence1:5k male births (B is rarer: 1:30k)
The genes for both F8 and F9 are onchromosome X
Despite being hereditary- hemophilianeed not involve family history (spontaneous mutation in gamete meiosis)
A patient with hemoph A will have intact ___ but longer ___.PT (extrinsic/Tissue pathway) ; PTT (intrinsic, starts at 12)
All daughters of a male hemoph patient areobligatory carriers
4 examples of how hemoph patients may present?(1) good old bruise/ecchymosis ; (2) muscle hematoma (e.g. after IM injection) ; (3) subgleal/cephalo hematoma (during birth) ; (4) hemophilic arthropathy
Tx?synthetic factor 8 or 9
FFP?Fresh frozen plasma (contains needed factor; easy to inject from home; preventative measure)
TorF: It's difficult to treat hemoph.False. The tx is pretty efficient (aside from AB's that might attack the foreign factor; more common in A)
In cases of family history, it's possible to perform ____ to ___ for healthy ____ only.PGD ; select ; embryos
It's unlikely that hemoph patients will only bediagnosed at old age
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Section 4: Bleeding disorders: Factor XI deficiency

Question Answer
The body can produce a stable clot even w/o factor XI if the anatomical location is notwhere the fibrinolytic system is highly active (e.g. gums, urinary tract, etc)
Factor 11 def patients usually suffer from bleedings due to ____ ____ rather than spontaneously.mucosal trauma
F11def is (inheritance?)-AD
Only ___ can identify F11def.PTT
Factor 11 (homodimer) > a monomer that has 4 ___.domains (apple 1-4)
Which domain in F11 makes the 2 monomers bind one another?Apple 4u
Mutation 2in Apple 2 (changes conformation of F11, inactivating it- THE WORSE of the 2 evils)
Mutation 3in Apple 4 (monomer binding...only in ashkenazi jews)
Severe XI def is defined asXI < 15% activity
Tx of F11def?hexakapron (lysine analogue- binds plasmin and prevents it from degrading fibrin)(given in minor surgical procedures)
In a 'high-risk' surgery, hexakapron is admin. along withFFP
FFPFresh frozen plasma is a blood product made from the liquid portion of whole blood. It is used to treat conditions in which there are low blood clotting factors (INR>1.5) or low levels of other blood proteins
Ppl w F11def have fewerCVAs, DVTs (MI incidence is similar to general population tho)
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