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IHO Week 6 (EH) > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

Clinical features platelet bleeding

A

Superficial (skin)
Petechiae (bigger areas = purpura)
Spontaneous

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2
Q

Clinical features factor bleeding

A

Deep (joints)
Big bleeds
Trauma

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3
Q

Von Willebrand Disease TYMK

A

Most common hereditary bleeding disorder
Autosomal dominant
vW factor decreased (or abnormal)
Variable severity

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4
Q

most common hereditary bleeding disorder

A

VW disease

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5
Q

most common factor disorder

A

hemophilia (VW is not considered a factor disorder)

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6
Q

inheritance VWD

A

Autosomal dominant

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7
Q

von willebrand factor

A

Huge multimeric protein

Decreased or abnormal in vW disease

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8
Q

von willebrand factor function

A

Glues platelets to subendothelium

Carries factor VIII

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9
Q

von willebrand factor made by

A

Made by megs and endothelial cells

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10
Q

von willebrand disease is a platelet/factor disorder

A

platelet - but if really bad, because carries factor VIII, can look like a factor disorder as well

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11
Q

Types of vW disease

A 
Type 1 (70%): Decreased vWF
Type 2 (25%): abnormal vWF
Type3 (5%): no vWF
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12
Q

symptoms of vW disease

A

Mucosal bleeding in most patients

Deep joint bleeding in severe cases

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13
Q

Lab Tests in Von Willebrand Disease

A 
Bleeding time: prolonged
PTT: prolonged (severe) (“corrects” with mixing study) 
INR: normal
vWF level decreased (normal in type 2)
Platelet aggregation studies abnormal
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14
Q

What binds to vWF

A

GP Ib on membrane of platelet (glycoprotein)

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15
Q

what doesn’t wF disease work with in platelet aggregation test?

A

Ristocetin

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16
Q

treatment of vW disease

A

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

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17
Q

Hemophilia A TYMK

A 
Most common factor deficiency
X-linked recessive in most cases 
    (30% are random mutations)
Factor VIII level decreased
Variable amount of “factor” bleeding
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18
Q

Most common factor deficiency

A

Hem A

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19
Q

Hem A inheritance

A

X-linked recessive

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20
Q

what factor is decreased in Hem a

A

Factor VIII

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21
Q

Symptoms Hem A

A 
Severity depends on amount of VIII
Typical “factor” bleeding
deep joint bleeding
prolonged bleeding after dental work
Rarely, mucosal hemorrhage
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22
Q

Lab Tests in Hem A

A

INR, TT, platelet count, bleeding time: normal (these are platelet issues, hem is a fibrin issue)
PTT: prolonged (“corrects” with mixing study)
Factor VIII assays: abnormal
DNA studies: abnormal

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23
Q

Treatment Hem A

A

DDAVP (release stores of vWF and VIII)

Factor VIII

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24
Q

Hem B TYMK

A

Factor IX level decreased
Much less common than hemophilia A
Same inheritance pattern
Same clinical and laboratory findings

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25
Q

what factor decreased in Hem B

A

Factor IX

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26
Q

Inheritance pattern Hem B

A

x-linked recessive (30% lab findings)

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27
Q

clinical findings Hem B

A 
Severity depends on amount of VIII
Typical “factor” bleeding
deep joint bleeding
prolonged bleeding after dental work
Rarely, mucosal hemorrhage
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28
Q

lab tests Hem B

A

INR, TT, platelet count, bleeding time: normal (these are platelet issues, hem is a fibrin issue)
PTT: prolonged (“corrects” with mixing study)
Factor VIII assays: abnormal
DNA studies: abnormal

29
Q

XI deficiency

A

bleeding only after trauma

30
Q

XIII deficiency

A

severe neonatal bleeding

31
Q

Bernard-Soulier Syndrome

A

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

32
Q

Glanzmann Thrombasthenia

A

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

33
Q

Gray Platelet Syndrome

A

No alpha granules
Big, empty platelets
Mild bleeding

34
Q

Delta granule deficiency

A

No delta granules

Can be part of syndrome (e.g., Chediak-Higashi)

35
Q

alpha granules

A

fibrinogen, vWF

36
Q

delta granules

A

serotonin, ADP, ca2+

37
Q

DIC TYMK

A

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

38
Q

Causes of DIC can be separated into

A

dumpers (into vascular system to set off cascade)

rippers (rip up endothelium)

