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HemeOnc > Week 3 - Coag > Flashcards

Week 3 - Coag Flashcards

1
Q
  • **how does thrombin limit coag cascade?
  • -activates protein _____ (esp on endothelial cells when thrombin bound to _______) which inactivates factors ___ and ____

–and also binds to ____ with heparin which acts as negative feedback loop on itself

A

PROMOTE - by turning fibrinogen (weak glue holding pl together) to fibrin (strong glue)

LIMIT

  • -activates protein C (enhanced by binding of thrombin to thrombomodulin on ENDOTHELIAL CELLS)&raquo_space;block factors 5a and 8a
  • -binds with heparin and ATIII&raquo_space; inactivates thrombin
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2
Q

primary vs hemostasis

  • def
  • clinical features of defects (bleeding where?)
  • labs (PT/PTT)

-what factors involved in intrinsic vs extrinisc pathway

A

PRIMARY = WEAK PLATELET PLUG

  • -platelet adhesion (Gp1b to Vwf) and aggregation (via fibringoen and gp2b3a)
  • -DEFECTS = mucosal / skin bleeding (#1 = nose)&raquo_space; low platelets / normal PT/PTT

SECONDARY = STRONG PLATELET GLUE

  • -glued together by making THROMBIN&raquo_space; converts fibrinogen (weak) to fibrin (strong)
  • -DEFECTs = severe bleeding (muscles, bones)&raquo_space; low platelets, Low PT (extrinsic - 7) and/or low PTT (intrinsic - 12, 11, 9, 8)
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3
Q
  • **Discuss the roll of amplification in the coagulation cascade
  • ex for each factor ___ activated of the extrinsic pathway, 10k ______ activated that converts fibrinogen to fibrin
A
  • 7&raquo_space; activates 10k thrombin

* amplification in every step of pathway (as more accumulates&raquo_space; more signals)

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4
Q 
Primary hemostasis
–Activation of platelets via receptors
–Needs \_\_\_\_\_ to initiate aggregatation 
–Creates a \_\_\_\_\_\_(white or red clot) 
–Dominant in \_\_\_\_(venous or arterial)  system
A 
Primary hemostasis
–Activation of platelets via receptors
–Needs vWF to aggregate
–Creates a platelet plug (white clot)
–Dominant in arterial system
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5
Q

Secondary hemostasis
–Requires the generation of _________ = factor _____
–Creation of fibrin from fibrinogen = (red or white clot)
–Dominant in the ____(venoous or arterial) syste

A

Secondary hemostasis
–Requires the generation of Thrombin (factor IIa)
–Creation of fibrin from fibrinogen (Red Clot)
–Dominant in the venous syste

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6
Q 
tissue factor (TF) activates factor \_\_\_\_\_ starting \_\_\_\_\_ pathway 
-requires \_\_\_\_ and \_\_\_\_
A

VERY IMPORTANT - if don’t have it they die

  • activates factor 7
  • starts the clot = EXTRINSIC PATHWAY

-requires Ca2+ and phospholipid

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7
Q

what factors are in common pathway

A

2,5,1 (2x5x1 = 10)

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8
Q

what factors in intrinsic and extrinsic pathway

  • ___pathway measured by PT
  • ____ measured by PTT
A

IN = 12, 11 , 9, 8 - PTT (more factors/letters)

EX = 7. - PT (less letters, less factors)

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9
Q
  • ____ initiates intrinsic pathway by activating ___ factor

- _____ initiates extrinsic pathway by activating ____ factor

A
  • SEC&raquo_space; 12&raquo_space; intrinsic

- TF&raquo_space; 7&raquo_space; extrinsic

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10
Q

3 things that block thrombosis:

  • heparin&raquo_space; blocks ___ factors (10/9/2 ; 7/10 ; or 8/10)
  • TFPI&raquo_space; blocks ____
  • thrombomodulin&raquo_space; _____

*which of these anti-coags is THROMBIN (2A) a part of?

