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Heme/Lymph 2 CP > 10b- Intro to Hemostasis- Bleeding Disorders > Flashcards

10b- Intro to Hemostasis- Bleeding Disorders Flashcards

1
Q

What are 5 defects in primary hemostasis?

A
  • skin and mucosal membrane hemorrhages are common (eg petechiae, epistaxis, menorrhagia)
  • Vascular abnormalities (eg Ehlers-Danlos)
  • Platelet function disorders
  • von Willebrand disease
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2
Q

What are 3 defects in secondary hemostasis?

A
  • bleeds into soft tissues or joint
  • clotting factor deficiencies
    • hemophilia
    • liver disease
  • exogenous
    • anticoagulants
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3
Q

What is thrombocytopenia? What 4 things are also on the differential diagnosis?

A
  • usually defined as <100,000/uL
  • Differential
    • decreased bone marrow: aplastic anemia
    • increased destruction (immune mediated, non-immune mediated)
    • dilutional: decreased platelets bc increased fluid
    • sequestration: big spleen harbors platelets
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4
Q

Thrombocytopenia is decreased platelets due to decreased _______

What 7 things cause thrombocytopenia?

A

Thrombocytopenia is decreased platelet production due to decreased megakaryocytes

  • aplastic anemia
  • vitamin B12/folate deficiency
  • leukemia, lymphoma, MDS
  • metastatic carcinoma
  • alcohol, toxins
  • drugs
  • infections
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5
Q

Thrombocytopenia caused by increased destruction can be immune mediated or non-immune mediated? What are 4 examples of immune mediated thrombocytopenia?

A

immune thrombocytopenia purpura (ITP)

Heparin-induced thrombocytopenia (HIT)

Transfusion/preganncy-associated allo-immune thrombocytopenia

drug (quinine, vancomycin)

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6
Q

What are 4 non-immune mediated causes of thrombocytopenia?

A

Disseminated intravascular coagulation (DIC)

Thrombotic thrombocytopenic purpura (TTP)

Hemolytic uremic syndrome (HUS)

Drug

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7
Q

WHat is Immunt Thrombocytopenia Purpura (ITP)

A

Caused by autoantibodies made against PLT antigens (GpIIb/IIIa, GpIb)

destruction of PLTs occurs by phagocytosis, Fc receptor mediated, loction is the spleen

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8
Q

What are the 2 categories of immune thrombocytopenia purpura (ITP)

A
  • Primary: unknown etiologies
  • Secondary: diseases we can identify
    • lupus
    • leukemia/lymphoma (eg CLL/SLL)
    • Drugs
    • Viruses (eg HIV, Hepatitis)
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9
Q

What are the clinical features of Immune Thrombocytopenia Purpura (ITP)? WHat are the 2 variant/the difference between them?

A
  • skin, mucosal bleeds
  • normal size spleen
  • variants:
    • Acute: self limited (< 6 months, post-viral, children)
    • Chronic: adults (females), persistent>6 months
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10
Q

What are the pathologic findings of ITP?

A

Thrombocytopenia (often quite low), large platelets (circled)

normal to increased megakaryocytes in marrow

white pulp expansion

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11
Q

How do we diagnose ITP?

A

presumptive

iagnosis of exclusion

no diagnostic test

normal clotting tests

bone marrow not required

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12
Q

How do we treat ITP?

A
  • May not be needed in children with self-limited disease
  • immunosuppresant therapy
    • steroids, intravenous immunoglobin (IVIG)
    • Anti-CD20 antibody
  • Thrombopoietin agonist
  • Splenectomy
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13
Q

What happens in drug-induced thrombocytopenia?

A

drug binds to IIa/IIIb to elicit antibody reaction.Phagocytic cells then localize to the antibody and destroy the platelet

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14
Q

What is dilutional thrombocytopenia? Sequestration thrombocytopenia?

A

Dilution: Massive transfusion of trauma patients

Sequestration: Seen with hypersplenism, increased storage fo Platelelets

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15
Q

What are platelet function disorders? List 5

A

heterogenous group of inherites and acquired, qulitative disorders with variable clinical presentation

Bernard Soullier

Glanzmann’s thrombasthenia

Storage pool disease

Aspirin

Uremia

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16
Q

What is the pathophysiology of Bernard Soullier?

