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Hematology/Immunology Part 1 > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

When should a patient be evaluated for bleeding disorders?

A

Spontaneous bleeding, excessive hemorrhage after surgery or trauma, menorrhagia, bleeding after venipuncture, excessive bleeding after labor/delivery.

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2
Q

What co-existing diseases can impair platelet or vessel wall interactions?

A

Renal failure and liver disease.

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3
Q

How can malignancy affect coagulation?

A

Recent chemotherapy can cause thrombocytopenia.

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4
Q

Which drugs can cause thrombocytopenia?

A

Aspirin, NSAIDs, and certain antibiotics.

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5
Q

What are the bleeding patterns associated with thrombocytopenia or platelet disorders?

A

Bleeding from skin and mucosa, GI bleeding, epistaxis, hemoptysis, spontaneous or immediate after trauma.

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6
Q

What are the bleeding patterns associated with factor deficiencies?

A

Bleeding from deeper tissue sites, such as hematomas, retroperitoneal hemorrhage, and hemarthroses, with more delayed onset.

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7
Q

What are petechiae?

A

Pinpoint skin hemorrhages less than 3mm in size.

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8
Q

What initiates the extrinsic pathway in the coagulation cascade?

A

External Vessel damage and tissue factor (TF), trauma/injury to vessel wall, tissue damage, hypoxia, sepsis, inflammation.

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9
Q

What is purpura?

A

Skin hemorrhages between 3-10mm, sometimes palpable.

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10
Q

What is another name for the extrinsic pathway?

A

Tissue Factor Pathway

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11
Q

Which factor is activated in the extrinsic pathway?

A

Tissue Factor III

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12
Q

What is the common pathway in the coagulation cascade?

A

The pathway where both intrinsic and extrinsic pathways converge, leading to fibrin formation.

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13
Q

What is ecchymosis?

A

A bruise larger than 1cm.

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14
Q

What initiates the intrinsic pathway in the coagulation cascade?

A

Contact with subendothelial surface damage

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15
Q

What is another name for the intrinsic pathway?

A

Contact Activation Pathway

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16
Q

What basic laboratory evaluations are used for bleeding disorders?

A

CBC (Hgb, Platelets), coagulation profile (PT/INR, aPTT), fibrinogen, D-Dimer.

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17
Q

What does PT/INR measure?

A

Clotting factors from the extrinsic pathway.

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18
Q

Which factors are involved in the intrinsic pathway?

A

HMWK, PK, XII, XI, IX, X,VIII

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19
Q

What does ‘a’ signify in the context of coagulation factors?

A

Activated

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20
Q

What does aPTT measure?

A

Clotting factors from the intrinsic pathway.

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21
Q

What is the role of Factor XIIIa in the common pathway?

A

Cross-links fibrin

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22
Q

What is the role of thrombin in the coagulation cascade?

A

Converts fibrinogen to fibrin

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23
Q

What are the normal ranges for PT, INR, and aPTT?

A

PT: 11-13.5 sec, INR: ≤ 1.1, aPTT: 21-35 sec.

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24
Q

What is the role of antithrombin in physiologic anticoagulation?

A

Inactivates thrombin and other factors

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25
Q

What is fibrinogen and where is it synthesized?

A

A protein synthesized by the liver.

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26
Q

What are the major causes of DIC?

A

Infections, sepsis, immunologic reactions, transfusion reactions, obstetric complications, malignancies, liver failure, acute pancreatitis, envenomation, ARDS, trauma, shock, vascular disorders.

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27
Q

What are some examples of immunologic reactions that can cause DIC?

A

Transfusion reactions and ABO incompatibility.

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28
Q

What obstetric complications can lead to DIC?

A

Septic abortion, retained contents, placental abruption.

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29
Q

What is the most common complication of severe hemophilia?

A

Acute Hemarthrosis

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30
Q

What accelerates the action of antithrombin?

A

Heparin

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31
Q

Which malignancies are associated with DIC?

A

Acute leukemia and adenocarcinoma.

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32
Q

What sensation precedes intense pain and swelling in acute hemarthrosis?

