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Master the Boards: Hematology > Coagulation Disorders > Flashcards

Coagulation Disorders Flashcards

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1
Q

How does Platelet bleeding present?

A

Superficial
Epistaxis, Gingival, Petechiae, purpura, Mucosal surfaces, vaginal bleeding

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2
Q

How does factor bleeding present?

A

Deep: joints and muscles

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3
Q

What is Idiopathic Thrombocytopenia (ITP)?

A

Diagnosis of exclusion with isolated thrombocytopenia and normal-sized spleen

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4
Q

What is unique about platelets in ITP?

A

They are large

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5
Q

What are occasional diagnostic tests for ITP?

A

Elevated Megakaryocytes
Antiplatelet Ab: lack specificity
US/CT: r/o hypersplenism
BM only indicated before splenectomy
Elevated Mean Platelet Volume: large platelets

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6
Q

How is ITP treated if PLT>30,000 without bleeding?

A

No treatment

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7
Q

How is ITP treated if PLT<30,000 with mild bleeding?

A

Glucocorticoids

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8
Q

How is ITP treated with Sever (GI/CNS) bleed and PLT <10,000?

A

IVIG, Anti-Rho

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9
Q

How is Recurrent episodes of steroid dependent ITP treated?

A

Splenectomy

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10
Q

What must you vaccinate against before performing Splenectomy?

A

N. Meningitidis
H. Influenzae
Pneumococcus

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11
Q

What drugs can be used if Splenectomy is not effective in treating ITP?

A

Thrombopoietins
Rituximab
Fostamatinib
Immune Suppressives

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12
Q

What Thrompoietin Drugs are commonly used in ITP?

A

Avatrombopag
Eltrombopag
Romiplostim

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13
Q

What is the role of Rituximab in ITP?

A

Removes lymphs that attack PLT

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14
Q

What is the role of Fostamatinib in ITP?

A

Splenic Tyrosine Kinase

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15
Q

What immune suppressive drugs are commonly used in ITP?

A

Azathiprine
Mycophenolate
Cyclosporine

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16
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand Disease (VWD)

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17
Q

What is Von Willebrand Disease (VWD)?

A

AD decreased level or functioning of VWF that leads to impaired platelet-endothelial binding

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18
Q

How does Von Willebrand Disease (VWD) commonly present?

A

Platelet related bleeding: epistaxis, gingival, gums with a normal platelet count

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19
Q

What drug markedly worsens Von Willibrand Disease (VWD)?

A

Aspirin

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20
Q

How is Von Willebrand Disease (VWD) treated?

A

Decreased VWF (Ag)
Ristocetin Cofactor assay: detects VWF dysfunction/activity
Factor VIII level
Normal PLT
Elevated aPTT
Bleeding time increased duration of bleedin g

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21
Q

How do you treat VWD?

A

DDAVP (Desmopressin): releases subendothelial stores of VWF

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22
Q

IF VWD Does not respond to DDAVP (Desmopressin), what do you use next?

A

Factor VIII replacement or VWF concentrate

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23
Q

What is the defect in Glanzmann Thrombasthenia?

A

IIb/IIIa defect

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24
Q

What is the defect in Bernard-Soulier Syndrome?

