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SBM hematology > coagulopathies > Flashcards

coagulopathies Flashcards

1
Q

name some hereditary primary bleeding disorders

A
  • vWD
  • qualitative platelet disorders (storage pool, Glanzmann, Bernard-Soulier)
  • congenital thrombocytopenia
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2
Q

name some acquired primary bleeding disorders

A 
Medications
Uremia
Thrombocytopenia
Immune
Non-immune
Cardiac bypass
Myeloproliferative d/o
Acquired vWD
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3
Q

name some hereditary secondary bleeding disorders

A

Hemophilia
Rare coagulation factor deficiencies
Fibrinolytic defects

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4
Q

name some acquired secondary bleeding disorders

A 
Liver disease
DIC
Vitamin K deficiency
Acquired coagulation factor inhibitors
Anticoagulation
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5
Q

acquired vessel wall bleeding disorders

A
  • vitamin C deficiency

- vascular trauma

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6
Q

vWD inheritance pattern

A

autosomal dominant

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7
Q

two functions of vWF

A
  • tethers platelets to collagen

- chaperones factor VIII

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8
Q

quantitative types of vWD

A

1 and 3

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9
Q

qualitative types of vWD

A

2

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10
Q

lab tests for vWD

A

coagulations screening

  • PT and TCT normal
  • aPTT possibly prolonged if factor VIII low
  • VIII low

platelet screening

  • prolonged PFA-100
  • prolonged bleeding time
  • abnormal aggregation with ristocetin
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11
Q

treatment for vWD

A
  • DDAVP (for type 1 and some type 2)
  • vWF replacement (cryoprecipitate, alphate)
  • antifibrinolytic therapy
  • birth control for menorrhagia
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12
Q

what is DDAVP and what does it do?

A

desmopressin, synthetic vasopressin

stimulates release of vWF and VIII from endothelial cells

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13
Q

when avoid DDAVP in treatment of vWD?

A

type 2B, exacerbates bleeding

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14
Q

deficiency in hemo A?

A

VIII

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15
Q

deficiency in hemo B?

A

IX

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16
Q

inheritance patterns of hemophilia

A
  • A and B are x-linked recessive

- C, parahemophilia are autosomal recessive

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17
Q

deficiency of hemo C?

A

XI

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18
Q

deficiency of parahemophilia?

A

V

19
Q

what is common in the female carrier state of hemophilia?

A

menorrhagia

20
Q

what are considered mild, moderate and severe for hemophilia A and B?

A

mild = 5-40% of factor level
moderate = 1-5% of factor level
severe = <1% of factor level
normal factor level is 50-150%

21
Q

expected labs in hemophilia A and B

A
  • platelet function normal
  • PT normal
  • prolonged aPTT except possibly in mild
  • aPTT will correct with mixing study
  • low specific factor levels
22
Q

bleeding manifestations in hemophilia?

A
  • may be delayed because primary still happens
  • bleeding into joints
  • CNS
  • bleeding after minor trauma
  • hematuria
23
Q

pathological issue with hemophilia

A

no thrombin burst with no IX or VIII

24
Q

hemophilia treatment

A
  • recombinant IX and VIII, replacing plasma derived
  • DDAVP sometimes effective in mild hemophilia
  • factors can be used prophylactically in severe hemophiliacs to keep factor level above 1%
25
Q

complications of hemophilia

A
  • chronic arthropathy
  • infectious diseases
  • development of antibodies against missing factor (might have to bypass using VIIa)
26
Q

explain acquired hemophilia

A
  • spontaneous autoantibody to factor VIII

- most are idiopathic

27
Q

labs in acquired hemophilia

A
  • platelets and PT normal
  • aPTT prolonged
  • aPTT will not correct with mixing study
  • low factor VIII
28
Q

treatment of acquired hemophilia

A
  • low antibody titer, treat with factor VIII
  • high antibody titer, treat with bypass agent (recombinant VII or FEIBA which includes II, VII, IX, X)
  • eradication by immune suppression
29
Q

characteristics of fibrinolytic bleeding

A
  • delayed 12-24 hours
  • mucocutaneous/oropharyngeal
  • menorrhagia, GU
30
Q

is hereditary or acquired fibrinolytic bleeding more common?

A

acquired

31
Q

explain hereditary fibrinolytic bleeding

A
  • autosomal recessive
  • deficiency in alpha-2-antiplasmin or PAI-1, leaving more plasmin to break down fibrin
  • over expression of plasminogen activator (Quebec platelet disorder)
32
Q

labs in fibrinolytic bleeding

A
  • PT and PTT may be prolonged because fibrin is being broken down
  • hard to distinguish from DIC
  • increased products of fibrin breakdown (d-dimers)
33
Q

treatment for fibrinolytic bleeding

A
  • plasma infusions
  • anti-fibrinolytic drugs (if no DIC)
    • Amicar
    • tranexamic acid
34
Q

what is the most common cause of bleeding due to vitamin K deficiency?

A

over anticoagulation with warfarin

35
Q

causes of vitamin K deficiency

A
  • dietary (malabsorption, alcoholism)
  • medications
  • coumarins (warfarin)
36
Q

labs in vitamin K deficiency

A
  • PT and aPTT prolonged (PT only in early deficiency)
  • PT and aPTT will correct with mixing study
  • platelets normal
  • decreased II, VII, IX, X
  • FACTOR V NORMAL!
37
Q

treatment of vitamin K deficiency

A
  • oral or IV vitamin K (corrects in hours)
  • fresh frozen plasm (only in emergencies, corrects factors immediately, must be used with vitamin K)
  • prothrombin complex concentrates (II, VII, IX, X)
38
Q

what is preserved in liver failure?

A

factor VIII

39
Q

what does liver disease look like for bleeding?

A
  • both bleeders and clotters, but bleeding usually wins out
40
Q

lab findings in coagulopathy of liver disease

A
  • prolonged PT/INR and aPTT
  • low factors in INCLUDING V, but VIII is still preserved
  • PT/INR is unreliable
41
Q

liver disease treatment

A
  • transplant

- vitamin K and fresh frozen plasma can be temporary fixes

42
Q

DIC - major relevant pathology for bleeding

A
  • excessive thrombin, increases fibrin and activates platelets
  • compensatory increase in APC, plasmin, and fibrinolysis
  • increased activation of endothelium, more TF, more coagulation
43
Q

labs in DIC

A
  • prolonged PT/INR and aPTT
  • elevated d-dimers
  • low platelets
  • low fibrinogen
  • anemia
  • RBC schistocytes
  • low AT, APC, S
44
Q

treatment for DIC

A
  • treat underlying cause
  • transfusion support
    plasma (coagulation factors)
    cryoprecipitate (fibrinogen)
    platelets/RBC
  • heparin (only in active thrombosis)

SBM hematology (14 decks)

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