Table 2

Tier 1 treatment options

DrugDoseResponse rate*Time to response (wk)Selected toxicities
Low-dose prednisone ≤5 mg orally once per day <10% N/A Weight gain, hyperglycemia, hypertension, osteoporosis, cataracts 
Rituximab 375 mg/m2 IV once per week × 4 (lower doses may be effective) 60% overall, 40% complete, 20%-25% at 5 y 1-8 Infusion reactions, serum sickness, HBV reactivation, PML (rare) 
Romiplostim 1-10 μg/kg SC once per week 80% overall, 40%-50% persistent 1-4 Reticulin fibrosis, rebound thrombocytopenia, thrombosis 
Eltrombopag 25-75 mg orally once per day 80% overall, 40%-50% persistent 1-2 Reticulin fibrosis, rebound thrombocytopenia, thrombosis, hepatotoxicity 
DrugDoseResponse rate*Time to response (wk)Selected toxicities
Low-dose prednisone ≤5 mg orally once per day <10% N/A Weight gain, hyperglycemia, hypertension, osteoporosis, cataracts 
Rituximab 375 mg/m2 IV once per week × 4 (lower doses may be effective) 60% overall, 40% complete, 20%-25% at 5 y 1-8 Infusion reactions, serum sickness, HBV reactivation, PML (rare) 
Romiplostim 1-10 μg/kg SC once per week 80% overall, 40%-50% persistent 1-4 Reticulin fibrosis, rebound thrombocytopenia, thrombosis 
Eltrombopag 25-75 mg orally once per day 80% overall, 40%-50% persistent 1-2 Reticulin fibrosis, rebound thrombocytopenia, thrombosis, hepatotoxicity 

HBV, hepatitis B virus; IV, intravenous; N/A, not applicable; PML, progressive multifocal leukoencephalopathy; SC, subcutaneous.

*

Studies vary in their definition of response.

In patients who are responding to intermediate or high doses of prednisone, we taper to low-dose prednisone. We do not start low-dose prednisone de novo.

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