A

HEPARIN&raquo_space; activate ATIII&raquo_space; blocks 10, 9 and and 2
(hep:thromb:ATIII complex also blocks thrombin)

TFPI = blocks tissue factor&raquo_space; block 7a, 10a

TM&raquo_space; 2a:C/S&raquo_space; 8 and 10

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11
Q

–Endothelium is an _______(anti or pro-coagulaant)

–Removing endothelium begins coagulation cascade via _____

As ______ remains on&raquo_space; inhibition will reassert control locally
» 3D reactions in the vessel remain slow&raquo_space; Endothelium adjacent to clot remains inhibitory

A

ANTI (inhibit coagulation dominates)

  • TF&raquo_space; activates 7 (extrinsic pathway)
  • THROMBIN (anti-coag by activating ATIII and C/S?)
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12
Q

***importance of membrane in coag/anticoag cascade

–membrane thought of as ____(2D or 3D) whereas lumen is _____(2D or 3D)

–reactions are FASTER in ______ (2D/3D) - why?? ***

A

–membrane = 2D vs. lumen = 3D

–reactions (whether its thrombin leading to initial coag explosion or its thrombin activating anti-thrombin on intact endothelial nearby) are FASTER in 2D (FASTER ON MEMBRANE)

–because in 2D&raquo_space; increase probability of molecules matching up properly / reacting in space

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13
Q

•Explain the functions of von Willebrand factor (vWF) in primary hemostasis,

  • ____ receptor on platelet binds to vWF
  • carrier protein for factor ______(increasing its 1/2 life)
A
  • vWF = allows for 1st stage of PRIMARY HEMO = vwf attaches to the collagen at injury site&raquo_space; allows for PLATELET ADHESION (tethers pls to collagen)
  • via Gp1B (on plt)
  • carrier protein for factor 8 (increases its half-life)
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14
Q

–Explain the common tests of vWF: Antigen testing, ristocetin, factor VIII level, and multimers, and explain how these tests can be used to classify von Willebrand’s disease

  • which test would dx TTP or subtypes of VWD type 2
  • test to order for VWD 2?
  • which would diagnose bernard-soulier syndrome
  • which would diagnose VWD 1 ?
  • VWD 3?

*which would use to test for TTP causing MHA?

A

–vWF antigen = measure amount of vwf in plasma (low in VWD 1 and 3)

–restocetin cofactor (vwf:Rcoa) = tests for Gp1b def (BERNARD -SOULIER) VWF ACTIVITY/QUALITY (TYPE 2 VWD)
((risto added to patients plasma (w/ vwf but NO plts) &raquo_space; expose to “fixed platelets”&raquo_space; watch agglutination))

-multimer analysis - distinguish subtypes of VWD type 2, diagnose TTP (adam def» Microangiopathic hemolytic anemia)

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15
Q

List the steps of platelet activation during primary hemostasis

-whats Gp1b, TXA2, Gp2b3a, ADP and FIBRINOGEN

A

1- vasoconstrition (endo releases endothelin)
2- platelet adhesion to vWF (via GP1b)
3- platelet degranulates&raquo_space; TXA2 (come over here guys!) and ADP&raquo_space; puts out GP2b3a (hand out for more platelets to join)
4- platelets aggregate to eachother via gp2b3a with FIBRINOGEN in middle

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16
Q

•Define the role of the platelet function assay as a screen of platelet function, and when platelet aggregation studies are indicated

  • -PFA-100 vs platelet aggregometry
  • -indications = measure platelet _____(quantity or quality) like in VWD type ____(1,2,3)
A

PFA-100 = platelet function analyzer&raquo_space; basically put blood into this fake blood vessel with fake injury and see howlong it takes to clot

platelet aggregometry - shine light into suspesion of platelet (cloudy) &raquo_space; add agonist for plts to bind&raquo_space; see increase transmisison of light if normal platelets (aggregates form&raquo_space; less cloudy)

INDICATION - when symptoms of QUALITATIVE platelet disorder (VWD type 2)

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17
Q

•Explain how the receptors Integrin αIIbβ3 (Fibrinogen receptor - Glanzmann Thrombasthenia) and GP Ib-IX-V (vWF receptor - Bernard Soulier Syndrome) function in primary hemostasis and how their absence interferes with platelet function

______ will aggultinate with ristocetin but _____ will not

A

–GT - integrina2b3 def = normal platelet count but primary hemostasis bleeding (petechiae, mucosal bleeding) because NO AGGREGATTION

