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Heme / Onc > SM_252b: Coags and Inherited Bleeding > Flashcards

SM_252b: Coags and Inherited Bleeding Flashcards

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1
Q

When there is vascular injury, ___

A

When there is vascular injury, thrombogenic components of subendothelial vessel wall become exposed and a hemostatic clot of platelets and fibrin form

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2
Q

Describe physiologic antithrombotic mechanisms

A

Physiologic antithrombotic mechanisms

  • Normal endothelium generates substances that inhibit coagulation: inhibitors of platelet activation
  • Antithrombotic mechanisms: antithrombin III, protein C and S, tissue factor pathway inhibitor, thrombomodulin, fibrinolytic system
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3
Q

Primary hemostasis is ___

A

Primary hemostasis is vasoconstriction and aggregated platelets temporarily plug the injured vessel

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4
Q

Secondary hemostasis is ____

A

Secondary hemostasis is when fibrin formation occurs to form a more permanent clot

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5
Q

Describe coagulation basics

A

Coagulation basics

  • Clotting factors are proteins -> converted to proteases during coagulation -> cleave prothrombin to thrombin -> thrombin cleaves fibrinogen to fibrin -> fibrin monomers polymerize to form the strands of the clot
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6
Q

Coagulation involves stages of ____, ____, and ____

A

Coagulation involves stages of initiation, amplification, and propagation

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7
Q

In initation of coagulation, ____

A

In initation of coagulation, a small amount of thrombin is produced

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8
Q

In amplification of coagulation, ____

A

In amplification of coagulation, thrombin further activates FV, VIII, and XI for a large burst of thrombin generation

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9
Q

Describe natural inhibitors of procoagulants

A

Natural inhibitors of procoagulants

  • Thrombin inhibited by antithrombin and thrombomodulin
  • Factor Va and VIIIa inhibited by activated protein C and its co-factor protein S
  • Factor Xa and TF-factor VIIa is inhibited by tissue factor pathway inhibitor
  • Factor XIa is inhibited by the protease nexin 2
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10
Q

____ is progressive breakdown of a thrombus

A

Fibrinolysis is progressive breakdown of a thrombus

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11
Q

tPA is the initator of fibrinolysis and converts ____ to ____

A

tPA is the initator of fibrinolysis and converts fibrin-bound plasminogen to plasmin

  • Plasmin lyses fibrin to soluble fibrin degradation products
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12
Q

____ are fragments of cross-linked fibrin that are soluble fibrin degradation products

A

D-dimers are fragments of cross-linked fibrin that are soluble fibrin degradation products

  • Reflect amount of fibrin degradation
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13
Q

Describe regulation of hemostasis

A

Regulation of hemostasis

  • Sequential steps activate specific procoagulants -> thrombin generation and fibrin formation
  • Each step is regulated by clotting inhibitors
  • Fibrinolysis results in clot lysis
  • Bleeding or thrombosis can occur when the hemostatic balance between clotting factors and inhibitors is altered
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14
Q

aPTT measures the ___ pathway

A

aPTT measures the intrinsic pathway

  • HMWK, FXII, FXI, FIX, FVIII
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15
Q

PT measures the ____ pathway

A

PT measures the extrinsic pathway

  • FVII
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16
Q

Describe causes of prolonged PT and normal aPTT

A

Prolonged PT and normal aPTT

  • FVII deficiency
  • Early Vitamin K deficiency (FII, FVII, FIX, FX)
  • Liver disease
  • Warfarin therapy (Vitamin K antagonist)
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17
Q

Describe causes of prolonged aPTT and normal PT

A

Prolonged aPTT and normal PT

  • FVIII or IX deficiency
  • Factor XI or XII deficiency
  • Decreases prekallikrein or high molecular weight kininogen
  • Inhibitor (lupus anticoagulant, FVIII inhibitor)
  • Anticoagulant: heparin
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18
Q

Describe causes of prolonged PT and prolonged aPTT

A

Prolonged PT and prolonged aPTT

  • FII, FV, or FX deficiency
  • Hypofibrinogenemia or dysfibrinogenemia
  • Vitamin K deficiency (FII, FVII, FIX, FX)
  • Liver disease
  • Warfarin therapy (Vitamin K antagonist)
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19
Q