A

Tingling or burning sensation

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33
Q

What is the role of proteins C and S in anticoagulation?

A

Destroy factors V and VIII

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34
Q

What are the physical exam findings in acute hemarthrosis?

A

Hot, swollen, and tender joint with erythema

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35
Q

What are the key features of acute DIC?

A

Introduction of tissue factor into the bloodstream, initiation of the extrinsic pathway, thrombin formation, platelet activation, excess consumption of clotting factors, elevated PT/INR, aPTT, thrombocytopenia, mucosal bleeding.

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36
Q

What happens to Vitamin K after it activates clotting factors?

A

It becomes de-activated.

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37
Q

What happens to clotting factors in acute DIC?

A

Excess consumption of clotting factors, leading to elevated PT/INR and aPTT.

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38
Q

What are the symptoms of acute hemarthrosis?

A

Pain, stiffness, limited mobility, maintained in flexed position

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39
Q

How are proteins C and S activated?

A

By thrombin

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40
Q

What does a low fibrinogen level indicate?

A

Decreased production or excessive clot formation.

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41
Q

Why does thrombocytopenia occur in acute DIC?

A

Bone marrow can’t keep up with platelet consumption.

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42
Q

Which enzyme re-activates Vitamin K?

A

Vitamin K reductase.

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43
Q

What is the immediate treatment for acute hemarthrosis?

A

Immediate factor replacement

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44
Q

What can deficiencies in anticoagulation mechanisms lead to?

A

Hypercoagulable state

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45
Q

What is D-Dimer?

A

A fibrin degradation product released when clots dissolve.

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46
Q

What does RICE stand for in the context of acute hemarthrosis treatment?

A

Rest, Ice, Compression, Elevation

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47
Q

What are the bleeding manifestations of acute DIC?

A

Petechiae, purpura, bleeding from mucosal sites, venipuncture sites, wounds.

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48
Q

How does Warfarin affect Vitamin K?

A

It inhibits Vitamin K reductase.

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49
Q

What is the mechanism of action of Coumadin (Warfarin)?

A

Vitamin K antagonist

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50
Q

Why is joint aspiration not recommended in acute hemarthrosis?

A

Risk of infection and worsening bleeding

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51
Q

What are the thrombotic manifestations of acute DIC?

A

Digital ischemia and gangrene, especially in patients on vasopressors.

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52
Q

What conditions can elevate D-Dimer levels?

A

Clot (DVT, PE) or DIC.

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53
Q

Why should NSAIDs be avoided in acute hemarthrosis?

A

They may exacerbate bleeding due to platelet inhibition

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54
Q

Which factors require vitamin K for activation?

A

Factors II, VII, IX, and X

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55
Q

What laboratory findings are indicative of DIC?

A

Prolonged PT/INR, aPTT, thrombocytopenia, increased FDP levels, elevated D-Dimer, decreased fibrinogen.

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56
Q

What can result from recurrent or untreated bleeds in hemophilia?

A

Chronic joint damage

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57
Q

What occurs during thrombosis?

A

Simultaneous activation of platelets and formation of fibrin in response to vessel injury.

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58
Q

What does exogenous Vitamin K do in the presence of Warfarin?

A

It provides the necessary active form of Vitamin K.

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59
Q

Which pathway does Warfarin mainly inhibit?

A

Extrinsic pathway and factor VII

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60
Q

What are the features of chronic joint damage in hemophilia?

A

Cartilage damage with cyst formation, bony erosion, flexion contractures

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61
Q

What is primary hemostasis?

A

Platelet activation.

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62
Q

How does chronic DIC differ from acute DIC?

A

Chronic DIC involves continuous or intermittent exposure to small amounts of tissue factor, is usually associated with malignancies, and is primarily a thrombotic disorder with recurrent superficial and deep venous thromboses.

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63
Q

What are the three sources of Vitamin K?

A

Dietary intake, synthetic drugs, and synthesis by intestinal flora.

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64
Q

How is the effect of Warfarin monitored?

A

By PT/INR

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65
Q

What is the primary characteristic of ITP diagnosis?

A

Isolated thrombocytopenia with normal WBC and RBC and no other cause identified.

