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Hematology > Disorders of Hemostasis & Thrombosis > Flashcards

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
Q

What are the 3 alternative txt for TTP?

A

Fresh Frozen Plasma
Corticosteroids
Splenectomy

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26
Q

What is contradicted in TTP?

A

Platelet transfusions

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27
Q

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

A

Hemolytic Uremic Syndrome

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28
Q

What is the patho of HUS?

A

occurring after E.coli infection, in which its toxins are localized to the kidneys leading to cell necrosis

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29
Q

What is the Lab findings in HUS?

A

Pentad
Blood in stools (+ E.coli), vomiting, usually no fever
decreased BUN/ Creatinine
increased LDH, indirect bilirubin

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30
Q

What is the txt for HUS?

A

Supportive care

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31
Q

What is the patho behind DIC?

A

uncontrolled activation of coagulation, leading to increased platelet destruction, and bleeding occurring at multiple sites

32
Q

What disorders are seen in DIC?

A

Sepsis, Cancer, Trauma, Aortic aneurysms, snake bites

33
Q

What is seen on a CBC peripheral smear?

A

decreased platelets, schistocytes

34
Q

What are lab findings of DIC?

A

low level of clotting factors V, VIII

35
Q

Txt for DIC?

A

TREAT UNDERLYING DISORDER

36
Q

What blood products can be given in DIC?

A

platelets
FFP
Cryoprecipitate

37
Q

How often are labs followed for DIC?

A

4-6hrs until DIC is resolved and underlying condition is treated

38
Q

Txt for persistent or refractory bleeding in DIC?

39
Q

What are C/I to heparin txt in DIC?

A

platelets are <50,000
GI or CNS bleeding
Conditions that surgery is indicated
Placental abruption

40
Q

What is HELLP syndrome?

A

Hemolysis
Elevated liver enzymes
Low platelets

41
Q

What is the txt for HELLP?

42
Q

Txt for Trousseau syndrome?

A

treat malignancy
heparin
blood products as indicated

43
Q

What is coagulation factor missing in Hemophilia A?

44
Q

Who is more prone to receiving hemophilia A?

45
Q

What are clinical findings of Hemophilia A?

A

pain in weight bearing joints (Hemarthrosis)

spontaneous bleeding

46
Q

What is the txt for Hemophilla A?

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
Q

Christmas disease?

A

Hemophilia B

48
Q

Which factor is deficient in Hemophilia B?

49
Q

What is the txt for mild, moderate, or severe Hemophilia B?

A

Factor IX concentrate

50
Q

Which factor is deficient in Hemophilia C?

51
Q

Prevalent among Ashkenazi Jewish decent?

A

Hemophilia C

52
Q

Txt for Hemophilia C?

A

Fresh frozen plasma (FFP)

53
Q

The most common inherited bleeding disorder?

A

von Willebrand’s Disease (vWD)

54
Q

What is the patho of vWD?

A

autosomal dominant disorder results from deficient or defective von Willebrand’s factor causing infective platelet adhesion

55
Q

Type 1 vWD?

A

quantitive abnormality

56
Q

Type 2 vWD?

A

qualitative defect

57
Q

Type 3 vWD?

A

Mutational homozygosity or double heterozygosity

58
Q

Decreased vWF can also lead to decreased ??

59
Q

What is clinical finding of vWD?

A

bleeding worse w/ ASA

60
Q

What is the MC type of vWD?

61
Q

What is the txt for type I?

A

minor bleeding: DDAVP

Major: DDAVP, vWF concentrate

62
Q

What is are clinical features of Vit K deficiency?

63
Q

TXT for Vit K deficiency?

A 
increase green leafy veges
Vitamin K (phytonadione) IV or oral
64
Q

What are two factors that the liver DOESN’T produce?

A

VIII and vWF

65
Q

What is the txt for coagulopathy of the liver?

A

FFP
Cryoprecipatie
Liver transplant

66
Q

A inherited autosomal dominant, acquired disease that occurs at a young age?

A

Antithrombin III Deficiency

67
Q

TXT for Antithrombin III deficiency?

A

IV heparin, then oral anticoagulation indefinitely

68
Q

Which is more common Protein C or S deficiency?

69
Q

TXT for protein C and S deficiency?

A

IV heparin, then oral anticoagulation indefinitely

70
Q

Mutation in F5 gene?

A

Factor V Leiden

71
Q

Txt for Factor V leiden?

A

IV heparin, then oral anticoagu indefinitely

72
Q

What are 4 ways in which acquired hypercoagulability happens?

A

pregnancy
malignancy
OCP
Lupus anticoagulant

73
Q

The most common complication and major cause of morbidity/death?

A

thrombosis

74
Q

What is the hallmark lab finding in polycythemia vera?

A

hematocrit > 54% in males

75
Q

Txt for polycythemia vera?

A

reduce blood volume to normal via Phlebotomy

76
Q

What is a commonly used myelosuppresive agent

A

Hydroxyurea

Hematology (4 decks)

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