heme/onc treatments

Question 1

how do you treat vonwillibrand disease

Answer 1

DDAVP (desmopressin

Question 2

Tx for protein C/S deficiency

Answer 2

Heparin and oral anticoag for life

Question 3

Tx for ITP- adults vs kids

Answer 3

Children- supportive care, IVIG for refractory

Adults- prednisone, IVIG (if refractory to steroids)

2nd line for refractory- splenectomy

Question 4

Tx for TTP

Answer 4

steroids

plasmapheresis

Question 5

Tx for sickle cell anemia

Answer 5

hydroxyuria

Question 6

treatment for B12 deficiency anemia

Answer 6

IV cyanocobalmin 1mg IM QD x7d, weekly x4wk then monthly for life

Question 7

Epoetin alfa (exogenous erythropoetin) should only be initiated in anemia of chronic disease if Hgb is below what level

at what level should they be stopped

Answer 7

start: Hgb <10

stopped >11 (b/c of incr risk of MI/CVA)

Question 8

tx for aplastic anemia (<50, >50/comorbidities)

Answer 8

• <50: bone marrow transplant
• >50/comorbidities: immunosuppressive agents, anti-thymocyte globulin (ATG) + cyclosporine and prednisone
• Hematopoietic growth factor (G-CSF (filgastrim)) reduces rate of infectiosn but does NOT alter course of the dz

Question 9

Tx for hereditary spherocytosis

Answer 9

folic acid

supportive transfusion

splenectomy for moderate-severe

Question 10

tx for autoimmune hemolytic anemia

Answer 10

1st l;ine= steroids

severe= splenectomy and even blood transfusions

Question 11

tx for polycythemia vera

Answer 11

• repeated phlebotomy to lower hct to <42%
• > 60y/o or h/o thrombosis-
  
  • myelosuppressive agent (hydroxyurea +/- ASA)

Question 12

tx for thrombocytosis

Answer 12

antiplatelet agents (ex anagrelide and low dose ASA)

Hydroxyurea sometimes useful for severe thrombocytosis

Question 13

how do you treat hemochromatosis

Answer 13

phlebotomy- weekly at first

once excess iron stores removed, patients managed w/ lifelong phlebotomies q2-4mo

Deferoxamine (chelation of iron- if unable to undergo phlebotomy)

Question 14

tx for sickle cell anemia

Answer 14

chemo, narcotics, blood transfusions

rarely- bone marrow transplant

Question 15

tx for thalassemia

Answer 15

• mild- no tx
• moderate (B thal)-folate, avoid oxidative stress
• Severe disease/major
  
  • blood transfusions weekly if severe anemia
  • Deferoxamine (iron chelator)
  • bone marrow transplant- definitive tx in major