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66
Q

What is secondary hemostasis?

A

Clotting factor activation leading to fibrin formation.

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67
Q

What is the prophylaxis strategy in severe hemophilia?

A

Scheduled administration of factors 2-3 times a week

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68
Q

What is the primary treatment approach for DIC?

A

Reverse the underlying cause if possible; all other measures are supportive and temporizing.

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69
Q

What percentage of intramuscular hematomas occur in hemophilia patients?

A

30%

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70
Q

What distinguishes primary ITP from secondary ITP?

A

Primary ITP has no identified trigger, while secondary ITP is preceded by infection or immunologic condition.

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71
Q

What role does von Willebrand Factor (vWF) play in hemostasis?

A

Mediates platelet adhesion to the site of vessel injury.

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72
Q

What supportive treatments are used in DIC?

A

Fresh frozen plasma, platelet transfusion, cryoprecipitate if fibrinogen is low.

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73
Q

What are common causes of intramuscular hematomas in hemophilia?

A

Physical trauma, IM injections

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74
Q

When is treatment necessary for ITP in children?

A

If there is bleeding, platelet counts are below 20,000/μl, or it is a chronic form.

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75
Q

What does a prolonged PT/INR indicate?

A

Vitamin K deficiency

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76
Q

What substances do adhered platelets release?

A

ADP and Thromboxane A2.

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77
Q

What is the first-line treatment for ITP?

A

Corticosteroids/Prednisone.

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78
Q

What is the role of heparin in anticoagulation?

A

Activates antithrombin

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79
Q

What conditions can lead to Vitamin K deficiency?

A

Malabsorption, eradication of gut flora, prolonged antibiotic use, critical care, prolonged NPO, broad-spectrum antibiotics, acute gastroenteritis, and diarrhea.

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80
Q

What is the coagulation cascade?

A

A series of reactions leading to the formation of cross-linked fibrin.

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81
Q

What is the fatality rate of intracerebral hemorrhage (ICH) in hemophilia?

A

30%

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82
Q

How long should corticosteroids be used for ITP treatment?

A

Not more than 3-4 weeks.

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83
Q

Why is heparin used cautiously in DIC?

A

Heparin could exacerbate bleeding and is reserved for DIC associated with malignancy and forms mainly manifested by thrombosis.

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84
Q

What activates the intrinsic pathway of the coagulation cascade?

A

Internal damage to the vessel wall.

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85
Q

What is the function of Intravenous Immune Globin (IVIG) in ITP treatment?

A

It blocks the Fc receptor on macrophages.

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86
Q

What activates the extrinsic pathway of the coagulation cascade?

A

External trauma or injury to the vessel wall, tissue damage, hypoxia, sepsis, inflammation.

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87
Q

What is a significant cause of death in liver failure related to coagulopathy?

A

Bleeding, particularly GI bleeding due to portal hypertension and esophageal varices.

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88
Q

Why should non-emergent platelet transfusions be avoided in ITP treatment?

A

They are not effective and can cause complications.

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89
Q

What is the end result of both the intrinsic and extrinsic pathways?

A

Activation of Factor X, leading to the common pathway.

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90
Q

What usually induces intracerebral hemorrhage in hemophilia patients?

A

Head trauma

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91
Q

Which factors does heparin inactivate?

A

Factors XII, XI, IX, X, and thrombin

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92
Q

What laboratory finding is associated with Vitamin K deficiency?

A

Prolonged PT/INR.

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93
Q

What is Rituximab used for in ITP treatment?

A

It targets B-cells which produce antibodies against platelets.

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94
Q

What type of bleeding should be avoided with NSAID use in hemophilia?

A

GI bleeding

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95
Q

Which pathways does heparin mainly inhibit?

A

Intrinsic and common pathways

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96
Q

What type of bleeding is seen with Vitamin K deficiency?

A

Bleeding with minor trauma, spontaneous bruising, petechiae, and spontaneous internal bleeding.

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97
Q

What is the basis for suspecting hemophilia in a patient?

A

Family and personal bleeding history

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98
Q

What coagulation profile tests are used in diagnosing hemophilia?

