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Disorders of Hemostasis & Thrombosis Flashcards

1
Q

Most common form of thrombocytopenia

A

Immune thrombocytopenia

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2
Q

What is the patho behind immune thrombocytopenia?

A

autoimmune, where pathogenic abx bind to platelets, and splenic macrophages destroy them

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3
Q

What age is the peak incidence for ITP?

A

2-4yrs

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4
Q

What does ITP usually follow?

A

viral illness (1-3wks)

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5
Q

What are two secondary ITP causes?

A

HIV and Hep C

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6
Q

What are 4 s/s of ITP?

A

ecchymosis, epistaxis, gingival bleeding, hemorrhagic bullae in mouth

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7
Q

What is seen on a CBC for ITP?

A

isolated thrombocytopenia

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8
Q

When should a bone marrow be done in ITP?

A
  • unexplained cytopenia >60yr

- pts don’t respond to primary ITP thx

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9
Q

Asymptomatic pt with unexplained isolated thrombocytopenia?

A

=ITP

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10
Q

Txt for symptomatic pt with low platelet count?

A

short course corticosteroids
+/- IVIG or anti- D (WinRho)
+/- platelets for bleeding

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11
Q

Txt for symptomatic pt with low platelet count and persistent ITP?

A

Short course corticosteroids
AND
IVIG or anti-D or Rituximab or thrombopoeitin receptor agonist
Or splenectomy

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12
Q

Txt for ITP in preggo?

A

Prednisone or IVIG infusions

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13
Q

Txt for ITP in preggo failure thx?

A

Splenectomy (1st/2nd trimester)

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14
Q

What are the platelet count ranges during preggo in order for txt?

A

1st- 10,000-30,000
2nd/3rd- >30,000
delivery -50,000

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15
Q

Txt for secondary ITP?

A

TREAT infections

IVIG or anti-D, splenectomy (HIV), interferon-alpha (Hep C), Eltrombopag (Hep C)

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16
Q

What is considered a true emergency?

A

Thrombotic Thrombocytopenic Purpura

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17
Q

What is the patho behind TTP?

A

deficiency of von Willebrand’s factor cleaving proteolytic enzyme, which increases vWF causing abnormal platelet aggregation

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18
Q

What is microangiopathic hemolytic anemia?

A

shearing of erythrocytes in the microcirculation

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19
Q

What is the pentad of clinical findings in TTP?

A

thrombocytopenia, microangiopathic hemolytic anemia, CNS abnormalities, fever and renal dysfunction

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20
Q

What is PARTNERS?

A 
Plasmapheresis
Abnormal 
Renal Failure
T?
N
E
R
S
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21
Q

What is seen on CBC peripheral smear for TTP?

A

schistocytes
helmet cells
polychromasia

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22
Q

What is seen on labs for TTP?

A

Reductions in vWFCP activity

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23
Q

What is seen on the CMP for TTP?

A

Increased BUN/Creatinine

LDH, indirect bilirubin

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24
Q

What is the txt for TTP?

A

Plasma exchange (Plasmapheresis)

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25
What are the 3 alternative txt for TTP?
Fresh Frozen Plasma Corticosteroids Splenectomy
26
What is contradicted in TTP?
Platelet transfusions
27
What is the most common cause of acute kidney failure in children?
Hemolytic Uremic Syndrome
28
What is the patho of HUS?
occurring after E.coli infection, in which its toxins are localized to the kidneys leading to cell necrosis
29
What is the Lab findings in HUS?
Pentad Blood in stools (+ E.coli), vomiting, usually no fever decreased BUN/ Creatinine increased LDH, indirect bilirubin
30
What is the txt for HUS?
Supportive care
31
What is the patho behind DIC?
uncontrolled activation of coagulation, leading to increased platelet destruction, and bleeding occurring at multiple sites
32
What disorders are seen in DIC?
Sepsis, Cancer, Trauma, Aortic aneurysms, snake bites
33
What is seen on a CBC peripheral smear?
decreased platelets, schistocytes
34
What are lab findings of DIC?
low level of clotting factors V, VIII
35
Txt for DIC?
TREAT UNDERLYING DISORDER
36
What blood products can be given in DIC?
platelets FFP Cryoprecipitate
37
How often are labs followed for DIC?
4-6hrs until DIC is resolved and underlying condition is treated
38
Txt for persistent or refractory bleeding in DIC?
Heparin
39
What are C/I to heparin txt in DIC?
platelets are <50,000 GI or CNS bleeding Conditions that surgery is indicated Placental abruption
40
What is HELLP syndrome?
Hemolysis Elevated liver enzymes Low platelets
41
What is the txt for HELLP?
Delivery
42
Txt for Trousseau syndrome?
treat malignancy heparin blood products as indicated
43
What is coagulation factor missing in Hemophilia A?
VIII
44
Who is more prone to receiving hemophilia A?
males
45
What are clinical findings of Hemophilia A?
pain in weight bearing joints (Hemarthrosis) | spontaneous bleeding
46
What is the txt for Hemophilla A?
severe- long term prophylactic of factor 2x to 3x weekly Mild/Minor- DDAVP Mild/Major- DDAVP or 8 concentrate Moderate/Severe/Minor/Major- Factor VIII
47
Christmas disease?
Hemophilia B
48
Which factor is deficient in Hemophilia B?
IX
49
What is the txt for mild, moderate, or severe Hemophilia B?
Factor IX concentrate
50
Which factor is deficient in Hemophilia C?
XI
51
Prevalent among Ashkenazi Jewish decent?
Hemophilia C
52
Txt for Hemophilia C?
Fresh frozen plasma (FFP)
53
The most common inherited bleeding disorder?
von Willebrand's Disease (vWD)
54
What is the patho of vWD?
autosomal dominant disorder results from deficient or defective von Willebrand's factor causing infective platelet adhesion
55
Type 1 vWD?
quantitive abnormality
56
Type 2 vWD?
qualitative defect
57
Type 3 vWD?
Mutational homozygosity or double heterozygosity
58
Decreased vWF can also lead to decreased ??
VIII
59
What is clinical finding of vWD?
bleeding worse w/ ASA
60
What is the MC type of vWD?
Type I
61
What is the txt for type I?
minor bleeding: DDAVP | Major: DDAVP, vWF concentrate
62
What is are clinical features of Vit K deficiency?
bleeding
63
TXT for Vit K deficiency?
``` increase green leafy veges Vitamin K (phytonadione) IV or oral ```
64
What are two factors that the liver DOESN'T produce?
VIII and vWF
65
What is the txt for coagulopathy of the liver?
FFP Cryoprecipatie Liver transplant
66
A inherited autosomal dominant, acquired disease that occurs at a young age?
Antithrombin III Deficiency
67
TXT for Antithrombin III deficiency?
IV heparin, then oral anticoagulation indefinitely
68
Which is more common Protein C or S deficiency?
C
69
TXT for protein C and S deficiency?
IV heparin, then oral anticoagulation indefinitely
70
Mutation in F5 gene?
Factor V Leiden
71
Txt for Factor V leiden?
IV heparin, then oral anticoagu indefinitely
72
What are 4 ways in which acquired hypercoagulability happens?
pregnancy malignancy OCP Lupus anticoagulant
73
The most common complication and major cause of morbidity/death?
thrombosis
74
What is the hallmark lab finding in polycythemia vera?
hematocrit > 54% in males
75
Txt for polycythemia vera?
reduce blood volume to normal via Phlebotomy
76
What is a commonly used myelosuppresive agent
Hydroxyurea

Hematology (4 decks)

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