Inherited vs acquired

Clinically severe or variable?

A

Gp1b deficiency on platelet memebrane

inherited

severe

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17
Q

What s the pathophysiology of Glanzmann’s thrombasthenia?

Inherited or acquired

severe or variable

A

GpIIb/IIIa deficiency on platelet memebrane

inherited

severe

18
Q

What is the pathophysiology of storage pool disease?

inherited or acquired

clinically severe or variably

A

granule, transit defect

inherited

variable

19
Q

What is the pahtology of aspirin platelet dysfunction? inherited or acquired? clinically sever or variable?

A

Cox inhibition

acquired

variable

20
Q

What is the most common inherited bleeding disorder? How is it inherited and how does that relate to severity of disease?

A

vonWillebrand disease

  • usually autosomal dominant, incidence may be underestimated given mild presentation. rarely acquired
  • clinically heterozygous
    • mild, revealed with surgery, dental procedure, trauma
    • moderate to severe, menorrhagia, spontaneous bleeding
    • dependent on clinical type
21
Q

Where is vW factor produced? What is the function? What is the structure of vWF

A
  • produced by endothelial cells, megakaryocytes/PLTs
  • Function:
    • When circulating: stabilize F8 (T1/2=2.4 hrs free to 12 hours bound)
    • Fixed: adhere to collagen and platelets through Gp1b
  • Structure: multimers-metalloproteases cleave freshly cut vWF into multimers. there should be equal amount of all of the multimers
22
Q

What are the 3 types of vWD? What are the characteristics of each of them?

A
  1. Type 1: Quantitative: most common A/D
    • clinically heterogenous usually mild (how much factor they have determines how severe the disease is)
  2. Type 2: Qualitative
    • 2A, 2B, 2M, 2N is A/R; others are usually A/D
    • moderate to severe bleeding (They make vWF but it is dysfunctional)
  3. Complete absence (Quantitative)
    • Rare, A/R
    • severe bleeding disorder, mimichking heterophilia
23
Q

How do we treat vWD?

A

desmopressin stimulates vWF release

factor concentrates

anti-fibrinolytics

oral concentration (to stop menorrhagia)

24
Q

What are the laboratory tests used to screen for vWD and what would we expect the results to be? What are the confirmatory studies? (sorry this is a long card)

A
  • Screen
    • CBC (normal PLT count)
    • PT (normal), aPTT (normal or prolonged)
    • vWF antigen levels
    • vWF activity (ristocetin)
    • Factor 8 activity
  • Confirmatory studies
    • multimer electrophoresis
    • specific biinding assays
    • platetlet aggregation
25
Q

In type 1, type 2 and type 3 vWD what would we expect for the vW antigen, vW activity, F8 activity?

A

Type 1: vW antigen decreased, vW activity normal or decreased, F8 activity Normal

Type 2: vW antigen normal, vW activity decreased, F8 activity normal or decreased

Type 3: vW antigen none, vW activity none, F8 activity very decreased

26
Q

What are 2 types of defects in secondary hemostasis?

A

Single clotting factors: hemophilia

Multiple clotting factors: liver disease, Vitamin K deficiency, exogenous: anticoagulants

**these are what Goljian calls late bleeding disorders**

27
Q

How is Hemophilia inherited?

A
  • X-linked recessive for hemophilia A, B
    • mostly males, females are carriers
    • 30% are sporadic new mutations
28
Q

What are the different types of hemophilia?

A
  • Hemophilia A- F8 deficiency, most common inherited disease associated with life threatening bleeds
  • Hemophilia B- F9 deficiency
  • Hemophilia C- F11 deficiency
    • A/R inheritance
    • not predicatable bleeding
29
Q

What is the clinical presentation of Hemophilia A,B

A
  • Spontaneous bleeds
    • hemarthrosis ledingin to progressive deformity
    • deep muscle bleeds
  • Easy bruising with trauma
  • Bleeding tracks with factor levels
30
Q

What are the results of the lab tests that are done for evaluation of Hemophilia?

