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heme/onc > bleeding disorders > Flashcards

bleeding disorders Flashcards

1
Q

interpretation of mixing studies:

correction of the test at time point 0 and at 2 hours implies

A

correction of the test at time point 0 and at 2 hours implies that the patient has a clotting factor deficiency

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2
Q

interpretation of mixing studies:

failure of the test to correct or a partial correction at 2 hours implies

A

failure of the test to correct or a partial correction at 2 hours implies that the patient has a circulating inhibitor protein (e.g. FVIII inhibitor, antiphospholipid antibodies, amyloid)

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3
Q

quantitates factor activity levels

A

Factor Assay

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4
Q

which factors are not measured by the PT or PTT

A

FXIII and the vWF:Ag

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5
Q

measures the conversion of fibrinogen to fibrin

A

thrombin time

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6
Q

thrombin time prolonged in (3)

A
  1. afibrinoginemia- no or very low fibrinogen
  2. dysfibrinoginemia- dysfunctional fibrin
  3. fibrin- heparin
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7
Q

von willebrand disease:

  1. bleeding disorder
  2. genetics
  3. vWF levels in blood type
  4. phenotype 1, 2 and 3
A
  1. most common bleeding disorder
  2. AD-variable penetrance and expression
  3. vWF levels lowest in type O and highest in type AB
    phenotype:
    i. partiaal quantitative deficiency
    ii. qualitative defects of vWF
    iii. severe or complete deficiency of vWF and moderately severe factor VIII deficiency
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8
Q

A large protein (850 - >10, 000 kDa) synthesized in endothelial cells (stored in Weibel-Palade bodies) and megakaryocytes (stored in platelet α granules)
It is assembled & circulates as multimers

A

vWF antigen

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9
Q

vWF has two major functions:

A
  1. mediated adhesion of platelets to endothelium

2. stabilizes FVIII in the circulation and as such has a role in blood coagulation

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10
Q

Acquired vWD

  1. autoantibodies
  2. hypothyroidism
  3. caridac
  4. other
A
  1. binding of vWF causes rapid clearance by the RES associated with SLE
  2. decreased synthesis of vWF
  3. destruction of vWF
  4. drugs
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11
Q

diagnosis of vWD (3)

A
  1. Pt is normal and PTT often normal but can be prolonged
  2. PFA-100 abnormal EPI and ADP
  3. specific testing required
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12
Q

diagnosis of vWD:

  1. FVIII activity
  2. vWF antigen
  3. vWF functional assay
A
  1. FVIII activity- low if vWF-ag low b/c vWF stabilizes FVIII in plasma
  2. vWF antigen- normal range diffres w/ blood type
  3. vWF functional assay- ristocetin cofactor assay (qualitative)
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13
Q

treatment of vWD

6

A
  1. DDAVP- desmopressin
  2. factor VIII plus vWF concentrates
  3. recombinant vWF concentrate
  4. treat underlying condition in acquired vWD
  5. Huma P
  6. cryoprecipitate
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14
Q
  1. useful in Type 1 vWD
  2. promotes release of stored vW-protein from the vascular endothelium
  3. Raises vWF level 2- 3 fold but decreased effect with multiple use
  4. is rarely used in type 2 subtypes, contraindicated in type 3
A

DDAVP- desmopressin

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15
Q
  1. replace vWF (e.g. Humate-P, Wilate, Alphanate)

2. required for Type 2, Type 3 and some Type 1 patient

A

factor VIII plus vWF concentrates

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16
Q

adjunct for the treatment of vWD (3)

A
  1. anti-fibrunolytic agent like epsilon aminocarpoic acid
  2. estrogens that can increas the level of vWF
  3. avoiding NSAIDS
17
Q
  1. X linked recessive disorder (males are affected)
  2. Carriers females can exhibit bleeding phenotype
  3. Bleeding due to FVIII deficiency
  4. Mutation are due to inversions, deletions, point mutations
  5. 1:5000 live male births
A

Hemophilia A

18
Q
  1. X linked recessive disorder (males are affected)
  2. Carrier females can exhibit bleeding phenotype
  3. Bleeding due to factor IX deficiency
  4. Multiple mutations including deletions & point mutations
  5. 1:25000 live births
A

Hemophilia B- Xmas dx.

