Bleeding, Thrombic and Fibrinolytic Disorders

Question 1

Immune Thrombocytopenia Purpura (ITP)

Answer 1

Antibodies bind to platelets resulting in their premature destruction.
Megakaryocytes may be injured as well - decreased production
Reduced TPO levels

Question 2

ITP Causes

Answer 2

Commonly occurs in childhood
Abrupt onset after viral infxn
Medications
Autoimmune

Question 3

ITP S/S

Answer 3

Blood blisters in mouth
Petichiae, purpura
spontaneous bruising
nosebleeds
gingival bleeding
retinal hemorrhage
menaggerhea
Menela, hematuria

Question 4

ITP Lab Findings

Answer 4

Thrombocytopenia
Normal RBC morphology
Prolonged bleeding time
\+/- anemia
PT/PTT normal
Normal or increased megakaryocytes

Question 5

ITP Tx

Answer 5

Prednisolone
IVIG
Platelet transfusion (temporary)
Dexamethasone

Question 6

Thrombotic Thrombocytopenia Purpura (TTP)

Answer 6

90% untreated mortality
Antibodies against ADAMTS-13 which cleaves clots
Extensive platelet aggregation and fibrin bridging
RBC sheering leads to destruction

Question 7

TTP lab findings

Answer 7

Thrombocytopenia

Hemolytic anemia

Question 8

TTP Causes

Answer 8

Generally occurs in adults
Primary: autoimmune
Secondary: Cancer, BMT, Pregnancy, Meds, HIV

Question 9

Which organs are damage from TTP?

Answer 9

Kidneys and Brain

Question 10

5 Main characterictics of TTP?

Answer 10

Thrombocytopenia
MIcroangiopathic hemolytic anemia
Neurologic symptoms
Kidney failure
Fever (75%)

Question 11

TTP SYmptoms

Answer 11

Malaise
Diarrhea
Thrombocytopenia, bruising, bleeding
Organ damage

Question 12

TTP Lab Findings

Answer 12

Microangiopathic hemolytic anemia
Elevated D.Bili
Decreased serum haptoglobin
Elevated LDH

Question 13

TTP Tx

Answer 13

Plasma exchange
Early dx and tx
90% untreated mortality

Question 14

Hemolytic Uremic Syndrome

Answer 14

Most commonly secondary to E. Coli but may be caused by others
Caused by endothelial damage secondary to bacterial toxins
Inflammation, leukocyte activation, platelet activation, thromboses, RBC’s destroyed

Question 15

Uremia = ?

Answer 15

Kidney failure

Question 16

HUS S/S

Answer 16

Bloody diarrhea
Abdominal pain
Decreased urine output
Hematuria
Renal Faiure
HTN
Neurologic changes
Edema

Question 17

HUS Tx

Answer 17

Transfuse RBC’s and platelets

Dialysis if uremia

Question 18

3 signs of HUS?

Answer 18

Microangiopathic hemolytic anemia
Acute kidney injury/renal failure
Thrombocytopenia

Question 19

Who is HUS more common in?

Answer 19

Children.

Question 20

Henoch-Schonelin Purpura

Answer 20

AKA IgA Vasculitis
characterized by::
Palpable purpura
Arthritis, arthralgias
Abd pain
Renal disease

Question 21

Henoch-Schonelin Purpura Facts

Answer 21

90% of cases occur in children 3 - 15 years old
Can be triggered by streptococcal URI
Should not have thrombocytopenia or coagulopathy

Question 22

Henoch-Schonelin Purpura Tx

Answer 22

NSAIDS and Glucocorticoids
Late-onset renal failure. Follow up needed
Biopsy of skin lesions, CBC, CMP, urinalysis

Question 23

Hemophilia

Answer 23

X-linked recessive trait
1:5,000 males
Females are carriers

Question 24

Hemophilia A

Answer 24

Factor VIII Deficiency
80% of patients
2/3 have severe disease

Question 25

Hemophilia B

Answer 25

Factor IX Deficiency | Also known as christmas disease

Question 26

Hemophilia C

Answer 26

Factor XI Deficiency Rare 1:1,000,000

Question 27

Bleeding sites in hemophilia

Answer 27

Spontaneous hemarthrosis Risk of intracerebral hemorrhage Joints, skin, muscle, GU, GI