–BS- GP1b def = plts can’t bind to vWF (NO ADHESION)&raquo_space; plts aggregate normally but don’t aggultinate with ristocetin

**GT will aggultinate with risto but BS syndrome will NOT

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18
Q

–arcidonic acid converted into _____ which is imortant for platelet aggregation and released via _____ GRANULES

–_____ also released via ______ GRANULES by platelets during degranulation phase that puts out gp 2b3a

A
  • thromboxane A2 = call signal&raquo_space; released via ALPHA granules
  • enzyme that converts = COX = inhibited by aspirin

-ADP released via DENSE granules&raquo_space; 2b3a

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19
Q

***define types 1, 2, and 3 von Willebrand’s disease (vWD) (do not worry about type 2 subtypes)

A
  • type 1 (auto-dom, variable penetrance)&raquo_space; NOT making ENOUGH vwf (primary due to decreased syntehsis and plasma secretion)
  • type 3 (auto recessive, rare)&raquo_space; not making ANY
  • type 2 = quality problem (defect in 1 of fxns of vwf with higher circulating vwf antigen levels)
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20
Q
  • plasma vWF circulates as _____(monomer or multimer) and converted into _____(mono or multi) by _____ enzyme
  • deficient in that enzyme get ______
A
  • multimer&raquo_space;> monomers VIA ADAMTS13

- no adam13&raquo_space; no Vwf&raquo_space; can’t initiate clots get MICROANGIOPTHY?

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21
Q

dx? 12 yo comes in with recurrent nose bleeds and petechiae, mom says runs in the family but not as severe, inc ptt, normal pt, increased bleeding time

A

type 1 VWD (auto dominant)

*inc bleeding time!!! … Would be NORMAL if hemophilia

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22
Q

dx? 12 year old comes in with recurrent nose bleeds and petechiae. Not super severe. vWF levels are NORMAL, ristocetin acofactor activity is low

A

type 2 VWD = quality problem (normal vwf levels tho)

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23
Q

clopidrogrel (plavix) = irreversible inhibitor of _____ leading to ______

A
  • inhibits ADP receptor&raquo_space; can’t put out Gp2b3a&raquo_space; platets can’t AGGREGATE
  • abciximab = monoclonal AB to integrin alpha2bgeta3
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24
Q

dx based on platelet aggregometry:

  • normal ADP and collagen curves, abnormal ristocetin curve
  • abnormal ADP and acollagen curves, mildly abnormal ristocetin
A
  • no ristocetin = no gp1b fxn = BS syndrome = NO ADHESION

- OK ristocetin, no ADP and collagen = gp2b3a problem (NO AGGREGATION)

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25
Q

Proximal VTE (to popliteal vein) _____(more or less) likely to recur, while Distal DVTs have _____ (more or) less risk of recurrence

A
  • proximal more > distal

- so anticoag can be more aggressive in distal

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26
Q

T/F - unprovoked DVT has lower risk of recurrence than most other causes (hospitalization, pregnancy, air travel, surgery)

T/F - surgery within the past 3 months has lower risk of recurrance than non-surgical risk factors (estrogens, hospitalization, pregnancy)

A
  • FALSE … unproved has HIGHEST RISK
  • TRUE
  • surgery-related = low
  • non-surgery-related = intermediate
  • unprovoked = HIGH
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27
Q

T/F - young men are at increased risk of another DVT and benefit from longer anti-coags

A

TRUE

-other benefitors = abnormal -D dimer or young-age (less-bleeding risk … so keep em on that anti-coags! )

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28
Q

D-dimers = _____

-should give continued warfarin (anticoag) to pateints with _____(normal or abnormal) D-dimer

A

-D-dimer = broken down fibrin mesh

  • normal d-dimer - warfarin benefit small
  • bad d-dimer(>500) - has greatest benefit of ongoing coagulation
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29
Q

T/F - presence of thrombophilia is NOT very predictive of increased recurrence risk

T/F - Patients with antiphospholipid antibodies, and combined defects have a HIGHER rate of recurrence

A

TRUE - …. they are just as much at risk as other people

*everybody who has had clot are at higher risk

TRUE - antiphospholipid antibodies are at greater risk

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30
Q

T/F - Patients with an unprovoked VTE, regardless of the underlying coagulation defect, have a HIGH rate of recurrence

