| Corticosteroids (Prednisone and Dexamethasone) |
| Mechanism of Action: (Multi-factorial) Down-regulates inflammatory cytokine production at transcriptional and translational level. Decreases leukocyte trafficking, including neutrophil migration. Decreases dendritic cells. Depletes T cells and B cells (T≫B). Also, effects T-lineage commitment.71
|
| Lymphocytes targeted: B, T, NK cells, monocytes, macrophages, neutrophils, and eosinophils |
| Pros: Most active drug for majority of patients with autoimmune cytopenias. Fast-acting. |
| Ability to titrate dose to response. Lots of data using it in combination with other drugs |
| Cons: Short term toxicities: Mild (hypertension, hyperglycemia, irritability, weight gain) |
| Long term toxicities: Potentially severe (avascular necrosis, cataracts, growth delay) |
| Infectious risk: Single agent - low. Combination therapy - Higher risk than most other oral agents. PCP prophylaxis should be strongly considered when steroids combined with other immune suppressants. Antifungal prophylaxis may also be needed. |
|
| IVIgG |
Mechanism of Action: (Multi-factorial). Poorly defined but proposed hypotheses include:72
competition with pathogenic IgGs for activating FcyRs (neonatal Fc receptor) saturation of FcRn by very high levels of IgG leads to breakdown of pathogenic IgGs exogenous IgG binds to targets cells which triggers effector cells to down-regulate immune response
|
| Pros: Active against ITP in majority of patients. Well-tolerated. Quick acting (<48 hours) |
| Cons: Short duration of activity. Patients with chronic disease may need frequent retreatment. Limited activity against AIHA or AIN. Aseptic meningitis & severe allergic reactions. |
| Infectious Risk: None with current products. Protective against certain infections. |
|
|
Anti-D immunoglobulin (anti-D)73
|
| Mechanism of Action: Only works in patients who are Rh+ by saturating FcyRs in spleen |
| Pros: Active against ITP in a majority of patients. Well-tolerated. Quick acting (<48 hours). |
| Cons: May cause hemolytic anemia. Only recommended for children with ITP who are Rh+, have a negative DAT, no anemia, and have not undergone splenectomy.6 Can also cause headache, fever, and chills. Rarely, can cause life-threatening DIC. NOT for children with AIHA or AIN. |
| Infectious Risk: None reported. |
