Hematologic Disorders

Sickle Cell

100

Where might the pain during a crisis be experienced for 

1) Adults

2) Pediatrics

1) Chest & Abdomen

2) Hands and Feet

200

List triggers for sickle cell crisis

-High altitude

-Cold exposure

-Heat

-Dehydration

-Stress

-Acidosis

300

Splenic Sequestration may cause what two cardiovascular signs

Hypotension and tachycardia

400

List 3-4 major complications that can be present during a sickle cell crisis

Pain, chronic anemia, splenic sequestration, infections, acute chest syndrome (dyspnea), stroke (hemiparesis, seizure), priapism

500

What medication is involved in the treatment and what does it do? (Hint: starts with an H)

-Hydroxyurea > decreases sickling and produces Hgb

Platelet Disorders

100

Chronic hypoxia and bone marrow disorders are both causes of what platelet disorder/lab value change? (thrombocytopenia or thrombocytosis)

Thrombocytosis

200

What are the names/medical terms for both elevated and decreased platelet counts

Elevated - thrombocytosis

Decreased/low - thrombocytopenia

300

At what platelet level (roughly) will you switch to an admission with platelet transfusions 

< 30,000

400

What is the term for redness or pain of the digits or hands in thrombocytosis

Erythromelalgia

500

What is the progression of symptoms with decreasing platelet counts (extra 100pts if provide rough ranges)?

1) > 50,000 = asymptomatic

2) 30,000-50,000 = bruising with minor traumas

3) 10,000-30,000 = spontaneous bruising + petechiae

4) <10,000 = internal bleeding

Hemophilia

100

What type of injection might you never want to give to a patient with hemophilia

IM injection

200

Hemophilia A is known as the Classic Hemophilia, but what factor are these patients deficient in?

Factor VIII (eight)

300

Rosenthal's Syndrome is a deficiency of what factor?

Hemophilia C and deficient in Factor XI (eleven)

400

What is the other name/disease Hemophilia B is known for and what factor are they deficient in?

Christmas disease and deficient in Factor IX (nine)

500

What is the name of the medication that stimulates the release (but not the formation) of Factor VIII (eight)?

DDAVP (Desmopressin Acetate)

DIC

100

_____ is a plasma protein involved in the formation of fibrin

Fibrinogen (is a clotting factor and is decreased in DIC)

200

What lab values decrease during DIC? (Hint: there are 4)

1) Hgb

2) Hct

3) Fibrinogen (clotting factors are getting consumed)

4) Platelets

300

What lab values increase during DIC (Hint: there are 3)

1) PT

2) PTT

3) D-Dimer (only measurable FDP)

400

Explain the role of fibrinolysis and FDPs (Fibrin Degradation Products) in DIC

Blood clots form and eventually release their own clot-busters known as FDPs and FDPs have further anticoagulant properties. (Several hundred micro clots can form in 2-3hrs producing multiple FDPs causing further bleeding)

500

List what blood products you might give in DIC and why (4)

1) PRBCs for bleeding 

2) Platelets for thrombocytopenia (fuel for clotting)

3) Cryoprecipitate for low fibrinogen levels (fuel for clotting)

4) FFP for increased PT & PTT

Lab values

100

Elevated RBCs (100 pts)

Polycythemia

200

Decreased/Low WBCs (100 pts)

Leukopenia

300

Elevated Platelets (100 pts)

Thrombocytosis 

400

Decreased/Low RBCs (100 pts)

Anemia

500

Which change will you see in platelets following a major viral disease (usually a few weeks after) and why (200 pts)

Viral dx will cause platelet destruction from immune response after illness resulting in thrombocytopenia