A

aPTT, PT/INR

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99
Q

How is the effect of heparin monitored?

A

By aPTT

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100
Q

What are the mechanisms of coagulopathy in liver failure?

A

Impaired clotting factor synthesis, impaired activation of vitamin K-dependent factors, ineffective y-carboxylation, platelet dysfunction, splenomegaly, suppression of thrombopoiesis by alcohol or folate deficiency.

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101
Q

What is the final product of the common pathway in the coagulation cascade?

A

Cross-linked fibrin mesh forming a stable clot.

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102
Q

What are the symptoms of HITT?

A

Hemorrhage or thrombosis, thrombocytopenia without evidence of thrombosis, skin necrosis due to focal thrombosis.

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103
Q

What does Thrombopoietin (TPO) Receptor Agonist do in ITP treatment?

A

Promotes growth and differentiation of platelets and increases production.

104
Q

At what platelet count might bleeding occur with minor trauma?

A

Less than 50,000.

105
Q

What is another name for the extrinsic pathway?

A

Tissue Factor Pathway.

106
Q

How is hemophilia confirmed?

A

Factor VIII and IX assay

107
Q

What is the role of Factor X in the coagulation cascade?

A

Activation leads to the common pathway and clot formation.

108
Q

When is a high-dose TPO Receptor Agonist given in ITP treatment?

A

In case of severe bleeding or in chronic form.

109
Q

Why is vitamin K ineffective in treating coagulopathy of liver failure?

A

The coagulopathy is due to a deficiency in the process of clotting factor synthesis, not a vitamin K deficiency.

110
Q

What are NOACs also known as?

A

Direct oral anticoagulants (DOACs)

111
Q

At what platelet count does spontaneous bruising and petechiae occur?

A

Less than 30,000.

112
Q

What are some examples of TPO Receptor Agonists?

A

Romiplostim (N-Plate), Eltrombopag (Promacta), Avatrombopag (Doptelet).

113
Q

What additional testing is available for hemophilia diagnosis?

A

Genetic testing

114
Q

What is the typical platelet count in liver failure coagulopathy?

A

Approximately 50,000.

115
Q

What are the common sites of thrombosis in HITT?

A

Venous thrombosis, pulmonary embolism, lower limb arterial thrombosis, cerebrovascular incidents, myocardial infarction.

116
Q

What is the goal of clotting factor replacement in hemophilia treatment?

A

25-30% normal factor activity level

117
Q

When is splenectomy indicated in ITP treatment?

A

If there is no response to medical management, requires repeated pharmacologic treatment, or severe recurrent hemorrhagic symptoms.

118
Q

Which factor does Dabigatran target?

A

Factor IIa (Thrombin)

119
Q

What is the patient outcome associated with HITT?

A

Mortality and morbidity, prolonged hospital stay.

120
Q

What immunizations are required before a splenectomy for ITP?

A

Meningococcus, pneumococcus, and H. influenza.

121
Q

What factor activity level is targeted before surgery in hemophilia patients?

A

50%

122
Q

What treatments are used for coagulopathy in liver failure?

A

Supportive treatments such as FFP and platelet transfusion for active bleeding or prior to procedures.

123
Q

At what platelet count does spontaneous internal bleeding occur?

A

Less than 10,000.

124
Q

Which factors do Rivaroxaban, Epixaban, and Edoxaban target?

A

Factor Xa

125
Q

What factors require vitamin K for activation?

A

Pro-coagulant factors II, VII, IX, X and anticoagulant factors Protein C and S.

126
Q

What factor activity level is required for limb- or life-threatening events in hemophilia?

A

80-100%

127
Q

What is another name for Factor XI deficiency?

A

Hemophilia C

128
Q

What are the hallmark features of Thrombotic Microangiopathies (TMAs)?

A

Microangiopathic Hemolytic Anemia (MAHA), thrombocytopenia, and ischemic tissue damage.

129
Q

What action should be taken when HITT is diagnosed?

A

Discontinuation of heparin therapy and replacing it with other anticoagulants like rHirudin or danaproid.

130
Q

What are the two main types of TMAs?