A

TRUE

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31
Q

give some inherited and acquired causes of THROMBOPHILIA

A 
Inherited
•Deficiencies of natural anticoagulants:
•Antithrombin (AT)
•Protein C
•Protein S
•Factor V Leiden Mutation
•Prothrombin Gene Mutation

Acquired
•Antiphospholipid syndrome

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32
Q

_____ deficiency seen in 2-9% of patients with VTE, activated by thrombin and vit-K dependent

A

C

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33
Q

would diagnose ____ defiency in patient with Venous embolisms by activity assay that Measures the ability of patient’s plasma to inhibit a known quantity of factor IIa or Xa.

A

Antithrombin (1% of VTE)

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34
Q

_____ is most common inherited cause of VTE, increasing risk of VTE by 5-7x

T/F - synergistic effect with oral contraceptives increasing risk

A

factor 5 liedan&raquo_space; mutation in factor 5 gene so that protein is resistant to factor Va inactivation by active protein C

TRUE

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35
Q

APC sensitivity ratio = aPTT in the presence of APC / aPTT in the absence of APC

  • used to diagnose _______ inherited cause of thrombophilia

•______ (abnormal or normal) ratio > 2.0

A

If factor 5 resistant (ie, HAVE factor V leiden mutation) then ratio will be LOW

Normal ratio > 2.0 (The presence of APC increases the aPTT)

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36
Q

T/F - protein C and S deficiency can BOTH lead to increased risk of DVT

A

TRUE … lead to thrombophilia (pro-coag)

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37
Q

anti-beta2 glycoprotein and aCL antibody can be used to dx ____ cause of thrombophilia

T/F - detectable levels sufficient for dx

A

2 ELISA ASSAYS for dx of ANTIPHOSPHOLIPID:

  • antib2 glycoprotein
  • aCL antibodies

*FALSE - need moderate to high titer (lots of ppl have detectable)

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38
Q

T/F - prothrombin gene mutation can lead to VTE

A

TRUE …Leads to higher plasma levels of prothrombin&raquo_space; more thrombin

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39
Q

antiphospholipid syndrome leads to increased risk of _____(thrombosis or bleeding)

-mech ?

A

THROMBOSIS

mech:
•Enhanced platelet activation with consequent aggregation
•Enhanced tissue factor expression through monocyte activation
•Enhanced expression of tissue factor and adhesion molecules on endothelial cells
•Inhibition of the protein C/S anticoagulant pathway
•Activation of complement

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40
Q

dx? 29 year old female with PMH of sun sensitivity, skin rash presents with DVT, see prolonged clotting time (HIGH PTT) that does NOT correct with mixing, clotting time shortens with addition of phospholipid (ie, phospholipid dependent)

A

LUPUS ANTICOAGULANT SYNDROME
•Phospholipid-dependent inhibitor (perform 2 tests as each is insensitive) (examples: Lupus aPTT test and DRVVT (Dilute Russell’s Viper Venom Test)
•Clotting assays with low amounts of phospholipid

Expect to see:
•Prolonged clotting time
•Does not correct with mixing (confirms that the aPTT is prolonged as a result of an inhibitor)
•Clotting time shortens with the addition of phospholipid (establishes phospholipid dependence

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41
Q

how do you dx antiphospholipid syndrome?

A

ELISA (to test for anticardio or beta-2 glycoprotein antibodies)

42
Q

-TTP caused by _____ while HUS caused by ______

A
  • TTP = ADAM mutation&raquo_space; can’t break up vWF into monomers&raquo_space; platelts can’t adhere
  • HUS = 90% caused by E.coli O157:H7&raquo_space; shiga toxin &raquo_space; damages endothelium leading to clot formation (esp in glomerulus)

HUS = 10% caused by mutation in compleent pathway (factor H) &raquo_space; complement activated on endo cells&raquo_space; damage causes thrombosis in glomerulus

43
Q

T/F - patients with lupus anticoagulant are at risk of bleeding, while antiphospholipid syndrome patients are at increased risk of thrombi

A

FALSE … lupus anticoag (present in 15-30% of patientswith lupus) is MISNOMER becuase actually INCREASES RISK OF THROMBUS

-named anticoag becuase it increases PTT but antibodies are thought to interact with platelet membrane phospholipids, increasing adhesion and aggregation of platelets, which accounts for the in vivo prothrombotic character

44
Q

factor _____ comes in after primary and secondary heostasis and starts adding bonds to clot

A

13 - hard to break up these bonds?

