14 tx Flashcards
extravasc hemolysis
splenectomy
hereditary spherocytosis (HS)
Splenectomy
sickle cell anemia
- Hydroxyurea (DNA synthesis inhibitor) increases HbF and has an anti-inflammatory effect.
- HSC transplant is possible
Bthalassemia major
transfusion dependent anemia beginning at 6-9 months of age.
a thalassemia: hydrops fetalis= 4 deletions
- Intrauterine fetal transfusion can save infants that used to die in utero.
- Lifelong dependence on transfusions (risk of iron overload).
- HSC transplant is curative.
Paroxysmal Nocturnal Hemoglobinuria (PNH)
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.
Warm Ab Type of immunohemolytic anemia
Remove initiating factors, immunosuppression or splenectomy.
periccious anemia
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.
anemia of chronic dis
tx the underlying condition to correct the microcytic, hypochromic anemia
aplastic anemia
- Bone marrow transplant (5 yr survival > 75%).
- Immunosuppression in older pts or those w/o a suitable donor
pure red cell aplasia
- Thymoma resection = 50% of pts have hematologic improvement.
- No thymoma? Tx with immunosuppression.
anemia from chronic renal failure
- Recombinant EPO
- +/ iron replacement therapy
Chronic Immune Thrombocytopenic Purpura (ITP)
- 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.
Acute Immune Thrombocytopenic Purpura (ITP)
- usu self limite
- severe –> glucocorticoids
heparin-induced thrombocytopenia
d/c heparin
