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Pathology > Bleeding and Bruising Disorders > Flashcards

Bleeding and Bruising Disorders Flashcards

1
Q

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

A

150,000-300,000

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2
Q

Thrombocytopenia platelet count

A

<70,000

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3
Q

When does a bleeding tendency develop - severe thrombocytopenia

A

<10,000 - 20,000/ml

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4
Q

Aplastic anemia

A

loss of all hematopietic cells

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5
Q

leukemia

A

replacement of normal cells with tumor cells

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6
Q

Infectious agents that affect megakaryocytes?

A

Rubella

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7
Q

What is the genetic pattern of inheritence for Hemophilia

A

Sex-linked, located on the X chromosome

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8
Q

Who is affected by hemophilia, daughters or sons?

A

Daughters will be asymptomatic carriers

Sons with have the bleeding disease

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9
Q

Which hemophilia is more common and more severe?

A

Hemophilia A (VIII) is more common and more severe

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10
Q

Which hemophilia is associated with factor IX deficiency?

A

Hemophilia B (Christmas disease)

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11
Q

Hemophilia: characteristic clinical findings

A
  • subcutaneous hematomoas

- hemarthrosis (bleeding around joint) –>joint deformity

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12
Q

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

A

Prolonged activated partial thromboplastin time (PTT)

[intrinsic pathway]

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13
Q

What two things are normal in hemophilia?

A
  1. Prothrombin (PT)
  2. Bleeding time

[extrinsic pathway]

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14
Q

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

A

frequent recombinant IV transfusions

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15
Q

Where does Von Willebrand Factor come from?

A
  1. endothelial cells (inner wall of blood cells)

2. Megakaryocytes

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16
Q

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

A

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

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17
Q

What is the most common hereditary coagulation abnormality in humans?

A

von Willebrand Disease (1 in 100)

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18
Q

Which two types of vWD are autosomal dominant?

A

Types 1 and 2 and autosomal dominant

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19
Q

Which type of vWD is autosomal recessive?

A

Type 3

20
Q

Which of the 3 types of vWD is most common?

A

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

21
Q

Which chromosome is the vWF gene located?

A

chromosome 12

22
Q

Which type of vWD is the most severe?

A

Type 3

-little to no vWD

23
Q

What are the clinical manifestations of type 1 and 2 vWD

A
  • easy bruising
  • nosebleeds
  • bleeding gums
  • Heavy menstrual periods
24
Q

What are the clinical manifestations of type 3 vWD?

A
  • Life threatening GI hemorrhage

- Hemarthrosis

25
Q

Treatment for vWD (3)

A
  1. Factor VIII
  2. vWF concentrate
  3. Cryoprecipitates
26
Q

Disseminated Intravascular Coagulation (progression)

A
  1. Microvasculature fibrin thrombi
  2. Consumption of platelets and coagulation factors
  3. Hemorrhagic diathesis
27
Q

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

A

Disseminated Intravascular Coagulation

28
Q

Name 6 causes of DIC

A
  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
29
Q

What are some examples of DIC via the intrinsic pathway?

A

Widespread injury to endothelial cells

30
Q

Compare the Extrinsic and Intrinsic Pathway

A

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)

31
Q

Obstetrical accidents that can trigger extrinsic pathway in DIC

A
  1. Premature separation of placenta (abruptio placenta)
  2. retained dead fetus
  3. septic abortions
  4. amniotic fluid embolism
32
Q

Microvascular obstruction is associated with widespread ischemic changes particularly where?

A
  1. Brain
  2. Kidneys
  3. Skin
  4. Lungs
  5. Adrenals
  6. GI tract
33
Q

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

A

Meningococcemia - massive adrenal hemorrhages (Waterhouse-Friderichsen Syndrome)

34
Q

What skin finding is associated with Waterhouse-Friderichsen Syndrome?

A

purpura

35
Q

Schistocytes

A

fragmented erythrocytes

36
Q

What is the consequence of consumption of activated platelets?

A

thrombocytopenia

37
Q

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

A
  1. Increased PT time (extrinsic) and PTT time (intrinsic)
  2. Decreased plasmin fibrinogen level
  3. Elevated Fibrinopeptide A and D dimers
38
Q

D dimer elevation is an indication of what?

A

fibrinolytic activation

high D dimer? –>bleeding diathasis

39
Q

Symptoms of DIC

A

Renal symptoms: mild azotemia to acute renal failure from bilateral cortical necrosis

Acute respiratory distress syndrome

Hemorrhage of GI ulcers

40
Q

What are 3 hallmarks of the bleeding diathesis?

A
  1. cerebral hemorrhage
  2. ecchymosis
  3. Hematuria
41
Q

TX for DIC

A

Heparin for cycle of intravascular coagulation

Platelets and clotting factors for bleeding diathesis

42
Q

Hereditary Disorder of Platelets (Bernard- Soulier Syndrome)

A

“Giant Platelet Syndrome”

  • Autosomal recessive
  • Big platelets
  • Fewer platelets
43
Q

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

A
  1. Thromocytopenia

2. Giant platelets on blood smear

44
Q

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

A

membrane protein defect

45
Q

When does Hereditary Disorder of Platelets manifest?

A

infancy or childhood

46
Q

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

A
  • ecchymosis
  • epistaxis
  • gingival bleeding

Pathology (15 decks)

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