A

Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS).

131
Q

In which populations is Factor XI deficiency more prevalent?

A

Ashkenazi and Iraqi Jewish populations

132
Q

What is the most common acquired thrombophilia?

A

Autoantibodies against phospholipids in endothelial cell membranes

133
Q

What is the inheritance pattern of Factor XI deficiency?

A

Autosomal recessive

134
Q

What are the advantages of NOACs over vitamin K antagonists?

A

Fewer significant bleeding events, no monitoring or dietary restrictions, antidotes available

135
Q

What triggers Thrombotic Thrombocytopenic Purpura (TTP)?

A

Endothelial injury from an inciting event releases vWF and increases platelet consumption.

136
Q

What are the causes of thrombocytopenia?

A

Decreased production, increased destruction, immune-mediated, non-immune mediated, platelet consumption, infection/sepsis, and drugs.

137
Q

How does warfarin affect vitamin K?

A

Warfarin inhibits vitamin K reductase, keeping vitamin K inactive.

138
Q

What are primary hypercoagulable states?

A

Decreased anticoagulant factors (Protein C, S, Antithrombin) and increased pro-coagulant factors (Factor 5).

139
Q

What type of bleeding is not common in Factor XI deficiency?

A

Spontaneous hemarthroses

140
Q

What enzyme moderates vWF in TTP?

A

ADAMTS13.

141
Q

Which antibody is often associated with Lupus?

A

Lupus Anticoagulant

142
Q

How can the effects of warfarin be reversed?

A

By administering exogenous vitamin K, which bypasses the action of warfarin.

143
Q

What are some clotting factor disorders?

A

Hemophilias A, B, C, Von Willebrand Disease, DIC, Liver Failure, Vitamin K deficiency

144
Q

What are examples of immune-mediated platelet disorders?

A

Immune Thrombocytopenic Purpura (ITP) and Thrombotic Microangiopathies (TMAs).

145
Q

What enzyme reactivates vitamin K?

A

Vitamin K reductase.

146
Q

What is the most common hereditary bleeding disorder worldwide?

A

Von Willebrand Disease (vWD)

147
Q

What is Hemophilia A?

A

Deficiency of clotting factor VIII

148
Q

What can induce auto-antibodies against ADAMTS13 in TTP?

A

Pregnancy, vasculitis, chemo, bone marrow transplant, drugs, infections (HIV).

149
Q

What are the three main autoantibodies involved in acquired thrombophilia?

A

Lupus Anticoagulant, Anticardiolipin Ab, Anti B-2 Glycoprotein I

150
Q

What are the presentation signs of acquired TTP?

A

Marked thrombocytopenia with neurologic abnormalities, fever, and renal failure.

151
Q

What is the inheritance pattern of Von Willebrand Disease?

A

Autosomal dominant

152
Q

What is Hemophilia B also known as?

A

Christmas Disease

153
Q

What are secondary hypercoagulable states?

A

Pregnancy, immobilization, malignancy, post-operative state, OCPs, antiphospholipid syndrome, hyper-homocysteinemia, myeloproliferative disorder.

154
Q

What are the lab findings in TTP?

A

Normal coags, hemolytic anemia with schistocytes, decreased ADAMTS13 level.

155
Q

With which conditions is acquired thrombophilia commonly associated?

A

Other autoimmune or rheumatologic conditions, such as Lupus

156
Q

What does Von Willebrand Factor (vWF) mediate?

A

Platelet adhesion and aggregation, stabilizes Factor VIII

157
Q

What is the treatment for TTP?

A

Plasma exchange to remove antibodies and replace the enzyme.

158
Q

What percentage of hypercoagulable states are venous?

A

90%.

159
Q

What does endothelial cell damage lead to in acquired thrombophilia?

A

Thrombosis (arterial and venous)

160
Q

What are examples of Thrombotic Microangiopathies (TMAs)?

A

Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS).

161
Q

What are the key features of Hemolytic-Uremic Syndrome (HUS)?

A

Acute renal failure, MAHA, thrombocytopenia.

162
Q

What are the types of Von Willebrand Disease based on?

