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Table 2 PDGFRA rearrangement in unusual adult myeloid neoplasms

From: Discovery of imatinib-responsive FIP1L1-PDGFRA mutation during refractory acute myeloid leukemia transformation of chronic myelomonocytic leukemia

Author/journal

Disease

Treatment

Outcome

Tang et al., Acta Haematol 2012;128:83–87

Myeloproliferative neoplasm with eosinophilia

Imatinib – started at 400 mg daily and maintained at 100 mg daily

Complete hematologic and molecular remission at 12 months

Papanikolaou et al., Ann Hematol. 2012 May;91(5):785-7

Chronic eosinophilic leukemia with lytic bone lesions

Imatinib 200 mg daily; on progression, nilotinib 400 mg BID

Progressed to erythroblastoid blast crisis after 2 years on TKI

Sorour et al., Br J Haematol. 2009 Jul;146(2):225-7

Acute myeloid leukemia with eosinophilia

Imatinib with FLAG-Ida followed by matched unrelated allograft

Relapsed with Imatinib resistance; started on Dasatinib but died 15 months later

Lierman et al., Leukemia. 2009 May;23(5):845-51

Chronic eosinophilic leukemia blast crisis with Imatinib resistance

Sorafenib 400 mg BID

Hematologic response without molecular response for 3 months

Zota et al., J Clin Oncol. 2008 Apr 20;26(12):2040-1

Chronic myelomonocytic leukemia

Imatinib 400 mg BID

Resolution of eosinophilia without other hematologic response; progressed to extramedullary disease

Florian et al., Leuk Res. 2006 Sep;30(9):1201-5

Systemic mastocytosis with chronic eosinophilic leukemia

Incomplete response to hydroxyurea, corticosteroids and interferon-alpha; started on imatinib 100 mg in 2002

Long-term response to low-dose Imatinib (50-100 mg) after inadequate responses to previous therapies

Von Bubnoff et al., Leukemia. 2005 Feb;19(2):286-7

Chronic myeloproliferative disorder with eosinophilia

Imatinib 100-400 mg daily

Hematologic and symptomatic response for 6 months; progression to myeloid blasts crisis and malignant pleural effusion

Saflet et al., Genes Chromosomes Cancer 2004 May;40(1):44-50

Atypical chronic myeloid leukemia with t(4;22) leading to formation of BCR-PDGFRA fusion gene

Imatinib 100 mg daily

Complete hematologic response at 7-month follow up