Table 3.

HES therapy

DrugUsual dosingSide effects*Comments
Imatinib 100-400 mg orally Cytopenias, hepatitis, diarrhea, edema, necrotizing myocarditis First line for PDGFR-associated myeloid neoplasms, second line for other forms of MHES 
Prednisone Varied, oral, swallowed, or intravenous Weight gain, osteopenia, diabetes, mood disturbance First line for most PDGFR-negative HES; adjunct for PDGFRA positive with cardiac involvement 
Hydroxyurea 1-2 g/d, oral Cytopenias, diarrhea Second line for idiopathic HES and PDGFRA-negative MHES; low dose may potentiate activity of interferon-α 
Interferon-α 1-3 mU subcutaneously daily or 3 times per week; varied (pegylated) Flu-like symptoms, depression, cytopenias, hypothyroidism, neuropathy, liver toxicity Second line for all forms of HES; preferred second line for LHES 
Methotrexate 7.5-20 mg weekly, orally or subcutaneously Cytopenias, liver toxicity, pneumonitis, desquamative skin rash, encephalopathy, secondary malignancy Alternative second-line agent for EGPA, HES with pulmonary involvement 
Cyclosporine 150 mg daily orally Nephrotoxicity, hypertension, neurotoxicity, secondary malignancy Little data to support use in HES, although anecdotal reports of efficacy in LHES 
DrugUsual dosingSide effects*Comments
Imatinib 100-400 mg orally Cytopenias, hepatitis, diarrhea, edema, necrotizing myocarditis First line for PDGFR-associated myeloid neoplasms, second line for other forms of MHES 
Prednisone Varied, oral, swallowed, or intravenous Weight gain, osteopenia, diabetes, mood disturbance First line for most PDGFR-negative HES; adjunct for PDGFRA positive with cardiac involvement 
Hydroxyurea 1-2 g/d, oral Cytopenias, diarrhea Second line for idiopathic HES and PDGFRA-negative MHES; low dose may potentiate activity of interferon-α 
Interferon-α 1-3 mU subcutaneously daily or 3 times per week; varied (pegylated) Flu-like symptoms, depression, cytopenias, hypothyroidism, neuropathy, liver toxicity Second line for all forms of HES; preferred second line for LHES 
Methotrexate 7.5-20 mg weekly, orally or subcutaneously Cytopenias, liver toxicity, pneumonitis, desquamative skin rash, encephalopathy, secondary malignancy Alternative second-line agent for EGPA, HES with pulmonary involvement 
Cyclosporine 150 mg daily orally Nephrotoxicity, hypertension, neurotoxicity, secondary malignancy Little data to support use in HES, although anecdotal reports of efficacy in LHES 
*

Not inclusive.

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