A

Different mutations

163
Q

What are the pregnancy-related complications of acquired thrombophilia?

A

Recurrent pregnancy loss/stillbirth

164
Q

What are the most common sites for venous thromboembolism (VTE)?

A

Extremity DVT and pulmonary embolism.

165
Q

What is the rarest type of Von Willebrand Disease?

A

Type 3

166
Q

What is the most frequent cause of HUS?

A

Shiga-toxin producing E. coli O157:H7 diarrhea.

167
Q

What are the common arterial complications of acquired thrombophilia?

A

Cerebrovascular events (stroke, TIA)

168
Q

What symptoms did the 30-year-old female present with?

A

Non-painful rash on lower extremities and mild gingival bleeding.

169
Q

What are some rare sites for VTE?

A

Mesenteric, cerebral, portal.

170
Q

What is the inheritance pattern of Hemophilias A and B?

A

X-linked recessive

171
Q

What are the physical exam findings in Von Willebrand Disease?

A

Petechiae, ecchymoses, hematoma

172
Q

What is the treatment for Shiga-toxin associated HUS (ST-HUS)?

A

Transfusion support and possibly dialysis.

173
Q

What are the common venous complications of acquired thrombophilia?

A

DVT, PE

174
Q

When should hypercoagulable states be suspected?

A

History of recurrent VTE, first VTE under 50 years old, first-degree relative with VTE under 50 years old, unexplained VTE.

175
Q

What percentage of Hemophilia cases arise as spontaneous mutations?

A

30%

176
Q

What is the treatment for complement-associated HUS (C-HUS)?

A

Eculizumab (Solaris).

177
Q

What laboratory findings are typical in Von Willebrand Disease?

A

Normal platelet levels, prolonged aPTT, normal PT/INR

178
Q

What is not usually associated with arterial thrombosis?

A

Hypercoagulable states.

179
Q

What is the clinical presentation of severe Hemophilia?

A

<1% of normal factor activity, spontaneous hemarthroses, hematomas, profuse hemorrhage with trauma or surgery

180
Q

What should be checked to distinguish between TTP and HUS?

A

ADAMTS13 level.

181
Q

What are the three plasma measurements used in diagnosing Von Willebrand Disease?

A

VWF Antigen, VWF Activity, Ristocetin Cofactor Activity

182
Q

What were the patient’s vital signs?

A

BP 110/70, P 80, R 16, T 97.8, SpO2 100%.

183
Q

What is the misnomer associated with Lupus Anticoagulant?

A

It is an antibody that prolongs aPTT by binding to phospholipid in the lab assay

184
Q

What is the treatment approach for Von Willebrand Disease?

A

Correction and prevention of bleeding during trauma, surgery

185
Q

What did the physical exam reveal about the patient’s skin?

A

Multiple small red punctate lesions that do not blanch on bilateral lower extremities.

186
Q

How does Lupus Anticoagulant act in the lab assay?

A

As an inhibitor, interfering with the test, falsely elevating the result

187
Q

What pharmacologic treatment is used in Type 1 Von Willebrand Disease?

A

DDAVP – Desmopressin

188
Q

What are the primary hypercoagulable states?

A

Protein C activity, Protein S activity, Antithrombin III activity, Factor V Leiden, Prothrombin gene mutation, Anticardiolipin antibody, Lupus anticoagulant, B-2 glycoprotein I.

189
Q

What determines the duration of anticoagulation in hypercoagulable states?

A

Diagnosis.

190
Q

What was the patient’s platelet count?

A

25,000.

191
Q

Is Lupus Anticoagulant clinically a bleeding disorder?

A

No

192
Q

What is Disseminated Intravascular Coagulation (DIC) also known as?

A

Consumptive Coagulopathy

193
Q

When should you suspect Lupus Anticoagulant?

A

With prolonged aPTT (and often INR) with no clinical bleeding, especially with history of thrombosis, miscarriage, or Lupus

194
Q

What characterizes Disseminated Intravascular Coagulation (DIC)?

A

Both clotting and bleeding

195
Q

When should labs ideally be ordered for hypercoagulable states?

A

Before initiating anticoagulation or 2 weeks after completing therapy.