Correction to normal on PT or PTT mixing study indicates ____

A

Correction to normal on PT or PTT mixing study indicates factor deficiencies

  • E.g. hemophilia, Vitamin K deficiency, warfarin therapy
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20
Q

No correction to normal on PT or PTT mixing study indicates ____

A

No correction to normal on PT or PTT mixing study indicates factor inhibitor present

  • Acquired specific factor inhibitor or lupus anticoagulant
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21
Q

Even if PT and aPTT are normal, ____ can be present

A

Even if PT and aPTT are normal, mild factor deficiency can be present

  • PT or PTT become abnormal only when coagulation factor levels drop below 30-40%
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22
Q

Describe causes of bleeding

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A

Causes of bleeding

  • Vascular / connective tissue: hereditary hemorrhagic telengiectasias, Ehlers-Danlos, scurvy (Vitamin C deficiency)
  • Hematologic: coagulopathy, platelet disorder (thrombocytopenia, platelet function abnormality)
  • Medications: anticoagulants, antiplatelets
23
Q

Describe inherited bleeding disorders

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A

Inherited bleeding disorders

  • Vascular: hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu), Ehlers-Danlos syndrome
  • Clotting factor deficiencies: VIII or IX, V / VII / X / XI, XIII, von Willebrand’s disease, hypofibrinogenemia / dysfibrinogenemia
  • Platelet disorders
24
Q

