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Steve IHO week 6 > Krafts- Friday Bleeding Disorders > Flashcards

Krafts- Friday Bleeding Disorders Flashcards

1
Q

Platelet bleeding: Clinical Presentation

A

Superficial (skin)
Petechiae
Spontaneous
often from mucosal membranes (eg. nose bleed)

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2
Q

Factor bleeding: Clinical Presentation

A

Deep (joints)
Big bleeds
Trauma

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3
Q

Petechiae

A

spotted red dots

blood in the skin outside the arteries and veins

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4
Q

Purpura

A

the combination of a lot of petechiae into one are so it looks like a continuous bleed

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5
Q

von Willebrand disease: Things you must know

A

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

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6
Q

What’s von Willebrand Factor?

A 
Huge multimeric protein
Made by megakaryocytes and endothelial cells
*Glues platelets to endothelium*
Carries factor VIII
Decreased or abnormal in vW disease
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7
Q

Types of Von Willebrand Disease

A 
Type 1 (70%): decreased vWF
Type 2 (25%):  abnormal vWF
Type 3 (5%):  no vWF
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8
Q

Symptoms of Von Willebrand Disease

A

Mucosal bleeding in most patients

Deep joint bleeding in severe cases

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9
Q

Lab Tests in Von Willebrand 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

GP Ib binds what

A

binds vWF

ristocetin –>

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11
Q

ristocetin

A

is an old antibiotic that makes you express high levels of GP Ib from your platelets.
Helps dx vWF disease because this will soak up the little vWF that the patient has in their blood

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12
Q

Treatment of Von Willebrand Disease

A

DDAVP (raises VIII and vWF levels) but you need to already be making some (won’t work for type III)

Cryoprecipitate (contains vWF and VIII)

Factor VIII

but try to avoid giving anything due to bleeding issues, these are if the patient isn’t doing well

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13
Q

Hemophilia A: Things you must know

A

Most common factor deficiency

X-linked recessive in most cases (30% are spontaneous mutations)

Factor VIII level decreased

Variable amount of “factor” bleeding

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14
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 hemorrhage

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15
Q

Lab Tests in Hemophilia A

A

INR, TT, platelet count, bleeding time: normal
PTT: prolonged (“corrects” with mixing study)
Factor VIII assays: abnormal
DNA studies: abnormal

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16
Q

Treatment of Hemophilia A

A

DDAVP

Factor VIII

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17
Q

Hemophilia B: Things you must know

A

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

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18
Q

Factor XI deficiency

A

bleeding only after trauma

super rare

19
Q

Factor XIII deficiency:

A

severe neonatal bleeding

super rare

20
Q

Bernard-Soulier Syndrome

A

Abnormal factor Ib
Abnormal adhesion
Big platelets
Severe bleeding

21
Q

Glanzmann Thrombasthenia

A

No factor IIb-IIIa
No aggregation
Severe bleeding

22
Q

Gray Platelet Syndrome

A

No alpha granules
Big, empty platelets
Mild bleeding

23
Q

Delta Granule Deficiency

A

No Delta granules (never would have guessed)

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

24
Q

Disseminated Intravascular Coagulation: Thinks you must know

A

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

25
Q

Causes of DIC

A

Dumpers: *Obstetric complications, Adenocarcinoma, Acute promyelocytic leukemia

Rippers: *Bacterial sepsis, *Trauma, Burns, Vasculitis

26
Q

Symptoms of DIC

A

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

27
Q

Lab Tests in DIC

A

INR, PTT, TT prolonged
FDPs: increased
Fibrinogen: decreased
*All of them have to be this way, no one Dx test

28
Q

Treatment of DIC

A

Treat underlying disorder

Support with blood products

29
Q

Idiopathic Thrombocytopenic Purpura: Things you must know

A

Antiplatelet antibodies
Acute vs. chronic
Diagnosis of exclusion
Steroids or splenectomy

30
Q

Pathogenesis of ITP

A

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

31
Q

Two Kinds of ITP: Tell me about chronic type

A 
Chronic
Adult women (usually young adult women)
Primary or secondary
Insidious: nosebleeds, easy bruising
Danger: bleeding into brain
32
Q

Two Kinds of ITP: Tell me about acute type

A 
Acute
Children
Abrupt; follows viral illness
Usually self-limiting
May become chronic
33
Q

Lab Tests in ITP

A

Signs of platelet destruction:
thrombocytopenia
normal/increased megakaryocytes
big platelets

INR/PTT normal
No specific diagnostic test for ITP

34
Q

Other Causes of Thrombocytopenia besides ITP

A 
Aplastic anemia
Bone marrow replacement
Big spleen
Consumptive processes (DIC, TTP, HUS)
Drugs
35
Q

Treatment of ITP

A

Glucocorticoids
Intravenous immunoglobulin
Splenectomy (site of destruction so it helps)

36
Q

Thrombotic Microangiopathies

A 
All have thrombi, thrombocytopenia, and MAHA
Include TTP and HUS
Can be hard to distinguish TTP from HUS
Something triggers platelet activation
Different from DIC!
37
Q

Thrombotic Thrombocytopenic Purpura: things to know

A

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

38
Q

Pathogenesis of 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

39
Q

Clinical Findings in TTP

A 
Hematuria, jaundice (MAHA)
Bleeding, bruising (thrombocytopenia)
Fever
Bizarre behavior (neurologic deficits)
Decreased urine output (renal failure)
40
Q

Treatment of TTP

A

Acquired TTP: plasmapheresis

Hereditary TTP: plasma infusions

41
Q

Hemolytic Uremic Syndrome: things you must know

A

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

42
Q

Pathogenesis of HUS

A

Epidemic (more common)
E. coli O157:H7 (raw hamburger)
Makes nasty toxin
Injures endothelial cells

Non-epidemic (rare)
Defect in complement factor H
Inherited or acquired

43
Q

Clinical Findings in HUS

A

Epidemic:
Children, elderly
Bloody diarrhea, then renal failure
Fatal in 5% of cases

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

44
Q

Treatment of HUS

A

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

Steve IHO week 6 (3 decks)

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