14 tx

Question 1

extravasc hemolysis

Answer 1

splenectomy

Question 2

hereditary spherocytosis (HS)

Answer 2

Splenectomy

Question 3

sickle cell anemia

Answer 3

• Hydroxyurea (DNA synthesis inhibitor) increases HbF and has an anti-inflammatory effect.
• HSC transplant is possible

Question 4

Bthalassemia major

Answer 4

transfusion dependent anemia beginning at 6-9 months of age.

Question 5

a thalassemia: hydrops fetalis= 4 deletions

Answer 5

• Intrauterine fetal transfusion can save infants that used to die in utero.
• Lifelong dependence on transfusions (risk of iron overload).
• HSC transplant is curative.

Question 6

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Answer 6

Ecluzimab (prevents C5 conversion to c5a).

• Decreased hemolysis and thrombosis and required transfusions.
• Risk of meningococcal infxn.

Immunosuppression may benefit some pts with marrow aplasia.

Definitive tx: HSC transplant.

Question 7

Warm Ab Type of immunohemolytic anemia

Answer 7

Remove initiating factors, immunosuppression or splenectomy.

Question 8

periccious anemia

Answer 8

High dose or parenteral admin of B12 or folate, but be careful with folate as it can cause worsening of the mental prbs without also giving B12.

Question 9

anemia of chronic dis

Answer 9

tx the underlying condition to correct the microcytic, hypochromic anemia

Question 10

aplastic anemia

Answer 10

• Bone marrow transplant (5 yr survival > 75%).

- Immunosuppression in older pts or those w/o a suitable donor

Question 11

pure red cell aplasia

Answer 11

• Thymoma resection = 50% of pts have hematologic improvement.
• No thymoma? Tx with immunosuppression.

Question 12

anemia from chronic renal failure

Answer 12

• Recombinant EPO

- +/ iron replacement therapy

Question 13

Chronic Immune Thrombocytopenic Purpura (ITP)

Answer 13

• Glucocorticoids, but most will relapse.
• Splenectomy.
• IVIG or rituximab (antiCD20 Ab) if relapse after splenectomy or if splenectomy contraindicated
• Thrombopoietin (TPO) mimetics may also stimulate platelet production.

Question 14

Acute Immune Thrombocytopenic Purpura (ITP)

Answer 14

• usu self limite

- severe –> glucocorticoids

Question 15

heparin-induced thrombocytopenia

Answer 15

d/c heparin

Question 16

Thrombotic Thrombocytopenic Purpura (TTP),

Answer 16

• Plasma exchange: removes aoutabs ans provides functional ADAMTS13
• Untreated: 100% mortality.

Question 17

atypical Hemolytic Uremic Syndrome (HUS)

Answer 17

• antibodies that inhib activationof compliment factor C5

- Immunosupression can help pts that have autoAbs to the inhibitory complement factors.

Question 18

typical Hemolytic Uremic Syndrome (HUS)

Answer 18

supportive

Question 19

Von Willebrand Disease

Answer 19

• Predominantly supportive Tx.

- May administer cryoprecipitate (replaces vwf) or DDAVP (causes release of vwf from endothelium).

Question 20

hemophilia A

Answer 20

Recombinant factor VIII infusions.

Question 21

hemophilia A

Answer 21

Recombinant factor XI infusions.

Question 22

DIC

Answer 22

the underlying cuase

Question 23

Transfusion-related Acute Lung Injury (TRALI)

Answer 23

unresponsive to diuretics, mostly suportive