Question 28

Hemophillic Arthropathy

Answer 28

Arthritis causes by bleeding into the joints

Question 29

Hemophilia Tx

Answer 29

``` Lifelong replacement of deficient clotting factor Usually 3 times per week Avoid Aspirin Cryoprecipitate works DDAVP ```

Question 30

Hemophilia Dx

Answer 30

Prolonged PTT PT, bleeding time, platelets are normal Dx is confirmed by decrease in factor VIII IX or Xi

Question 31

von Willebrand DIsease factor

Answer 31

Binds platelets to form initial platelet plug | Binds Factor VIII to prolong its half life

Question 32

von WIllebrand Disease (vWD)

Answer 32

Autosomal dominant affecting both sexes Most common inherited bleeding disorder Type 1: 75 - 80% of patients They don't have enough

Question 33

vWD Symptoms

Answer 33

Easy bruising, skin bleeding, prolonged bleeding, bleeding from mucosal surfaces Nosebleeds > 10 mins in childhood Lifelong easy bruising Heavy menstruation

Question 34

vWD lab fndings

Answer 34

Plasma vDW antigen Factor VIII activity PT normal PTT normal or prolonged

Question 35

vWD Tx

Answer 35

Treat symptomatically DDAVP Avoid aspirin

Question 36

Disseminated Intravascular Coagulation (DIC)

Answer 36

Simultaneous thrombosis and hemorrhage Massive release of tissue factor Tissue factor sets coag system in place Coag occurs Clotting factors and inhibitors are consumed clots trap circulation leading to ischemia

Question 37

DIC activates?

Answer 37

The complement and kinin systems which leads to shock.

Question 38

Causes of DIC

Answer 38

MANY | Cancer, Obstetric complications, Sepsis, Infxn, Massive tissue injury (trauma, burns), Snake bites

Question 39

DIC summary

Answer 39

Massive tissue release causes a massive release of clotting factors and inhibitors, these are all used up so clots roam freely and bleeding does not stop. This causes simultaneous thrombosis and hemorrhage and multi-system, failure.

Question 40

DIC S/S

Answer 40

``` Thrombosis and hemorrhage Petechiae, purpura, gangrene Renal, liver failure Acute illness Slow onset in cancer patients ```

Question 41

DIC lab findings

Answer 41

Thrombocytopenia Prolonged PT and PTT Low Fibrinogen Schistocytes

Question 42

DIC Tx

Answer 42

Underlying cause Anticoagulants to prevent imminent death Platelets < 50k = platelets or coag factor FFP or cryo to keep fibro > 100 if significantly elevated PT/INR

Question 43

Things that lead to hypercoagulable states?

Answer 43

``` Atherosclerosis DM Tobacco use Cancers Elevated platelets ```

Question 44

Conditions that accelerate clotting

Answer 44

``` Pregnancy OCP Postsurgical state Malignancy Hereditary clotting disorders ```

Question 45

Other abnormal clotting risk factors

Answer 45

Stasis/immobility Low cardiac output Obesity Sleep apnea

Question 46

Protein C deficiency

Answer 46

Protein C inactivates factors V and VIII thereby inhibiting coagulation. A deficiency leads to prolonged action of factors V and VIII.

Question 47

Protein C S/S

Answer 47

``` Nothing recognized until clots form Thrombosis DVT Pulmonary embolism Thromboplebitis ```

Question 48

Protein C work up

Answer 48

``` Protein C PTT, PT Thrombin time Bleeding time Hereditary history ```

Question 49

Protein C Tx

Answer 49

Anticoagulants if high risk (surgery, hospitalization) | Chronic anticoagulation of hx of thrombosis

Question 50

Protein S deficiency

Answer 50

Protein S is needed for proper fxn of protein C. S/S and Tx are the SAME BOOOOOOM!!!!!!!!!!!!!

Question 51

Antithrombin III Deficiency (A3D)

Answer 51

``` Hereditary disorder that is very serious Usually autosomal dominant Recurrent venous thrombosis, PE, repetitive miscarriage 60% have thrombotic episodes. Peak age of onset is 15 - 35 years old. ```

Question 52

Antithrombin is a major inhibitor of factors ____ and ___.

Answer 52

X and IX Without this, you have an increased tendency to form clots. 50% reduction = clots.

Question 53

A3D W/U

Answer 53

Antithrombin-heparin cofactor assay** | Standard coag tests should be normal

Question 54

A3D Tx?!?

Answer 54

Same as Protein C and S Disorders!!!! | BOOM BABBY!!!!! SHITCHEA

Question 55

Factor V Leiden

Answer 55

Most common genetic disorder to cause DVT. 5% of general population Venous blood is more likely to clot Lack of factor V leiden, works with protein C Same symptoms as protein C.

Question 56

Factor V Leiden Tx.

Answer 56

Same as all the others. Simply stunning. It must be my birthday.

Question 57

Antiphospholipid antibody

Answer 57

Autoimmune hypercoagulable state caused by said antibodies. Lead to arterial and venous clots Pregnancy complications End organ damage

Question 58

Types of antiphospholipid antibodies

Answer 58

Lupus anticoagulant Anti-cardiolipin antibody Anti-beta2 - glycoprotein-I

Question 59

Antiphospholipid Dx

Answer 59

Presence of antibodies mentioned in previous slide PLUS a history of thrombosis

Question 60

Antiphospholipid Tx

Answer 60

aspirin, warfarin