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Heme/Onc > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

Which pathway does PT represent?

A

Extrinsic pathway (warfarin)

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2
Q

Which pathway does PTT represent?

A

Intrinsic pathway (heparin)

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3
Q

What is an INR?

A

International normalized ratio
It is a standardized PT so that you can compare values between labs

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4
Q

What is hemophilia A caused by?

A

Congenital Factor VIII deficiency

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5
Q

What is hemophilia B caused by?

A

Congenital Factor XI deficiency

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6
Q

What is the presentation of hemophilia?

A

Recurrent hemarthroses and easy bruising and bleeding

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7
Q

What is the most common x-linked disease?

A

Hemophilia A

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8
Q

What type of inheritance is hemophilia?

A

X linked recessive

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9
Q

What is a possible progression of hemophilia?

A

Development of inhibitors to clotting factors that they are already deficient in

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10
Q

What laboratory findings support a hemophilia diagnois?

A

Low factor level (III or XI)
aPTT is prolonged
PT is normal
CBC is normal

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11
Q

What is the severity of hemophilia determined by?

A

The level of factor activity

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12
Q

What can hemophiliacs take for pain?

A

Celebrex (COX-2 selective), opioids
Avoid NSAIDS

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13
Q

What is the prognosis of hemophilia?

A

Generally full lives if managed well
Deaths typically due to HIV and hepatitis
At risk of ICH

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14
Q

What patient education is necessary for hemophilia?

A

Avoid contact sports

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15
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand Disease

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16
Q

What is the function of vWF?

A

Bridges platelets and tethers them to a site of vascular injury

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17
Q

What is T1 vWD?

A

Most common type
Not enough vWF
Autosomal dominant
None to severe disease

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18
Q

What is T2 vWD?

A

Second most common type
Due to dysfunctional vWF
Autosomal dom or recessive
Mod to severe bleeding

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19
Q

What is T3 vWD?

A

Least common type
Absence of vWF
Autosomal recessive
Severe manifestations (looks similar to Hemophilia A

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20
Q

What are the most common signs of von Willebrand disease?

A

Nosebleeds and hematomas

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21
Q

What are common laboratory findings to support a vWD diagnosis?

A

Bleeding time is prolonged or normal (T1)
aPPT is mildly prolonged or normal (2/2 low FVIII)
PT is normal

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22
Q

What affects vWF levels?

A

Physiologic stress
ABO blood type
->Can be intermittently normal in vWD

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23
Q

What is the treatment for vWD?

A

Desmopressin (DDAVP)
rVWF
vWF/FVIII concentrates

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24
Q

What is factor XI deficiency?

A

Autosomal recessive disease common in ashkenazi jewish decent
known as hemophilia C

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25
Q

What is the presentation of factor IX deficiency?

A

Mild bleeding

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26
Q

What is the treatment for factor XI deficiency?

A

Factor XI concentrate for bleeding
Can also use FFP

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27
Q

What is thrombocytopenia?

A

Too few platelets so cannot form a primary plug or secondary clot

28
Q

What are the symptoms of thrombocytopenia?

A

Epistaxis, gum bleeding, GI bleeds

29
Q

What are the causes of thrombocytopenia?

A

Decreased production of platelets
Increased sequestration of platelets
Pregnancy -> volume overload

30
Q

What are the causes of destructive or consumption thrombocytopenia?

A

Spenomegaly
Antibodies (ITP)
Drugs
Massive bleeds
Thrombus formation

31
Q

How does splenomegaly cause thrombocytopenia?

A

Spleen has upregulated clearance of platelets. Caused by liver disease or malignancy. Must remove spleen or treat underlying cause.

32
Q

What is ITP?

A

Immune Thrombocytopenic purpura
Form auto-antibodies against PLT antigens

33
Q

What is ITP caused by?

A

Unknown
Could be post viral syndrome
Could be associated with immunodeficiency
Diagnosis of exclusion

34
Q

What are the symptoms of ITP?

A

Sudden onset petechial rash, bruising, and bleeding in otherwise healthy child

35
Q

How do you diagnose ITP?

A

PLT <100k
Normal CBC and blood smear
No other clinically apparent associated conditions

36
Q

What is the treatment for ITP?

A

Watchful waiting, frequently spontaneously resolves
First treatment is corticosteroids, then IVIG, then platelet transfusions, then splenectomy

37
Q

What is drug related destruction caused by?

