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IHO Week 6 (Final) > Bleeding Disorders - Krafts > Flashcards

Bleeding Disorders - Krafts Flashcards

1
Q

What are the Hereditary Bleeding Disorders?

A

von Willebrrand Disease
Hemophilia A and B
Hereditary Platelet Disorder

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2
Q

What are the Acquired Bleeding Disorders?

A

DIC
ITP
TTP/HUS

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3
Q

What factors are seen when the disorder is due to platelet bleeding?

A

Superficial (skin)
Petechiae
Spontaneous - no accident

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4
Q

What factors are seen when the disorder is due to factor bleeding?

A

Deep (joints)
Big Bleeds
Trauma

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5
Q

What are the characteristics of von Willebrand Disease?

A

Most common hereditary bleeding
Autosomal Dominant
von Willebrand factor decreased
Variable severity

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6
Q

What is the clinical finding of Type 1 Von Willebrand Disease?

A

Decreased vWF

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7
Q

What is the clinical finding of Type 2 Von Willebrand Disease?

A

Abnormal vWF

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8
Q

What is the clinical finding of Type 3 Von Willebrand Disease?

A

No vWF

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9
Q

What is von Willebrand Factor?

A

Huge multimeric protein made by Megakaryocytes and Endothelial cells
Glues platelets to endothelium

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10
Q

What factor does von Willebrand Factor carry?

A

Factor VIII (Intrinsic Pathway)

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11
Q

What are the symptoms of von Willebrand Disease?

A 
Most patients are asymptomatic
Mucosal bleeding (most)
Deep joint bleeding (severe)
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12
Q

What lab tests would be checked and what would the values be in von Willebrand Disease?

A 
Bleeding Time = prolonged
PTT = prolonged 
Mixing Study = corrected
INR = Normal
vWF level = decreased (except in Type 2)
Platelet aggregation = abnormal
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13
Q

What membrane bound proteins are in the platelet?

A

Phospholipids activate coag factors
GP Ia binds collagen
GP Ib binds vWF
GP IIb-IIIa binds fibrinogen

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14
Q

What makes platelets express the GP Ib membrane bound protein?

A

Ristocetin (does not work in vWD = missing vWF)

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15
Q

What is the treatment for Von Willebrand Disease?

A

DDAVP = raises factor VIII and vWF levels
Cryoprecipitate = contains vWF and VIII
Factor VIII

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16
Q

What are the genetic characteristics of Hemophilia inheritance?

A

X-Linked Recessive Disorder

~30% are random mutations

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17
Q

What are the characteristics of Hemophilia A?

A

Most common factor deficiency

Factor VIII level decreased

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18
Q

What are the symptoms of Hemophilia A?

A

Typical “Factor” Bleeding with Deep Joint bleeding or prolonged bleeding after dental work (preceding trauma)
Rarely = Mucosal Hemorrhage or Petechiae
Severity depends on amount of VIII

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19
Q

What lab tests are used to diagnose Hemophilia A and what are the expected values?

A

Normal: INR, TT, Platelet Count, Bleeding Time
PTT = prolonged
Mixing Study = corrected
Factor VIII assay and DNA studies = abnormal

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20
Q

What is the treatment for Hemophilia A?

A

DDAVP

Factor VIII

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21
Q

What are the characteristics of Hemophilia B?

A

Factor IX decreased
Less common than Hemophilia A
X-Linked Recessive Disorder
Same clinical and lab findings

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22
Q

What happens with a factor XI deficiency?

A

Bleeding only after trauma

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23
Q

What happens with a factor XIII deficiency?

A

Severe neonatal bleeding

24
Q

What are the Hereditary Platelet Disorders?

A

Bernard-Soulier Syndrome
Glanzmann Thrombasthenia
Gray Platelet Syndrome
Delta-Granule Deficiency

25
Q

What are the characteristics of Bernard-Soulier Syndrome?

A

Abnormal GP Ib
Abnormal adhesion
Big platelets
Severe bleeding

26
Q

What are the characteristics for Glanzmann Thrombasthenia?

A

No GP IIb-IIIa fibrinogen receptor
No aggregation
Severe bleeding

27
Q

What are the characteristics for Gray Platelet Syndrome?

A

No alpha granules
Empty, Big platelets
Mild bleeding

28
Q

What are the characteristics for delta-Granule Deficiency?

