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Flashcards in Bleeding and Thrombocytopenia Exercise Deck (13):
1

List the clincial characteristics for primary (platelet) bleeding

  • Site
  • Bleeding after minor cuts?
  • Petechiae
  • Ecchymoses
  • Hemarthroses, muscle hematomas
  • Bleeding after surgery

PRIMARY

  • Site: Skin, mucous membranes (gingivae, nares, GI, GU tract)
  • Bleeding after minor cuts? yes *sorry this was wrong before*
  • Petechiae: present
  • Ecchymoses: small, superficial
  • Hemarthroses, muscle hematomas: rare
  • Bleeding after surgery: intermediate, mild

 

 

2

List the clincial characteristics for secondary (clotting factor) bleeding

  • Site
  • Bleeding after minor cuts?
  • Petechiae
  • Ecchymoses
  • Hemarthroses, muscle hematomas
  • Bleeding after surgery

  • Site: deep in soft tissues (joints, muscles)
  • Bleeding after minor cuts? Not usually
  • Petechiae: Absent
  • Ecchymoses: large, palpable
  • Hemarthroses, muscle hematomas: common
  • Bleeding after surgery: delayed, severe

3

What is the key characteristic of a blood smear in a patient with ITP?

Large platelets--almost the size of RBCs. Indicates immature platelets and high cell turnover. 

4

How do you treat an adult with ITP?

How do you treat a child with ITP?

Adults: IVIG. IVIG is thought to overwhelm macrophages so they destroy fewer platelets coated with auto-abs. Treat with steroids after to decrease auto-ab production. 

Children: do not need treatment as most cases resolve sponatenously. Peak incidence is early childhood (1-6yo). More likely to be due to virus. Only 25% of cases result in chronic disease. 

5

Describe the following for DIC:

  • Pathology:
  • Lab findings:
  • Associations: 
  • Treatment: 

 

  • DIC is an acquired syndrome, characterized by systemic intravascular coagulation. *Coagulation always comes first, then followed by bleeding. Always caused by something else. 
  • Lab: elevated PT, PTT, thrombin time, dereased factor activities, elevated D-dimer, somtimes thrombocytopenia and hemolytic anemia. 
  • Associations: microvascular infarction and hemorrahge. Bleeding complications are common. Other organs affected include liver, kidneys and CNS. 
  • Tx: Correct underlying disorder (IE ceftriaxone for meningitis) and supprotive therapy (factor replacement).

6

What are the most important tests for DIC?

D-Dimer is the best test for DIC (although non-specific). D-dimer is incative of clot formation that has been broken down. Always + for DIC, but also elevated in cancer, surgery, pregnancy, etc. 

 

Fibrinogen is another important test (expect low leves). 

7

Hemophilia

  • Primary disorder for Hemophilia A, B, and C (C not tested)?
  • Inheritance pattern and severity among family?
  • Incidence?
  • Treatment? 

Hemophilia

  • A: deficiency in factor VIII, B: deficiency in factor IX, C: deficiency in factor XI. 
  • Inheritance: A and B are x-linked recessive. Severity level is consistent amont family members. 30% of hemophilia are new mutations. Hemophilia C is autosomal and affects M and F equally. 
  • Incidence: A—20/100k males, B—5/100k males. About half of all cases are severe.
  • Treatment: Replace what's missing. Recombinant factor IX or VIII. 

8

Child with PMH of multiple sprained ankles and easy bruising. What kind of bleeding is this?

Kids with hemophilia have bleeding into joints. Kids running around with swollen ankle probably has hemorrhage in the ankle!

9

If PT or PTT is elevated, what's the next step?

1:1 mixing study!

If mixing study corrects the time, then you have a factor deficiency. 

If not, there is an inhibitor in the blood that is preventing the clotting. 

10

VWF

  • Synthesis:
  • Storage:
  • Functions:
  • Levels with age, race, and blood group:
  • Increased by?
  • Decreased by?

VWF

  • Synthesis:endothelial cells, megakaryocytes
  • Storage: endothelial cells, alpha granules in platelets
  • Functions: adhesion of platelets to sites of injury, binds and protects factor VIII in circulation
  • Levels with aging (increases), race (15% higher in blacks), and blood group (30% lower in type O):
  • Increased by? Sympathetic stimulation, surgery, trauma, exercise, and pregnancy.
  • Decreased by? hypothyroidism

11

VWF disease (VWD)

  • Incidence:
  • Pathology
  • Clinical characteristics:
  • Treatment:
  • Additional stuff: 

VWF disease (VWD)

  • Incidence: 0.1-1%, most common inherited bleeding disorder!
  • Pathology: impaired primary hemostasis (mucocutaneous). 
  • Clinical characteristics: Mucosal bleeding (epistaxis and menorrhagia), postoperative bleeding, bleeding from oral cavity, GI bleeding.
  • Treatment: 
    • DDAVP (desmopressin acetate): synthetic analogue of vasopressing, releases VWF from endothelial storage.
    • Transfusion therapy: viral-inactivated VWF-FVIII concentrate. Cryopreipitate (fibrinogen, VIII, VWF, XIII)--in emergency cases when nothing else is available. 
  • Additional stuff: Bleeding disorders are common contributors of iron-deficiency anemia in menstruating females. 

12

Woman with heavy periods, nosebleeds during childhood, and easy bruising, often associated with lumps.

  • Disorder: 
  • Characterize the bleeding:
  • Blood smear: 
  • Other findings:
  • Tx: 

  • Disorder: VWD
  • Characterize the bleeding: Mucocutaenous and deep (nosebleeds, menorrhagia, muscle hematoma--lumps). 
  • Blood smear: microcytic, hypochromic, anisopoikilocytosis (increased RDW)
  • Other findings: low F VIII, because VWF is needed to prolong its activity in circulation. 
  • Tx: desmopressin--released VWF from endothelial cells. 

13