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IHO Week 6 > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

Platelet bleeding

A

Superificial
Petechiae
Spontaneous

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2
Q

Factor bleeding

A

Deep (joints)
Trauma
Big Bleeds

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3
Q

Von Willebrand Disease - must knows

A

Most common hereditary bleeding disorder (NOT factor)
Autosomal dominant
vW factor decreased or abnormal
Variable severity

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4
Q

What is vWF

A

Huge multimeric protein made by megakaryocytes and endothelial cells
Glues platelets to subendothelium
Carries factor VIII

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5
Q

Type 1 vWF

A

Decreased vWF (70% of cases)

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6
Q

Type 2 vWF

A

Abnormal vWF (25% of cases)

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7
Q

Type 3 vWF

A

No vWF (5%)

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8
Q

Symptoms of vWF disease

A

Mucosal bleeding

Deep joint bleeding (severe cases)

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9
Q

Lab tests in vWF disease

A 
Bleeding time: prolonged
PTT: prolonged (corrects with mixing study)
INR: normal
vWF: level decreased (normal in type 2)
Platelet aggregation studies abnormal
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10
Q

What binds vWF?

A

GP Ib (membrane)

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11
Q

Treatment of vWF disease

A

DDAVP (raise VIII and vWF levels)
Cryoprecipitate (contains vWF and VIII)
Factor VIII

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12
Q

Hemophilia A is a ______ genetic disorder

A

X linked

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13
Q

Dads affected with hemophilia A

A

will have carrier daughters

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14
Q

Moms who are carriers of hemophilia A

A

Son may have disease, daugher may carry

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15
Q

Hemophilia A

A

Most common FACTOR deficiency

Factor VIII decreased

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16
Q

Symptoms of Hemophilia A

A

Severity depends on amount of VIII
Typical ‘factor’ bleeding (deep joint bleeding, prolonged bleeding after dental work)
Rarely - mucosal hemhorrhage

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17
Q

Lab tests in hemophilia A

A

INR, TT, Platelet count, bleeding time: NORMAL
PTT: prolonged (corrects with mixing)
Factor VIII assays: abnormal
DNA studies: abnormal

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18
Q

Treatment of Hemophilia A

A

DDAVP

Factor VIII

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19
Q

Hemophilia B

A

Factor IX deficiency
Less common than Hemophilia A
Same inheritance pattern (x linked)
Same clinical and laboratory findings

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20
Q

Factor XI deficiency

A

Bleeding only after trauma (rare)

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21
Q

Factor XIII deficiency

A

severe neonatal bleeding (no cross links, can kill you)

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22
Q

Hereditary platelet disorders (4)

A
  1. Bernard Soulier Syndrome
  2. Glanzmann Thrombasthenia
  3. Gray Platelet Syndrome
  4. Delta Granule Deficiency
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23
Q

Bernard Soulier Syndrome

A

Abnormal Ib (binds vWF)
Abnormal adhesion
Big platelets
Severe Bleeding

24
Q

Glanzmann Thrombasthenia

A

No IIb-IIIa (binds fibrinogen)
No aggregation
Severe bleeding

25
Q

Gray Platelet syndrome

A

No a-granules - why gray
Big, empty platelets
Mild bleeding

26
Q

Delta granule deficiency

A

No d-granules

Can be part of syndrome (Chediak-Higashi)

27
Q

Acquired Bleeding disorders (2)

A
  1. DIC

2. Idiopathic Thrombocyopenic Purpura

28
Q

Disseminated Intravascular Coagulation (DIC)

A

Lots of underlying disorders
Something triggers coagulation, causing thrombosis
Platelets and factors get used up, cause bleeding
Microangiopathic hemolytic anemia

29
Q

Causes of DIC

A

Dumpers and Rippers

30
Q

Dumpers

A

OB complications
Adenocarcinoma
AML-3 (granules are pro coag)

31
Q

Rippers

A

Bacterial sepsis
Trauma
Burns
Vasculitis

32
Q

What are the big 4 for DIC (hint.. MOST)

A

Malignancy
Obstetric Complications
Sepsis
Trauma

33
Q

Symptoms of DIC

A

Can be nsidious or fulminant
Multisystem disease
Thrombosis and or bleeding

34
Q

Lab tests in DIC

A

INR, PTT, TT prolonged
FDPs increased
Fibrinogen decreased

35
Q

Treatment of DIC

A

Treat underlying disorder

Support with blood products

36
Q

Idiopathic Thrombocytopenic Purpura

A

Antiplatelet antibodies
Acute vs chronic
Diagnosis of exclusion
Steroids or splenectomy

37
Q

Pathogenesis of ITP

A

Autoantibodies to GP IIb-IIa or Ib
Bind to platelets
Macrophages eat platelets

38
Q

Two kinds of ITP

A

Chronic or Acute

39
Q

Chronic ITP

A

Adult women
Primary or secondary
Insidious - nosebleeds, easy bruising
Danger - bleeding into brain

40
Q

Acute ITP

A

Children
Abrupt following illness
Usually self limiting
May become chronic

41
Q

Lab tests in ITP

A
  • Signs of platelet destruction : thrombocytopenia, normal/increased megakaryocytes, big platelets
  • INR/PTT normal
  • No specific diagnostic test
42
Q

Thrombocytopenia

A

Caused by many things - aplastic anemia, bone marrow replacement, big speen, consumptive processes (DIC, TTP, HUS), drugs

43
Q

Treatment of ITP

A

glucocorticoids
splenectomy
IV immunoglobulin

44
Q

Thrombotic microangiopathies

A

All have thrombi, thrombocytopenia and MAHA
Include TTP and HUS
Hard to distinguish from TTP from HUS

45
Q

in thrombotic microangiopathies something triggers ______

A

platelet aactivation

46
Q

Thrombotic Thrombocytopenic Purpora

A

Pentad: MAHA, thrombocytopenia, renal failure, fever, neurologic defects

47
Q

TTP deficiency

A

ADAMTS13

Big vWF aren’t cleaved, trap platelets

48
Q

How do deal with TTP

A

plasmapheresis or plasma infusions

49
Q

Brief pathogenesis of TTP

A

Just released vWF is unusually large (UL), causes vWF to aggregate. Usually ADAMTS13 cleaves into less active bits. TTP is deficient in ADAMTS13

50
Q

Clinical findings of TTP

A 
Hematuria, jaundice (MAHA)
Bleeding, brusing (thrombocytopenia)
Fever
Decreased urine output (Renal Failure)
Bizarre behavior (Neurological defects)
51
Q

Treatment of TTP

A

Acquired: plasmapheresis
Hereditary: Plasma infusions

52
Q

Hemolytic Uremic Syndrome

A

MAHA and thrombocytopenia
Epidemic (e coli) vs. non epidemic
Toxin? damages epithelium
Treat supportively

53
Q

Pathogenesis of HUS - epidemic

A

E coli O157:H7 (raw hamburger)
Makes nasty toxin
Injures endothelial cells

54
Q

Pathogenesis of HUS - non epiodemic

A

Defect in complement factor H

Inherited or acquired

55
Q

Clinical findings in HUS - epidemic

A

Children and elderly
Bloodly diarrhea, renal failure
Fatal in 5% of cases

56
Q

Clinical findings in HUS - non epidemic

A

Renal failure
Relapsing remitting course
Fatal in 50% of cases

57
Q

Treatment of HUS

A

Supportive Care
Dialysis
Not antibiotics (increase toxin release)

IHO Week 6 (7 decks)

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