A

Immune mediated destruction, increases clearance and destruction of PLTs.
Commonly after ABx

38
Q

What is the presentation of drug-related destruction?

A

Severe thrombocytopenia and mucocutaenous bleeding 7-14 days after taking the new drug

39
Q

How to treat drug-related thrombocytopenia?

A

Stop the drug
Might need corticosteroids

40
Q

What is bleeding associated consumption?

A

Caused by massive bleeding that forces the patient to consume PLTs beyond rates of production
DIC like state

41
Q

What is the treatment for bleeding associated consumption?

A

Blood product infusions and intervention to stop the bleeds

42
Q

What is the cause of hypoproliferative thrombocytopenia?

A

Most commonly caused by bone marrow failure
Can be due to Leukemia/Lymphoma/myelodysplasia/aplasia
Cancers metastatic to bone marrow
Severe infection (CMV, HIV)
Radiation/chemo

43
Q

What is the treatment for hypoproliferative thrombocytopenia?

A

Viral - typically self resolving
Cancer - treat the cancer to restore counts
Might need stem cell transplant (esp aplasia)

44
Q

What are qualitative PLT disorders?

A

PLT count is normal but there are defects to the PLT that causes them to not function well.

45
Q

What is the most common cause of qualitative PLT disorders?

A

Iatrogenic or acquired. Can be congenital but that is rare

46
Q

How do qualitative PLT disorders manifest?

A

Mucus membrane bleeding

47
Q

What is the treatment for qualitative PLT disorders?

A

Platelet transfusion

48
Q

What are the most common causes of acquired/iatrogenic PLT defects?

A

Aspirin, Plavix, NSAIDs

Aspirin and Plavix are irreversible (5-10 days)
NSAIDS are reversible (24-48h)

49
Q

What is thrombotic microangiopathy?

A

Incorporation of platelets into thrombi of the microvasculature

50
Q

What is thrombotic thrombocytopenic purpura?

A

A disorder from a severe deficiency in ADAMTS13 that causes small clots form more frequently

51
Q

What is the cause of TTP?

A

Unknown

52
Q

What lab findings do you expect to see in TTP?

A

Large multimeters of vWF
Lack plasma protease

53
Q

What is the presentation of TTP?

A

Acute or subacute symptoms related to neurological dysfunction, anemia, or thrombocytopenia. Might also see fevers and dark urine

54
Q

How do you diagnose TTP?

A

Elevated WBC
Hgb of 8-9
Platelet 20-50k
Smear shows schistocytosis
PT/PTT are normal
Elevated D-dimer
Fibrinogen is high
LDH and bilirubin are high
Direct Coombs negative

55
Q

What is the treatment for TTP?

A

Plasma exchange with FFP
95% mortality rate without plasma exchange
DO NOT just give platelets

56
Q

What is HUS?

A

Hemolytic-uremic syndrome
Progressive renal failure that is associated with microangiopathic hemolytic anemia and thrombocytopenia

57
Q

What is the most common cause of acute renal failure in children?

A

HUS, also seen in adults though

58
Q

What i the presentation of HUS?

A

Gastroenteritis (bloody diarrhea)
Lethargy
Seisures
Renal failure
Anuria
HTN
Edema
Pallor

59
Q

What is the cause of HUS?

A

Shiga-like toxin
E coli 0157:H7

60
Q

What laboratory findings support HUS?

A

Elevated BUN/CR
Severe anemia and thrombocytopenia
Bilirubin and LDH are elevated
Get a stool sample

61
Q

What is the treatment for HUS?

A

Supportive care only
Do not do a plasma exchange

62
Q

What is disseminated intravascular coagulopathy?

A

Uncontrolled local or systemic activation of coagulation leading to thrombocytopenia and depletion of coagulation factors and fibrinogen

63
Q

What are lab findings that support DIC?

A

Prolonged PT and PTT
Thrombocytopenia
Decreased fibrinogen
Elevated d-dimer
Schistocytes on blood smear

64
Q

What disorders are associated with DIC?

A

Sepsis
Cancer/trauma/burns/pregnancy
AAA, cavernous hemangiomas
Snake bites

65
Q

What clinical features support DIC?

A

Multiple sites of bleeding
Purpura and petechiae

66
Q

What is the treatment for DIC?

A

Ask hematology LOL
Treat the cause, transfuse patient if bleeding, use heparin if persistent

Heme/Onc (10 decks)

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