A

Ib/IX defect

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25
How des Glanzmann Thrombasthenia present?
Platelet-type bleeding: epistaxis, petechiae with a normal platelet count
26
How does Bernard-Soulier syndrome present?
Platelet-type bleeding: epistaxis, petechiae with a normal platelet count
27
How is Glanzmann Throbathenia diagnosed?
Platelet studies
28
How is Bernard-Soulier Syndrome diagnosed?
Platelet studies
29
What is the distinguishing feature that separates Bernard-soulier from Glanzmann?
Bernard-Soulier has giant platelets
30
How do you treat Glanzmann and Bernard-Soulier?
Desmopressin: release VWF and factor VIIIa Tranexamic Acid and Epsilon-Aminocaproic acid: inhibit fibrinolysis and plasminogen Recombinat Factor VIIa Estrogen to upregulate VWF
31
How does hemophilia present?
Delayed joint or muscle bleeding in a male child
32
What patients present with hemophilia?
Male: it is XLR
33
Why is bleeding delayed in Hemophilia?
Primary hemostatic plug is with platelets
34
What is the most accurate test for Hemophilia?
Specific assay for factor VIII or IX
35
What happens to PT with Hemophilia?
It is normal
36
What happens to aPTT in Hemophilia?
Prolonged: but corrects to normal with mixing study
37
How do you treat mild bleeding in Hemophilia?
DDAVP
38
How do you treat severe bleeding in Hemophilia?
Replace specific factor deficiency Exception: severe bleeding from factor VIII Ab replace with factor VII Mixing studies with normal plasma to correct aPTT to normal
39
How does Factor XII Deficiency present?
Elevated aPTT but no bleeding
40
How do you treat Factor XII deficiency?
no treatment needed
41
How does Factor XI Deficiency Present?
increased bleeding with trauma or surgery
42
What coagulation study results are associated with Factor XI deficiency?
normal PT prolonged aPTT Mixing study corrects the aPTT to normal
43
What do you use to stop the bleeding in Factor XI deficiency?
Fresh Frozen Plasma (FFP)
44
How does Disseminated Intravascular Coagulation (DIC) present?
Patients with definite risk with bleeding related to both clotting factor deficiency and thrombocytopenia
45
What are common definitive risks associated with DIC?
Sepsis, Burns, Snake Bites Abruptio placentae or amniotic fluid embolus Trauma causing tissue factor release, Cancer
46
What coagulation study results are associated with DIC?
Elevated PT Elevated aPTT Low platelet count Elevated D-dimer and fibrin split products Decreased Fibrinogen: from consumption
47
How do treat DIC with PLT <50,000 and serious bleeding?
Replace platelets and clotting factors using FFP
48
How to treat DIC bleeding not controlled with FFP?
Cryoprecipitate to replace fibrinogen
49
What is the most common Hypercoagulable state (Thrombophilia)?
Factor V Leiden Mutation
50
What is Factor V Leiden?
mutation makes Factor V resistant to the normal anticoagulant effect of Protein C (protein-C resistance)
51
What anticoagulation proteins are vitamin K dependent?
Protein C and Protein S
52
What is a clue to suggest Protein C or Protein S deficiency?
Increased clotting just after the start of warfarin: more common with protein C deficiency
53
What is the only thrombophilia that increases the aPTT?
Anti-phospholipid syndrome
54
What drug is superior to DOAC in patients with APL, Metal Valves or MItral stenosis (MS)?
Warfarin
55
What is Heparin-Induced Thrombocytopenia (HIT) more common with?
Unfractionated Heparin
56
What is Heparin-Induced Thrombocytopenia (HIT)?
Ab-mediated platelet activation
57
How does Heparin-Induced Thrombocytopenia (HIT) Present?
5-10 days after the start of heparin with a marked drop in platelet count >30% Both venous and arterial thromboses can occur: but venous clots are more common Bleeding rarely occurs: platelets just precipitate out
58
How do you confirm the diagnosis of HIT?
ELISA for Platelet factor 4 (PF4) Ab or serotonin release assay
59
How do you treat HIT?
STop all Heparain-containing products immediately Give Fondaparinux or Direct Thrombin Inhibitors
60
What Direct thrombin Inhibitors are used for HIT?
Argatroban or Bivalirudin
61
What can happen if you transfuse PLT into a patient with HIT?
It can worsen Thrombosis
62
What are the Antiphospholipid (APL) syndromes?
Lupus Anticoagulant beta-2-glycoprotein and anticardiolipin Ab
63
What do Antiphospholipid (APL) syndromes cause?
Thrombosis
64
What coagulation abnormality is only is in APL syndromes amongst Thrombophilia?
increased aPTT
65
What are anticardiolipin antibodies associated with?
Multiple spontaneous abortions
66
What is the best initial test for Antiphospholipid Syndomres?
Mixing study
67
What is seen on mixing study for Antiphospholipid Syndromes?
The aPTT will remain elevated after the mix due to circulating inhibitor
68
What is the most accurate test for Lupus anticoagulant?
Russell Viper Venom Test
69
What is the only thrombophilia important to test for with the first clot?
APL Syndrome
70
how do you treat Antiphospholipid Syndromes?
Heparin and Warfarin -lifelong anticoagulation for APL syndrome
71
How Does ABO incompatibility Transfusion reaction present?
Sudden Dyspnea/chest pain during transfusion dark urine Hemolysis
72
How is ABO incompatibility transfusion reaction tested/treated?
Re-type Blood UA with hemoglobin Hyperkalemia Give IV fluids and treat like Rhabdo
73
How does a minor blood group transfusion reaction present?
Delay jaundice with hemolysis
74
How is a minor blood group transfusion reaction tested/treated?
Elevated Indirect Bilirubin No therapy
75
How do Ab to Donor WBC in transfusion present?
Fever only, no hemolysis
76
How is Ab to donor WBC transfusion reaction tested/treated?
WBC filter in future
77
How does a transfusion reaction to Ab to donor plasma proteins present?
Urticaria without hemolysis
78
How do you test/treat a transfusion reaction to Ab to donor plasma proteins?
Give antihistamines +/- steroids and wash blood in future
79
How does Transfusion-related acute lung injury (TRALI) present
Dyspnea, hypoxia and abnormal x-ray: no hemolysis
80
What causes TRALI?
Ab against recipient WBC in transfusion
81
How is TRALI tested/treated?
Transient Hypoxia resolves spontaneously

Master the Boards: Hematology (7 decks)

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