196
Q

What is the leading cause of morbidity and mortality in severe Hemophilia?

A

Intracranial hemorrhage (ICH)

197
Q

What is the most likely diagnosis for the patient?

A

Immune Thrombocytopenic Purpura (ITP).

198
Q

What causes clotting in Disseminated Intravascular Coagulation (DIC)?

A

Circulating thrombin

199
Q

What effect does warfarin have on Protein C and S?

A

Decreases their levels.

200
Q

What are the clinical criteria for diagnosing Antiphospholipid Syndrome?

A

History of arterial or venous thrombus or pregnancy loss

201
Q

At what age do spontaneous hemarthroses typically occur in severe Hemophilia?

A

Between 9-18 months

202
Q

What is the typical platelet count in acute ITP?

A

Less than 20,000/μl.

203
Q

What lab test is used to diagnose Lupus Anticoagulant?

A

Specific LA assay with elevated titers

204
Q

What effect does heparin have on antithrombin activity?

A

Decreases measurable activity.

205
Q

What is the clinical presentation of moderate Hemophilia?

A

1-5% of normal factor activity, hemarthroses develop at 2-5 years of age

206
Q

What causes bleeding in Disseminated Intravascular Coagulation (DIC)?

A

Depletion of clotting factors and excessive fibrinolysis

207
Q

What is the typical platelet count in chronic ITP?

A

30,000 - 80,000/μl.

208
Q

What percentage of children with ITP experience spontaneous remission?

A

83%.

209
Q

Is there any data suggesting different treatment for APLS compared to other VTE?

A

No

210
Q

What is the clinical presentation of mild Hemophilia?

A

> 5% of normal factor activity, spontaneous bleeding uncommon, severe bleeding after trauma/surgery

211
Q

What are the major causes of Disseminated Intravascular Coagulation (DIC)?

A

Infections, immunologic reactions, obstetric complications, malignancies, liver failure, acute pancreatitis, envenomation, ARDS, trauma, shock, vascular disorders

212
Q

What is the antepartum prophylaxis for pregnancy in APLS?

A

ASA, heparin

213
Q

What is the most common complication of severe Hemophilia?

A

Acute hemarthrosis

214
Q

What percentage of adults with ITP experience spontaneous remission?

A

2%.

215
Q

What is homocysteine?

A

An amino acid derivative that interacts with endothelium, predisposing to atherosclerosis, arterial and venous thrombosis

216
Q

What are the types of antithrombin deficiency?

A

Type I (quantitative deficiency) and Type II (qualitative deficiency).

217
Q

What is the response rate to splenectomy in acute ITP?

A

71%.

218
Q

What is the prevalence of antithrombin deficiency in VTE cases?

A

1-2% of all VTE, 2.5% recurrent or age under 45.

219
Q

What gene defect is associated with hereditary hyperhomocysteinemia?

A

MTHFR gene

220
Q

What are the symptoms of acute hemarthrosis?

A

Tingling or burning sensation in joint, intense pain, swelling, hot, swollen and tender joint with erythema, pain, stiffness, limited mobility, maintained in flexed position

221
Q

What is the response rate to splenectomy in chronic ITP?

A

66%.

222
Q

What is the diagnosis method for antithrombin deficiency?

A

Functional assays and quantitative assays.

223
Q

What is the most common primary thrombophilia?

A

Factor V Leiden mutation.

224
Q

What are the clinical signs of ITP?

A

Petechiae on skin, mucous membranes, and GU system.

225
Q

What is the bleeding severity in ITP correlated with?

A

Platelet counts.

226
Q

What nutritional deficiencies can lead to acquired hyperhomocysteinemia?

A

B12, folate, pyridoxine

227
Q

What are some drugs associated with thrombocytopenia?

