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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
What is the presentation of factor IX deficiency?
Mild bleeding
26
What is the treatment for factor XI deficiency?
Factor XI concentrate for bleeding Can also use FFP
27
What is thrombocytopenia?
Too few platelets so cannot form a primary plug or secondary clot
28
What are the symptoms of thrombocytopenia?
Epistaxis, gum bleeding, GI bleeds
29
What are the causes of thrombocytopenia?
Decreased production of platelets Increased sequestration of platelets Pregnancy -> volume overload
30
What are the causes of destructive or consumption thrombocytopenia?
Spenomegaly Antibodies (ITP) Drugs Massive bleeds Thrombus formation
31
How does splenomegaly cause thrombocytopenia?
Spleen has upregulated clearance of platelets. Caused by liver disease or malignancy. Must remove spleen or treat underlying cause.
32
What is ITP?
Immune Thrombocytopenic purpura Form auto-antibodies against PLT antigens
33
What is ITP caused by?
Unknown Could be post viral syndrome Could be associated with immunodeficiency Diagnosis of exclusion
34
What are the symptoms of ITP?
Sudden onset petechial rash, bruising, and bleeding in otherwise healthy child
35
How do you diagnose ITP?
PLT <100k Normal CBC and blood smear No other clinically apparent associated conditions
36
What is the treatment for ITP?
Watchful waiting, frequently spontaneously resolves First treatment is corticosteroids, then IVIG, then platelet transfusions, then splenectomy
37
What is drug related destruction caused by?
Immune mediated destruction, increases clearance and destruction of PLTs. Commonly after ABx
38
What is the presentation of drug-related destruction?
Severe thrombocytopenia and mucocutaenous bleeding 7-14 days after taking the new drug
39
How to treat drug-related thrombocytopenia?
Stop the drug Might need corticosteroids
40
What is bleeding associated consumption?
Caused by massive bleeding that forces the patient to consume PLTs beyond rates of production DIC like state
41
What is the treatment for bleeding associated consumption?
Blood product infusions and intervention to stop the bleeds
42
What is the cause of hypoproliferative thrombocytopenia?
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
What is the treatment for hypoproliferative thrombocytopenia?
Viral - typically self resolving Cancer - treat the cancer to restore counts Might need stem cell transplant (esp aplasia)
44
What are qualitative PLT disorders?
PLT count is normal but there are defects to the PLT that causes them to not function well.
45
What is the most common cause of qualitative PLT disorders?
Iatrogenic or acquired. Can be congenital but that is rare
46
How do qualitative PLT disorders manifest?
Mucus membrane bleeding
47
What is the treatment for qualitative PLT disorders?
Platelet transfusion
48
What are the most common causes of acquired/iatrogenic PLT defects?
Aspirin, Plavix, NSAIDs Aspirin and Plavix are irreversible (5-10 days) NSAIDS are reversible (24-48h)
49
What is thrombotic microangiopathy?
Incorporation of platelets into thrombi of the microvasculature
50
What is thrombotic thrombocytopenic purpura?
A disorder from a severe deficiency in ADAMTS13 that causes small clots form more frequently
51
What is the cause of TTP?
Unknown
52
What lab findings do you expect to see in TTP?
Large multimeters of vWF Lack plasma protease
53
What is the presentation of TTP?
Acute or subacute symptoms related to neurological dysfunction, anemia, or thrombocytopenia. Might also see fevers and dark urine
54
How do you diagnose TTP?
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
What is the treatment for TTP?
Plasma exchange with FFP 95% mortality rate without plasma exchange DO NOT just give platelets
56
What is HUS?
Hemolytic-uremic syndrome Progressive renal failure that is associated with microangiopathic hemolytic anemia and thrombocytopenia
57
What is the most common cause of acute renal failure in children?
HUS, also seen in adults though
58
What i the presentation of HUS?
Gastroenteritis (bloody diarrhea) Lethargy Seisures Renal failure Anuria HTN Edema Pallor
59
What is the cause of HUS?
Shiga-like toxin E coli 0157:H7
60
What laboratory findings support HUS?
Elevated BUN/CR Severe anemia and thrombocytopenia Bilirubin and LDH are elevated Get a stool sample
61
What is the treatment for HUS?
Supportive care only Do not do a plasma exchange
62
What is disseminated intravascular coagulopathy?
Uncontrolled local or systemic activation of coagulation leading to thrombocytopenia and depletion of coagulation factors and fibrinogen
63
What are lab findings that support DIC?
Prolonged PT and PTT Thrombocytopenia Decreased fibrinogen Elevated d-dimer Schistocytes on blood smear
64
What disorders are associated with DIC?
Sepsis Cancer/trauma/burns/pregnancy AAA, cavernous hemangiomas Snake bites
65
What clinical features support DIC?
Multiple sites of bleeding Purpura and petechiae
66
What is the treatment for DIC?
Ask hematology LOL Treat the cause, transfuse patient if bleeding, use heparin if persistent

Heme/Onc (10 decks)

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