Bleeding and Bruising Disorders

Question 1

What is the normal platelet count per microliter in healthy blood?

Answer 1

150,000-300,000

Question 2

Thrombocytopenia platelet count

Answer 2

<70,000

Question 3

When does a bleeding tendency develop - severe thrombocytopenia

Answer 3

<10,000 - 20,000/ml

Question 4

Aplastic anemia

Answer 4

loss of all hematopietic cells

Question 5

leukemia

Answer 5

replacement of normal cells with tumor cells

Question 6

Infectious agents that affect megakaryocytes?

Answer 6

Rubella

Question 7

What is the genetic pattern of inheritence for Hemophilia

Answer 7

Sex-linked, located on the X chromosome

Question 8

Who is affected by hemophilia, daughters or sons?

Answer 8

Daughters will be asymptomatic carriers

Sons with have the bleeding disease

Question 9

Which hemophilia is more common and more severe?

Answer 9

Hemophilia A (VIII) is more common and more severe

Question 10

Which hemophilia is associated with factor IX deficiency?

Answer 10

Hemophilia B (Christmas disease)

Question 11

Hemophilia: characteristic clinical findings

Answer 11

• subcutaneous hematomoas

- hemarthrosis (bleeding around joint) –>joint deformity

Question 12

What is the most significant abnormality in Hemophilia (lab)?

Answer 12

Prolonged activated partial thromboplastin time (PTT)

[intrinsic pathway]

Question 13

What two things are normal in hemophilia?

Answer 13

1. Prothrombin (PT)
2. Bleeding time

[extrinsic pathway]

Question 14

Why are hemophiliacs at high risk for hepatitis B or C or HIV?

Answer 14

frequent recombinant IV transfusions

Question 15

Where does Von Willebrand Factor come from?

Answer 15

1. endothelial cells (inner wall of blood cells)

2. Megakaryocytes

Question 16

In the plasma what does vWF bind to and protect to promote sealing off the subendothelial injury?

Answer 16

vWF binds to and protects Factor VIII

important for platelet adhesion

so, when you don’t have vWF Factor VIII doesn’t work as well

Question 17

What is the most common hereditary coagulation abnormality in humans?

Answer 17

von Willebrand Disease (1 in 100)

Question 18

Which two types of vWD are autosomal dominant?

Answer 18

Types 1 and 2 and autosomal dominant

Question 19

Which type of vWD is autosomal recessive?

Answer 19

Type 3

Question 20

Which of the 3 types of vWD is most common?

Answer 20

Type 1, which is mild. Only an issue during minor surgery and dental extractions

Question 21

Which chromosome is the vWF gene located?

Answer 21

chromosome 12

Question 22

Which type of vWD is the most severe?

Answer 22

Type 3

-little to no vWD

Question 23

What are the clinical manifestations of type 1 and 2 vWD

Answer 23

• easy bruising
• nosebleeds
• bleeding gums
• Heavy menstrual periods

Question 24

What are the clinical manifestations of type 3 vWD?

Answer 24

• Life threatening GI hemorrhage

- Hemarthrosis

Question 25

Treatment for vWD (3)

Answer 25

1. Factor VIII 2. vWF concentrate 3. Cryoprecipitates

Question 26

Disseminated Intravascular Coagulation (progression)

Answer 26

1. Microvasculature fibrin thrombi 2. Consumption of platelets and coagulation factors 3. Hemorrhagic diathesis

Question 27

A serious and often fatal, acquired thrombohemorrhagic disorder occuring as a secondary complication to a variety of diseases and emergency situations

Answer 27

Disseminated Intravascular Coagulation

Question 28

Name 6 causes of DIC

Answer 28

1. Massive Trauma (shock) 2. Gram-negative sepsis 3. Obstetric Emergencies 4. Metastatic and primary carcinomas (ex. mucin-producing adenocarcinomas) 5. Hematopoietic Disorders (AML) [granules start clotting cascade] 6. Other: Surgery, Burns, Hypotensive states

Question 29

What are some examples of DIC via the intrinsic pathway?

Answer 29

Widespread injury to endothelial cells

Question 30

Compare the Extrinsic and Intrinsic Pathway

Answer 30

Extrinsic- triggered by release of procoagulant issue factor into circulation Intrinsic- Widespread injury to the endothelial cells (ex. Burn, heat stroke, viral, rickettsial infection, antibody-antigen complexes)

Question 31

Obstetrical accidents that can trigger extrinsic pathway in DIC

Answer 31

1. Premature separation of placenta (abruptio placenta) 2. retained dead fetus 3. septic abortions 4. amniotic fluid embolism

Question 32

Microvascular obstruction is associated with widespread ischemic changes particularly where?

Answer 32

1. Brain 2. Kidneys 3. Skin 4. Lungs 5. Adrenals 6. GI tract

Question 33

What is the cause of massive adrenal hemorrhages, shock and death?

Answer 33

Meningococcemia - massive adrenal hemorrhages (Waterhouse-Friderichsen Syndrome)

Question 34

What skin finding is associated with Waterhouse-Friderichsen Syndrome?

Answer 34

purpura

Question 35

Schistocytes

Answer 35

fragmented erythrocytes

Question 36

What is the consequence of consumption of activated platelets?

Answer 36

thrombocytopenia

Question 37

What will be seen on labs with depletion in clotting factors?

Answer 37

1. Increased PT time (extrinsic) and PTT time (intrinsic) 2. Decreased plasmin fibrinogen level 3. Elevated Fibrinopeptide A and D dimers

Question 38

D dimer elevation is an indication of what?

Answer 38

fibrinolytic activation | high D dimer? -->bleeding diathasis

Question 39

Symptoms of DIC

Answer 39

Renal symptoms: mild azotemia to acute renal failure from bilateral cortical necrosis Acute respiratory distress syndrome Hemorrhage of GI ulcers

Question 40

What are 3 hallmarks of the bleeding diathesis?

Answer 40

1. cerebral hemorrhage 2. ecchymosis 3. Hematuria

Question 41

TX for DIC

Answer 41

Heparin for cycle of intravascular coagulation Platelets and clotting factors for bleeding diathesis

Question 42

Hereditary Disorder of Platelets (Bernard- Soulier Syndrome)

Answer 42

"Giant Platelet Syndrome" - Autosomal recessive - Big platelets - Fewer platelets

Question 43

How is diagnosis of Hereditary Disorder of Platelets (Bernard-Soulier Syndrome) made?

Answer 43

1. Thromocytopenia | 2. Giant platelets on blood smear

Question 44

Why does Hereditary Disorder of Platelets (Bernard-Soulier Syndrome) occur?

Answer 44

membrane protein defect

Question 45

When does Hereditary Disorder of Platelets manifest?

Answer 45

infancy or childhood

Question 46

How does Hereditary Disorder of Platelets (Bernard- Soulier Syndrome) manifest?

Answer 46

- ecchymosis - epistaxis - gingival bleeding