A

Acetaminophen, acetazolamide, allopurinol, alprenolol, amiodarone, ampicillin, amrinone, antazoline, aspirin, carbamazepine, cephalexin, cephalothin, chlorothiazide, chlorpheniramine, chlorthalidone, cimetidine, diazepam, desipramine, digitoxin, digoxin, diltiazem, diphenylhydantoin, furosemide, gentamicin, gold salts, heparin, heroin, imipramine, iopanoic acid, lidocaine, meprobamate, methicillin, methyldopa, minoxidil, morphine, novobiocin, para-aminosalicylic acid, penicillin, phenylbutazone, procainamide, quinidine, quinine, rifampin, spironolactone, sulfisoxazole, trimethoprim-sulfamethoxazole, valproic acid, vancomycin.

228
Q

What is the second most common primary thrombophilia?

A

Prothrombin G20210 A.

229
Q

What is the risk of spontaneous intracranial bleeding in ITP with platelet counts less than 5,000/μl?

A

High risk.

230
Q

What is the incidence of Heparin-induced Thrombocytopenia and Thrombosis (HITT)?

A

Approximately 6%.

231
Q

What are the treatment options for hypercoagulable states?

A

Heparin, argatroban, warfarin, direct oral anticoagulants (DOACs) like dabigatran, rivaroxaban, apixaban.

232
Q

How is ITP diagnosed?

A

By exclusion.

233
Q

Is there evidence that vitamin supplementation reduces thrombosis in hyperhomocysteinemia?

A

No

234
Q

Is hyperhomocysteinemia routinely checked as part of VTE work-up?

A

No

235
Q

What factors influence the choice of anticoagulant regimen?

A

Comorbidities, compliance, side effects, and safety.

236
Q

What lab findings are typical in ITP?

A

Isolated thrombocytopenia with normal WBC and RBC.

237
Q

What causes HITT?

A

Antibody reacts with heparin/platelet factor 4 complex.

238
Q

What is primary ITP?

A

ITP with no identified trigger.

239
Q

What is the duration of treatment for the first VTE episode in primary hypercoagulable states?

A

6-12 months, then weigh benefit vs. risk of bleeding complications.

240
Q

What are some factors predisposing to thrombosis?

A

Chronic DIC, immobility, indwelling lines, tumors compressing vessels

241
Q

What is secondary ITP?

A

ITP preceded by infection or immunologic condition.

242
Q

When does HITT typically onset?

A

More than 6 days of heparin exposure; amnestic response sooner.

243
Q

What are the most common complications of malignancy related to thrombosis?

A

DVT and PE

244
Q

When is lifelong therapy recommended in primary hypercoagulable states?

A

Life-threatening events, unusual sites, more than one inherited deficiency, homozygous Factor V Leiden, recurrent VTE.

245
Q

What are some secondary hypercoagulable states?

A

Obesity, trauma, OCPs, nephrotic syndrome, diabetes, paroxysmal nocturnal hemoglobinuria

246
Q

What is the prophylactic anticoagulation recommendation for patients with known deficiency but no history of VTE?

A

During high-risk states like surgery or pregnancy.

247
Q

What happens to the platelet count in HITT?

A

It drops to half of the baseline, may still be within the normal range.

248
Q

What is the most common acquired thrombophilia?

A

Antiphospholipid syndrome.

249
Q

Which type of heparin is more likely to cause HITT?

A

Bovine unfractionated heparin.

250
Q

What are the autoantibodies involved in antiphospholipid syndrome?

A

Lupus anticoagulant, anticardiolipin antibody, anti B-2 glycoprotein I.

251
Q

What are the symptoms of HITT?

A

Hemorrhage or thrombosis, skin necrosis, venous thrombosis, pulmonary embolism, lower limb arterial thrombosis, cerebrovascular incidents, myocardial infarction.

252
Q

What conditions are commonly associated with antiphospholipid syndrome?

A

Other autoimmune or rheumatologic conditions like lupus.

253
Q

What complications are associated with antiphospholipid syndrome?

A

Recurrent pregnancy loss, stillbirth, cerebrovascular incidents, DVT, PE.

254
Q

What is the outcome for patients with HITT?

A

Associated with mortality and morbidity; prolonged hospital stay.

255
Q

What is the action to take when HITT is diagnosed?

A

Discontinuation of heparin therapy and replacing it with other anticoagulants (rHirudin, danaproid).

Hematology/Immunology Part 1 (12 decks)

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