A
  • aPTT prolonged, PT normal
  • aPTT mixing study shows correction
  • factor levels/activities relate to severity
    • severe= <1% F8 or F9
    • moderate= 1-5% F8 or F9
    • Mild= 6-50% F8 or F9
31
Q

A deficiency in what factor is asymptomatic? What are the clinical manifestations and associated conditions of deficiencies in factor 1 (fibrinogen), 2, 5, 7, 10, 12, 13?

A

Factor 12 is asymptomatic and all the rest are associated with bleeding.

5=exposure to topical bovine thrombin

10= Amyloid

13= Poor wound healing infertility in men

32
Q

inhibitors (autoantibodies) against any clotting factor can develop and cause a deficiency in the setting of _______, ______ and ________

A

Inhibitors (auto-antibodies) against any clotting factor can develop and cause a deficieicny in the setting of autoimmune disorders, medications, and malignancies

33
Q

How can liver disease cause coagulopathy?

A
  • Deficiency of mulitple coagulation factors:
    • procoagulants: factors 2, 5, 7, 9, 10
    • anticoagulants: antithrombin III, Protein C, Protein S
  • Thrombocytopenia: hypersplenism (in pts w cirrhosis-associated splenomegaly), decreased thombopoietin production
  • Vitamin K deficiency
  • Fibrinogen abnormalities (low fibrinogen, dysfibrinogenemia-dysfunctional protein)
  • Therapy of coagulation disorder
34
Q

What is the therapy for coagulation disroders from liver failure?

A
  • Treatment is not required if patient is not bleeing
  • If bleeding
    • Vtamin K replacement (rarely corrects PT)
    • Replacement: plasmsa, cryoprecipitate, platelets
    • Therapy specific for bleed site (ex: banding varicies)
35
Q

When do we see coagulopathy of liver disease?

A
  • Acute liver injury: Hepatitis, toxin (acetominophen, alcohol)
  • Chronic liver disease: Cirrhosis
36
Q

What do the labs show during evaluation of coagulopathy due to liver disease?

A

Prolonged (PT, PTT, TT)

Low fibrinogen activity

Normal or decreased platelets

low factor activities except F8, vWF

37
Q

How is vitamin K related to coagulation? What things cause VItamin K deficiency?

A
  • Dependent factors: F2, F7, F9, F10, Protein C and S
  • Cofactor for gamma carboxylation
  • provided by diet and bacterial flora
38
Q

What causes Vitamin K deficiency and what is the treatment?

A
  • Deficiency caused by:
    • Drugs (warfarin, antibiotics bc they decrease bowel flora)
    • malabsorption or dietray deficiency
    • liver disease
    • new borns
  • Treatment:
    • Vitamin K replacement
    • Fresh frozen plasma for emergencies
    • Newborns receive injection at birth
39
Q

What are the lab results of vitamin k deficiency?

A

prolonged PT, aPTT, decreased protein C, S

40
Q

What clotting factors does Warfarin inhibit? How about Heparin? What are 2 direct thrombin inhibitors? Factor X inhibitors?

A

Warfarin: F2, f7, F9, F10, protein C and S

Heparin: F2, F9, F10, accelerates AT3 activity

Direct thrombin inhibitors: F2

Factor X inhibitors: F10

41
Q

How do we measure anticoagulation activity?

A

Warfarin: INR

Heparin: PTT/anti- Xa assay

Some DTIs: PTT

42
Q

What is the bleeding mechanism of platelet granule deficiency?

Hemophilia A

Hemophilia B

Hemophilia C

Liver disease

Vitamin K deficiency/warfarin

A

platelet granule deficiency: platelet dysfunction

Hemophilia A: Factor 8 deficiency

Hemophilia B: Factor 9 deficiency

Hemophilia C: Factor 11 deficiency

Liver disease: Factor deficiencies or dysfunction

Vitamin K deficiency/warfarin: Deficiency factors 2, 7, 9, 10

Heme/Lymph 2 CP (16 decks)

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