Describe clues to a bleeding disorder

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A

Clues to a bleeding disorder

  • Abnormal bruising especially spontaneous
  • Recurrent epistaxis or gum bleeding
  • Prior surgical bleeding
  • Dental extractions complicated by bleeding
  • Menorrhagia
  • History of soft tissue or joint hemorrhage especially with normal activities
  • Iron deficiency or need for transfusions
  • Family history of bleeding disorders
25
Describe clues to bleeding disorders on physical exam
Clues to bleeding disorders on physical exam * Petechiae: thrombocytopenia * Ecchymoses: clotting factor disorder, von Willebrand's disease, connective tissue disorders, medications, platelet disorders * Soft tissue hemorrhage: deficiency of a coagulation factor * Telangiectasias (skin, nasal, oral, nails): hereditary hemorrhagic telangiectasias
26
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is ____ that presents with ____ and \_\_\_\_
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is abnormality in the vascular wall that presents with mucocutaneous telangiectasias and arteriovenous malformations
27
\_\_\_ is an autosomal dominant disorder that may present with recurrent bleeding from nose or GI tract
Hereditary hemorrhagic telangiectasia is an autosomal dominant disorder that may present with recurrent bleeding from nose or GI tract
28
Diagnosis of hereditary hemorrhagic telangiectasia is \_\_\_
Diagnosis of hereditary hemorrhagic telangiectasia is clinical * No specific lab tests
29
Hemophilia A results from a ___ deficiency
Hemophilia A results from a Factor VIII deficiency * Inversion of intron 22 on factor VIII
30
Hemophilia B results from a ____ deficiency
Hemophilia B results from a Factor IX deficiency
31
Hemophilia A or B severity depends on \_\_\_
Hemophilia A or B severity depends on level of factor activity * Severe hemophilia: spontaneous joint hemorrhages (hemarthroses), intramuscular hemorrhage, intracranial hemorrhage, serious bleeding with trauma or injury * Moderate and mild: bleeding with surgery or dental procedures, bleeding with trauma or injury
32
Women are ___ of hemophilia A or B
Women are carriers of hemophilia A or B * Can have clinically significant low factor levels (\< 40%) * Can have beeding complications
33
Joint bleeds are typically ____ in hemophilia
Joint bleeds are typically spontaneous in hemophilia
34
Describe treatment of hemophilia
Hemophilia treatment * Severe hemophilia: prevention or prompt treatment of bleeding, early treatment and prophylactic therapy for joint bleeds * Mild hemophilia: prevention of bleeding with surgery or trauma
35
\_\_\_\_ is used to treat bleeding in hemophilia due to mild Factor VIII deficiency
Desmopressin is used to treat bleeding in hemophilia due to mild Factor VIII deficiency
36
Antibodies can develop in patients with hemophilia against factor concentrates, leading to \_\_\_\_
Antibodies can develop in patients with hemophilia against factor concentrates, leading to resistance to treatment
37
\_\_\_ are used to treat hemophilia due to Factor VIII or IX deficiency
Factor concentrates are used to treat hemophilia due to Factor VIII or IX deficiency
38
VWF functions to ____ and \_\_\_\_
VWF functions to bind platelet to the endothelium and increases circulating half-life of Factor VIII because binding protein for Factor VIII
39
Describe vWF
vWF * Gene on chromosome 12 * Protein synthesized by endothelial cells and megakaryocytes * vWF multimers are stored in Weibel-Palade bodies or alpha granules in platelets
40
Describe lab tests for von Willebrand disease
Lab tests for von Willebrand disease * Screening testsL bleeding time, PTT * Specific tests: von Willebrand antigen, von Willebrand activity, Factor VIII, and Von Willebrand multimer analysis
41
Describe types of von Willebrand disease
Types of von Willebrand disease * Type 1: partial quantitative deficiency of vWF antigen * Type 2: qualitative defect of vWF antigen * Type 3: severe quantitative deficiency (similar to severe hemophilia A)
42
Describe Type 1 von Willebrand disease
Type 1 von Willebrand disease * Most common * Decrease in vWF, von Willebrand activity, and FVIII * Symptoms: easy bruising, epistaxis, menorrhagia, bleeding with dental extractions or tonsillectomy * Not all patients with low vWF levels will bleed * Patients with Type O blood have lower vWF levels than patients with other blood types
43
Describe Type 2 von Willebrand disease
Type 2 von Willebrand disease * Functional defect of protein * Type 2A: absence of high molecular weight multimers * Type 2B: increasing binding of vWF to platelet glycoprotein 1b resulting in vWF and platelet clearance from circulation * Type 2N: abnormal binding of FVIII to vWF -\> increased clearance of FVIII * Type 2M: impaired binding to collagen or platelet glycoprotein 1b
44
45
Describe Type 3 von Willebrand disease
Type 3 von Willebrand disease * Severe deficiency of von Willebrand protein * FVIII levels are very low * Can have severe bleeding and similar to severe hemophilia
46
Treatment of von Willebrand disease involves ___ or \_\_\_
Treatment of von Willebrand disease involves desmopressin or vWF concentrate
47
Hemophilia C is \_\_\_\_
Hemophilia C is Factor XI deficiency * Autosomal
48
Describe Factor XI
Factor XI * Activates Factor IX -\> results in thrombin generation * Thrombin activates Factor XI -\> creates positive feedback loop that leads to further thrombin generation * Thrombin generation leads to thrombin activatable fibrinolysis inhibitor * Factor XI deficiency: less thrombin generation and less TAFI -\> increased fibrinolysis
49
Describe bleeding in Factor XI deficiency
Factor XI deficiency bleeding * Variable hemorrhage * Bleeding with surgery or trauma * Spontaneous joint or muscle hemorrhage unlikely * Mucocutaneous bleeding: ecchymoses, gum bleeding, epistaxis, menorrhagia * Delayed post-surgical bleeding
50
Factor XI deficiency is treated with ____ or \_\_\_\_
Factor XI deficiency is treated with fresh frozen plasma or antifibrinolytic agents such as e-aminocaproic acid / transexamic acid
51
\_\_\_ deficiency is the only coagulation factor not reflected in PT or PTT
Factor XIII deficiency is the only coagulation factor not reflected in PT or PTT * Cross-links fibrin * Homozygotes have severe deficiency * Treat with FFP, cryoprecipitate, or plasma-derived factor XIII concentration (fibrogammin)
52
Describe clotting factor deficiences NOT associated with bleeding
Clotting factor deficiences NOT associated with bleeding * Contact activation factors: factor XII, HMWK, prekallikrein * Result in elevated PTT but no increased risk of bleeding
53
Describe disorders of fibrinogen
Disorders of fibrinogen * Congenital afibrinogenemia * Hypofibrinogenemia * Dysfibrinogenemia Treat with cryoprecipitate or plasma-derived fibrinogen concentrate (Riastap)

Heme / Onc (41 decks)

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