A

No delta granules

Associated with Chediak-Higashi syndrome

29
Q

What are the characteristics of Disseminated Intravascular Coagulation (DIC)?

A

Something triggers coagulation causing thrombosis

Platelets and factors get used up causing bleeding

30
Q

What is the major issue in DIC?

A

Microangiopathic Hemolytic Anemia

31
Q

What are the “Dumper” causes of DIC?

A

Dumper = coagulation substances are dumped into the blood
Obstetric complications
Adenocarcinoma
Acute Promyelocytic Leukemia

32
Q

What are the “Ripper” causes of DIC?

A 
Ripper = Anything that will damage the endothelium
Bacterial Sepsis
Trauma
Burns
Vasculitis
33
Q

What are the top 4 causes of DIC?

A 
MOST common causes of DIC:
Malignancy
Obstetric Complications
Sepsis
Trauma
34
Q

What are the symptoms of DIC?

A

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

35
Q

What lab tests are used to diagnose DIC and what are the expected values?

A

Prolonged INR, PTT, TT
Increased FDPs
Decreased Fibrinogen

36
Q

What is the treatment of DIC?

A

Treat underlying disorder while supporting with blood products

37
Q

What are the characteristics of Idiopathic Throbocytopenic Purpura?

A

Antiplatelet Antibodies
Acute vs. Chronic
Diagnosis of Exclusion

38
Q

What is the treatment of Idiopathic Thrombocytopenic Purpura?

A

Steroids (Glucocorticoids)
IVIG
Splenectomy

39
Q

What is the pathogenesis of Idiopathic Thrombocytopenic Purpura?

A

Autoantibodies to GP IIb-IIIa or GP Ib
Binds to Platelets
Splenic macrophages eat platelets

40
Q

What are the characteristics of Chronic Idiopathic Thrombocytopenic Purpura?

A

Primary or Secondary
Adult Women
Insidious: Nosebleeds, Easy Bruising
DANGER: Bleeding in Brain

41
Q

What are the characteristics of Acute Idiopathic Thrombocytopenic Purpura?

A

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

42
Q

What lab tests are used to diagnose Idiopathic Thrombocytopenic Purpura and what are the expected values?

A 
Normal INR/PTT
Signs of platelet destruction:
-Thrombocytopenia
-Normal/Increased megakaryocytes
-Big Platelets
43
Q

What are some other causes of Throbocytopenia besides ITP?

A 
Aplastic anemia
Bone marrow replacement
Large spleen
Consumptive processes (DIC, TTP, HUS)
Drugs
44
Q

What are the characteristics of Thrombotic Microangiopathies (TTP and HUS)?

A

Thrombi, Throbocytopenia, AND MAHA

Something triggers Platelet Activation

45
Q

What are the symptoms of Thrombotic Thrombocytopenic Purpura (TTP)?

A

MAHA, Thrombocytopenia, Fever, Neurologic Defects, Renal Failure

46
Q

What is the deficiency associated with TTP?

A

ADAMTS13

47
Q

What is the treatment of TTP?

A 
Plasmapheresis (Acquired TTP)
Plasma infusions (Hereditary TTP)
48
Q

What is the pathogenesis of TTP?

A

Big vWF multimers that trap platelets

49
Q

What is the normal function of ADAMTS13?

A

To cleave unusually large vWF into less active bits

50
Q

What are the clinical findings of TTP?

A 
Hematuria, Jaundice (MAHA)
Bleeding, Bruising (Thrombocytopenia)
Fever
Bizarre Behavior (Neurologic Deficits)
Decreased Urine Output (Renal Failure)
51
Q

What is the most common pathogenesis of Hemolytic Uremic Syndrome (HUS)?

A

Epidemic usually caused by E. coli toxin-induced injury to endothelial cells

52
Q

What are the characteristics of HUS?

A

MAHA and Thrombocytopenia

Toxin that damages endothelium

53
Q

What is the pathogenesis of Non-Epidemic HUS?

A

Defect in complement factor H

Inherited or Acquired

54
Q

What are the clinical findings of Epidemic HUS?

A

Children/Elderly

Bloody diarrhea, then renal failure

55
Q

What are the clinical findings of Non-Epidemic HUS?

A

Renal Failure
Relapsing-Remitting Course
Fatal in 50% of cases

56
Q

What is the treatment of HUS?

A

Supportive Care

Dialysis

IHO Week 6 (Final) (11 decks)

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