45
Q
  • plasminogen activated to plasmin by ______
  • released by ______
  • plasmin role?
  • what is PAI1?
A
  • tPA (anchored in clot) or uPA (able to work in soluble form)
  • released by endothelial cells (and by inc thrombin levels - another ex of anticoag activity)
  • PLASMIN = degrades fibrin glue (FIBRINOLYSIS)

-plasminogen activatory inhibitor 1 (PAI1)&raquo_space; block TPA

46
Q

dx? 8 year old presnts with bloody diarrhea for 3x days, Cr and BUN show acute renal failure, ate undercooked meat last week, schistocytes on smear

A

HUS = #1 cause of ARF in kids

47
Q
  • Factor H ______(supresses or promotes) C3b convertase
  • deficiency leads to ____(+/-) complement
  • leads to _____ dx
A
  • factor H SUPRESSES complenet
  • so defiency allows TOO MUCH compleemnt activation > get hemolysis

-causes Minority (10%) of HUS

48
Q

Complement, 3 ways to activate (to get to C_____)

  • classical ?
  • lectin?
  • alternative?

*which is: activated by Ab-Ag, always activated, activated by cell wall components

A

C3b!!!!
Classical (Ab bound to Ag)
Lectin (bacterial cell wall)
Alternative pathway (always on)

49
Q
  • DIC leads to increased risk of _______(thrombosis, bleeding, both)
  • mech?
A

-BOTH!
Def= widespread microthrombi from oversctivation of coag pathwya&raquo_space; all those clots use up all the platelets&raquo_space; low circulating platelets = BLEED RISK

50
Q

dx? 42 y F presents with fever, altered mental status and fatigue, RBC low, plts Low, WBC normal, fever, see Petechiae on arms and mild purpura on back, see schistocytes on smear

A

thrombotic thrombocytopenic purpura

*similar presentation to HUS (except that is more common in kids and affects KIDNEYS) while TTP classically affects CNS !

51
Q

List the signs and symptoms of thrombocytopenia and recognize when thrombocytopenia is an emergency

  • -def =?
    • emergency = ?
A

DEF =

52
Q

Describe the general mechanisms by which thrombocytopenia develops.
Give ex of each

  • def production
  • accelerated destruction
  • abnormal pooling
A

-deficient production (AML, ALL)

  • accelerated destruction (ITP, HIT)
  • used up in clots (DIC, TTP, HUS)
  • abnormal pooling (ex - ENLARGED SPLEEN)
  • false + (pseudothrombopenia)
53
Q

Formulate a differential diagnosis for patients with thrombocytopenia, using data obtained from the history, physical, and peripheral smear

A

-k

54
Q

Differentiate between genetic and acquired thrombocytopenia by comparing and contrasting 3 representative diseases: heparin induced thrombocytopenia (HIT), Immune Thrombocytopenia (ITP), and MYH-9 disorders.

  • which has giant plts
  • what is most common cuase of thrombocytopenia
  • which has WBC inclusions
  • which do you transfuse with plts as rx
A
  • HIT - due to heparin, large plts on smear, no WBC inclusions, new/acute onset, DON”T TRANFUSE (can be at HIGHER RISK OF PLT)
  • ITP - due to IgG, most common cause of thrombocytopenia in KIDS AND ADULTS, kids usually self-limiting, adults worse, large but not giant plts on smear, no inclusions, DONT TRANFUSE
  • MYH9 - presents early (genetic) , ALWAYS GIANT PLT ON SMEAR, usually WBC inclusions, YES TRANFUSE
55
Q

**Review the characteristics and differential of a non-blanching / vasculitic rash and distinguish petechiae from vasculitis.

  • -petechiae is _____(palp/not palp) and ____(blanching/not blanchIng)
  • -vasculitis is _____(palp/not palp) and ____(blanching/not blanchIng
A
  • non-palp = petechiae (small) or ecchymosis (big)
  • palp = vasculitis (RAISED)
  • no blanch = all!
  • so both non blanch, but vasculitis is RAISED
56
Q

dx? bruise looking dots, thing that is NON-BLANCHING and slightly raised is = ____(petechiae, vasculitis, ecchymosis?)