19
Q

sites of recurrent hemarthroses with hypertrophy of synovial sheath over time and painful arthritis

A

target joints

20
Q

hemophilia

  1. both characterized by
  2. occur
A
  1. characterized by spontaneous hemarthorses

2. occur w/in the first year of life

21
Q
  1. Severe hemophilia: measured factor levels of ____; spontaneous and trauma related bleeding
  2. Moderate hemophilia: factor levels of_____; bleeding with trauma and rare spontaneous bleeding
  3. Mild hemophilia: factor levels of _____; bleeding with surgery and trauma, no spontaneous bleeding
  4. Very mild hemophilia: factor levels of ____; bleeding with significant trauma
A
  1. Severe hemophilia: measured factor levels of <1%; spontaneous and trauma related bleeding
  2. Moderate hemophilia: factor levels of 1-5%; bleeding with trauma and rare spontaneous bleeding
  3. Mild hemophilia: factor levels of 5- 25%; bleeding with surgery and trauma, no spontaneous bleeding
  4. Very mild hemophilia: factor levels of 25-50%; bleeding with significant trauma
22
Q

diagnosis of hemophilia:

  1. Pt
  2. PTT
  3. mixing studies
  4. factor assay
A
  1. Pt- normal
  2. PTT- prolonged– FVIII and FIX
  3. mixing studies- correction with 1:1 mix with normal plasma at both time 0 and at 2 hours
  4. factor assay - quantify level of FVIII or FIX
23
Q

therapies for hemophilia

A
  1. PRICE
  2. antifibrinolytics
  3. plasma derived- FVIII or FIX concentrates
  4. recombinant - genetically engineered
24
Q

complications of hemophilia

A
  1. artrhopathy
  2. infections
  3. inhibitors- neutralizad by high affinity IgG
25
Q

what is the goal of supression of inhibitors

A

desensitization – induce tolerance of the factor VIII or IX protein, stop production of the factor VIII or IX antibody

26
Q

bypass agents for patients with inhibitor issue used in acute hemorrhage

A
  1. recombinant FVIIa and emicizumab-kxwh
27
Q

chronic management of hemophilia (3)

A
  1. prophylactic therapy
  2. on demand treatment- replacement factor for elective procedures and with bleeds
  3. patient/family education
28
Q

hemophilia C

  1. deficiency in
  2. increased icidence in
  3. variable
  4. provoked usually
  5. treatment
A
  1. deficiency in FXI
  2. increased incidence in Ashkenazi Jews
  3. variable bleeding phenotype
  4. provoked bleeding after dental work
  5. treatment- Amicar or FFP/rFVIIa
29
Q

Acquired hemophilia:

  1. ab to
  2. occurs in
  3. consequence
  4. underlying disorder
  5. treatment
A
  1. ab to FVIII
  2. 75% after age 50
  3. death due to bleeding
  4. underlying disorder: none> autoimmune disorder
  5. treat underlying disorder, immunosupression
30
Q

thrombotic disorder that causes a consumptive coagulopathy

often resulting in hemorrhage, thrombosis, multi-organ failure

A

Disseminated Intravascular Coagulation

31
Q

DIC pathogenesis

  1. known as
  2. dysregulated thrombin generation leading to
  3. platelets, coagulation factors, fibrinogen all show a
  4. D-dimer
  5. PT and PTT
  6. treatment
A
  1. known as microangiopathic hemolytic anemia
  2. dysregulated thrombin generation leading to intravascular fibrin formation and secondary fibrinolysis
  3. platelets, coagulation factors, fibrinogen all show a decrease
  4. D-dimer is elevated
  5. Pt and PTT are prolonged
  6. manage bleeding patients and correct underlying disease
32
Q

hepatic cirrhosis is associated with severe coagulopathy

A

yep

33
Q

hemorrhagic disease of the newborn

A

it is due b/c the gut is relatively devoid of bacteria and the hepatocytes are immature

***Vitamin K is given IM to all newborns to prevent bleeding in the newborn period

34
Q

The bleeding time is a screening test which relies only on

A

integrity of blood vessels, platelet number and function and the von Willebrand factor.

35
Q

Schistocytes on the smear (microangiopathy) accompanied by low platelets, elevated PT and elevated PTT are the laboratory findings in

A

Disseminated Intravascular Coagulation (DIC)

36
Q

microangiopathy and thrombocytopenia but coagulation tests are normal

A

TTP (Thrombotic Thrombocytopenic Purpura) and HUS (Hemolytic Uremic Syndrome)

heme/onc (30 decks)

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