Success of short-term, higher-dose imatinib mesylate to induce clinical response in FIP1L1-PDGFRalpha-negative hypereosinophilic syndrome.
Presence of the oncogenic mutation FIP1L1-PDGFRalpha in hypereosinophilic patients is predictive of hematologic response to imatinib mesylate. However, most patients with hypereosinophilic syndrome (HES) do not have this mutation and have not responded to imatinib doses traditionally successful in patients who test positive for FIP1L1-PDGFRalpha. A patient with FIP1L1-PDGFRalpha-negative HES who had intolerance of interferon alpha-2b and hydroxyurea was treated with escalating doses of imatinib. At 800 mg of imatinib daily, eosinophilia was controlled, allowing prednisone tapering and control of clinical and laboratory-detected abnormalities. HES patients who test negative for FIP1L1-PDGFRalpha may benefit from a trial of higher-dose imatinib.
Butterfield
Joseph H
JH
The Division of Allergic Diseases, Mayo Clinic, Rochester, MN 55905, USA. butterfield.joseph@mayo.edu
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D000970:Antineoplastic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D001549:Benzamides; D004305:Dose-Response Relationship, Drug; D006801:Humans; D006918:Hydroxyurea / Q000008:administration & dosage / Q000009:adverse effects; D017681:Hypereosinophilic Syndrome / Q000188:drug therapy* / Q000235:genetics; D000068877:Imatinib Mesylate; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000008:administration & dosage / Q000009:adverse effects; D008297:Male; D009154:Mutation; D015514:Oncogene Proteins, Fusion; D010879:Piperazines / Q000008:administration & dosage*; D011239:Prednisolone / Q000008:administration & dosage*; D011743:Pyrimidines / Q000008:administration & dosage*; D020796:Receptor, Platelet-Derived Growth Factor alpha; D011994:Recombinant Proteins; D013997:Time Factors; D039221:mRNA Cleavage and Polyadenylation Factors
D000893:Anti-Inflammatory Agents; D000970:Antineoplastic Agents; D001549:Benzamides; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D011743:Pyrimidines; D011994:Recombinant Proteins; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; D011239:Prednisolone; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha; D006918:Hydroxyurea