A

VASCULITIS = palpable purpura resulint from blood vessel INFLAMMATION (raised and non-blanching)

57
Q

MYH-9 disorders lead to _____ (Hemophilia, Thrombocytopenia, immune thrombocytopenic purpura, microangipathic anemia)

A

THROMBOCYTOPENIA with large platelets (may-hegglin anamology)

  • auto-dom inheritance
  • MYH9 mutation = involved in cell motility and architecture in platelets, blood cells, kidney cells etc
58
Q

_____ gene mutation accounts for patient who presents with recurrent nose bleeds that are mild, on smear see GIANT PLATELETS and leukocyte inclusions, ristocetin test normal with plt count of 4K

-should you transfuse??

A

MYH9

-YES! since under 10K (or tranfuse if higher and they are undergoing surgery ! )

59
Q

dx? 32 year old comes in with recurrent nose bleeds and easy bleeding, has protein in urine and CKD stage I, deafness and cataracts. Several family members have similar findings Smear shows GIANT PLATELETS, inclusing in leukocytes

A

fechtner syndrome (another MYH-9 plts disorder) - similar to May-Megglin except + nephritis, deafness and cataracts

60
Q

what’s found in May-Hegglin anaomoly

  • smear?
  • plt count
  • leukocyte inclusions +/-
  • nephritis and deafness +/-
  • cataracts +/-
A
  • GIANNTTT
  • LOW (THROMBOCYTOPENIA)
  • leukocyte inculsions Y
  • no
  • no
61
Q

dx? 29 y old F with PMH of lupus, presents with recurrent nose bleeding and petechiae, plt count of 40k , large plts on smear, normal PT and PTT, increased megakaryocytes on bone marrow biopsy

A

ITP - immune thrombocytopenic purpura

62
Q

ddx of thrombocytopenia with schistoytes on smear

A

-chistocytes = something is SHEARING

  • TTP or HUS (microangiopathic hemolytic anemia)
  • DIC
  • malignant hypertension

*look for high LDH and low hepatoglobin and high indirect bilirubin

63
Q

dx? patient with DVT receiving heparin develops ecchymoses and blood in stool, plt count of 40k, large on smear, PT/PTT normal

  • should you stop heparin??
  • should you transfuse plts?
  • should you keep with anticoagulate?
A
  • -yes
  • -NO! becase HIT can lead to INC plt count
  • -yes
64
Q

T/F - druginduced thrombocytopenia can lead to platelet activation and increased risk of thrombosis

T/F- a common cuase of drug-induced thrombocytopenia is HEPARIN

A

TRUE (because of cytokiens released to immune response to immune complex antibodies that attach to plts)

TRUE !! heparin binds to Platelet factor-4 (PF-4) which IgG attaches to&raquo_space; spleen eats (thrombocytopenia) and ccytokines released&raquo_space; plt aggregation&raquo_space; thrombosis

65
Q

T/F - platelet transfusion are treatment choice for immune thrombocytopenic purpura

A

FALSE FALSE FALSE!

  • Ig will just destroy it
  • give them IVIG (decoy) and steroids short-term

-splenectomy as last resort

66
Q

dx? 30 year old F with no PMH, has had petechiae and nose bleed for 10 days, had a cold 2 weeks a go, plt count is 4,000, normal WBC, normal PT/PTT, give platelet transfusion that doesn’t improve after 2 hours

rx?

A

ITP!! give her steroids!!

67
Q

what is most likely diagnose if child presents with recurrent gum and mucosal bleeding, reports that there is a family history of “free bleeding” after mild injuries (like flu shots, brushing teeth etc)

**order of tests?

A

1 - count , #2 - PFA (plt fxn assay) then # 3 - vWD panel (vWF antigen, ristocetin, factor 8, vWF multimers)

-vWD = most common inherited coag disorder (like 1 in 100 to 1000)

68
Q

deep tissue bleeding in _____(hemophilia A, B, vWD type 1 disease?)