39
Q

DIC causes: Dumpers

A

Obstetric complications
Adenocarcinoma
Acute promyelocytic leukemia

40
Q

DIC causes: Rippers

A

Bacterial sepsis
Trauma
Burns
Vasculitis

41
Q

Causes of DIC to remember

A 
Malignancy
OB complications
Sepsis
Trauma
(MOST)
42
Q

Symptoms of DIC

A

Insidious or fulminant
Multi-system disease
Thrombosis and/or bleeding

43
Q

Lab Tests in DIC

A

INR, PTT, TT prolonged
FDPs: increased
Fibrinogen: decreased

44
Q

Treatment of DIC

A

Treat underlying disorder

Support with blood products

45
Q

Idiopathic thrombocytopenia Purpura TYMK

A 
Antiplatelet antibodies
Acute vs. chronic 
Diagnosis of exclusion
Steroids or splenectomy
Remember how different this is from DIC
46
Q

Pathogenesis of ITP

A

Autoantibodies to platelets = GP IIb-IIIa or Ib
Bind to platelets (yummy!)
Splenic macrophages eat platelets

47
Q

two kinds of itp

A

chronic

acute

48
Q

chronic ITP

A

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

49
Q

acute ITP

A

Children
Abrupt; follows viral illness
Usually self-limiting
May become chronic

50
Q

Lab tests ITP

A 
Signs of platelet destruction:
- thrombocytopenia
- normal/increased megakaryocytes
- big platelets 
INR/PTT normal
No specific (ab) diagnostic test for ITP
51
Q

Other causes of thrombocytopenia

A 
Aplastic anemia
Bone marrow replacement
Big spleen
Consumptive processes (DIC, TTP, HUS)
Drugs
52
Q

tx of ITP

A

Glucocorticoids
Splenectomy
Intravenous immunoglobulin

53
Q

Thrombotic microangiopathies all have

A

thrombi, thrombocytopenia, and MAH

54
Q

Thrombotic microangiopathies include

A

TTP and HUS (can be hard to distinguish)

Different from DIC

55
Q

thrombotic microangiopathies = something triggers _______

A

platelet activation

56
Q

Thrombotic thrombocytopenic purpura TYMK

A

Pentad: MAHA, thrombocytopenia, fever, neurologic defects, renal failure
Deficiency of ADAMTS13
Big vWF multimers trap platelets
Plasmapheresis or plasma infusions

57
Q

pentad TTP

A

MAHA, thrombocytopenia, fever, neurologic defects, renal failure

58
Q

TTP deficiency in

A

ADAMTS13

59
Q

Pathogenesis TTP

A

Just-released vWF is unusually large (UL)
UL vWF causes platelet aggregation
ADAMTS13 cleaves UL vWF into less active bits!
TTP is due to ADAMTS13 deficiency

60
Q

Clinical findings in TTP

A 
Hematuria, jaundice (MAHA)
Bleeding, bruising (thrombocytopenia)
Fever
Bizarre behavior (neurologic deficits)
Decreased urine output (renal failure)
61
Q

Treatment of TTP

A

Acquired TTP: plasmapheresis

Hereditary TTP: plasma infusions

62
Q

Hemolytic Uremic Syndrome TYMK

A

MAHA and thrombocytopenia
Epidemic (E. coli) vs. non-epidemic
Toxin (or ?) damages endothelium
Treat supportively

63
Q

Pathogenesis of HUS has 2 main causes

A

Epidemic

Non-epidemic

64
Q

HUS epidemic pathogenesis

A

E. coli O157:H7 (raw hamburger)
Makes nasty toxin (shiga or shiga-like)
Injures endothelial cells

65
Q

HUS non-epidemic pathogenesis

A

Defect in complement factor H (protective)
Inherited or acquired
How does this activate platelets?

66
Q

HUS epidemic associated with usually

A

E. coli O157:H7

67
Q

Clinical findings in HUS epidemic

A

Children, elderly
Bloody diarrhea, then renal failure
Dont give Abx (expose toxin)
Fatal in 5% of cases

68
Q

Clinical findings in HUS non-epidemic

A

Renal failure
Relapsing-remitting course
Fatal in 50% of cases

69
Q

Treatment HUS

A

Supportive care
Dialysis
NOT antibiotics (may increase toxin release!)

IHO Week 6 (EH) (9 decks)

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