A

A and B

69
Q

dx? 9 year old with no family history of bleeding presents with post surgical bleeding after appendicitis. Scan shows deep tissue and joint bleeding. Has history of mucosal bleeding as well. Low factor 8, high PTT, normal PT, normal plt count and bleeding time

A

hemophilia A (8) de novo … confirm with FVIII test and genetics

70
Q

mixing that improves (lowers PTT) is ____(hemophilia or antibody) and one that does not is ______(hem or antibody)

*wha

A

lower/correct = hemophilia
no correction = antibody

*anti-phospholipid antibody probably

**note - remember you need a surface, Ca2+ and lipid in order to artificialyl mimic clot in “blue top” tube

71
Q

*always start on ______ (hep/war) first before starting ___ (hep/war)

A
  • heparin then do coumadin (warfarin)

- NEVER DO WARFARIN BY ITSELF (get dreaded skin bleeding)

72
Q

dx? 8 year old with PMH of recurrent otitis media has taken 3 courses of antiboitcs in past year, presents with recrurent nose bleeds and easy bruising, WBC is high, PTand PTT are high, normal platelet count

A

Vitamin K deficiency (From antibiotcis!! - kilsl bacteria – bacteria needed to make vit K&raquo_space; vit K needed for 2,7,9 and 10 and C = intrinsic (10,9) and extrinsic (7) pathway

73
Q

dx of high PT and high PTT? (4)

A
  • vitamin K deficiency
  • liver failure
  • DIC
  • fibrinolysis (plasmin overactivity) from prostatectomy (urokinase release activates plasmin) or liver cirrhosis (decreased alpha2-antiplasmin = inhibitor of plasmin)
74
Q

dx if high PTT, normal PT (3)

-most common?

A

*extrinsic pathway problem

  • hemophilia A/B
  • vWD – MOST COMMON!
  • other extrinsic factor abnormalities (12,11)
75
Q

clinical criteria for DIC

  • activation if _______(primary, secondary, both)
  • factors??
  • organ signs/damage needed for criteria???
A 
–Activation of coagulation
•Primary
•Secondary
–Activation of fibrinolysis
–Consumption of factors and inhibitors
–Evidence of end organ damage
•Not organ specific like HUS and TTP
76
Q

causes of DIC (TOMS)

A

Sepsis ***

–Malignancy ***
•Adult Carcinomas
•Acute Promyelocytic Leukemia (M3)

–Trauma / Head Trauma / Burns ***

–Obstetric causes ***
•Abruptio placenta
•Amniotic embolism
•Eclampsia
•Retained fetus
77
Q

_____ is most frequent assoc with DIC

A

SEPSIS. -Most frequent association with DIC

•Contributes to ARDS and multi-system organ failure

78
Q

T/F - PT and PTT not useful for dx of DIC because they can be up or down

A

TRUe

79
Q

In DIC p,atelet count is ______

A

Generally low but extremely variable

80
Q

T/ F - fibrin split products usually elevated in DIC

A

t

81
Q

______ indicates activation of thrombin and plasmin, while ____ only indicates plasmin activation

A

D - both

FSP - plasmin only

82
Q

therapy for DIC

  • give FFP if _____
  • give plts if _____
  • give cryoprecipitate if ______

T/F - fibrinolytics recommended to break up clots

A 
FFP (for prolonged PT/PTT)
–Contains all aspect of the clotting cascades
–Need to account for volume
•Cryoprecipitate (keep Fibrinogen >100)
–High levels of fibrinogen
–Factor VIII
•Platelets (keep >50K)
–Consumed
•Fibrinolytics
–Not recommended
83
Q

clinical criteria for DIC

  • activation if _______(primary, secondary, both)
  • factors??
  • organ signs/damage needed for criteria???
A 
–Activation of coagulation
•Primary
•Secondary
–Activation of fibrinolysis
–Consumption of factors and inhibitors
–Evidence of end organ damage
•Not organ specific like HUS and TTP
84
Q

causes of DIC (TOMS)

A

Sepsis ***

–Malignancy ***
•Adult Carcinomas
•Acute Promyelocytic Leukemia (M3)

–Trauma / Head Trauma / Burns ***

–Obstetric causes ***
•Abruptio placenta
•Amniotic embolism
•Eclampsia
•Retained fetus
85
Q

_____ is most frequent assoc with DIC

A

SEPSIS. -Most frequent association with DIC

•Contributes to ARDS and multi-system organ failure

86
Q

T/F - PT and PTT not useful for dx of DIC because they can be up or down

A

TRUE

87
Q

In DIC p,atelet count is ______

A

Generally low but extremely variable

88
Q

T/ F - fibrin split products usually elevated in DIC

A

TRUE

89
Q

______ indicates activation of thrombin and plasmin, while ____ only indicates plasmin activation

A

D - both

FSP - plasmin only

90
Q

if you have high retic, what RPI (low, normal, high) would indicate DECOMOENSATED anemia (ie, improper bone marrow response)

A

LOW RPI and high retic = BM cant keep up (trying to by increasing retics, but still anemic)

91
Q

spur cells seen in _____(3-4, S.O.A.R)

A

-Severe liver disease
–Obstructive jaundice
–Abeta lipoproteinemia
–Rare blood groups – McCloud phenotype

92
Q

sickle cell is ______(auto-dom, auto-rec, X-linked)

  • mutation?
  • impact on blood cell? (ie pathophys)
  • causes HbS in _____(ox or deox state)

-complications (2-3)

A

AUTORECESSIVE

  • glu&raquo_space; val substutiton
  • causes POLYMERIZATION of hemoglobin chains in the DEOXY state (pair up and polymerixes&raquo_space; all these polymers leads to a rigid cell&raquo_space; susceptible to sickling and fragmentation

DEOXY STATE = which there is more sickling when there is LOW o2

  • don’t carry o2 as well
  • free hg can consume NO&raquo_space; causes BV to CONSTRICT
  • vasoocclusion
  • shorted RBC survival
  • suseptible to infection
93
Q

T/F - people with Hgb AS (trait) can lead to problems under exxtreme stress like strenuous excercise

T/F - ppl with SS are usually worse than ppl with SC genotype

A

TRUE (rare tho) - usually asymptomatic

TRUE (SC very variable tho)

94
Q

T/F - patient with HgB SS usually presents in first 2 months of life

T/F - Hgb SS does not affect RBC life-span but does make them less functional (ie, carry less o2)

A

FALSE … usually after 6 months (1-2) with dactylitis

*first few months have HgB F (2 alpha, 2 gamma) so no symptoms

FALSE - span very short (~13 days)

95
Q

give prophylaxis _______ to ppl with sickle cell b/c very susceptible to pneumococcal disease

A
  • penicillin

- pneumococcal disease

96
Q

T/F - warm whether precipitates (increases) sickling in SCA patient

T/F - infection and dehydration can make stroke and actue chest syndromes and other omplications more common in pt with SCA

T/F - hydroxyurea decreases sickling rate in SCA pt and can be used as treatment

A
  • false - cold weather (don’t know why)
  • true
  • True!!!! … can be used as rx (interferes with polymerization)
97
Q

ACUTE CHEST SYNDROME is dreaded complication of ________ form of hemolytic anemia

  • what is pathophys
  • T/F - can’t distinguish from pneuonia so should treat with antiboiotics
A

SICKLE CELL&raquo_space; acute chest syndrome = get infarcts in BM&raquo_space; get throbi that embolizeto lungs

  • TRUE - indistinguishable from pneumonia! (chest pain, fever, caushing, hypoxia) &raquo_space; coverwith antiboditics and o2
  • transfusion if anemic
98
Q

T/F - there is increased prevlance of myocardial infarctions, strokes and hyperlipidemia in sickle cell patients

T/F- as SC patients there is increeased risk of complications like renal disease, liverdisease and pulmonary hypertension

A

FALSE - do NOT have increased risk of MI, but do have increased risk of stroke (why - not well understood)

TRUE

99
Q

What are indications for CHRONIC TRANSFUSIONS vs. EPISODIC transfusions in SCA pt?

A

CHRONIC =

  • progressive organ failure
  • stroke prevention
  • recurrent acute chest syndrome

Episodic - symptoms, ACS, organ failure, stroke

100
Q

_______ (3) organ failure (-opathys) are common in SCA patients.

-other complications?

A
  • eyes (retinopathy)
  • kidneys (nephropathYu)
  • liver disease

OTHERS = stroke, joints, necrosis/ulceration

HemeOnc (3 decks)

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