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. 2019 Jan 3;20:10–14. doi: 10.12659/AJCR.911854

Table 1.

Previously reported cases of essential thrombocythemia (ET) with transformation to chronic myelogenous leukemia (CML) with the emergence of the Philadelphia chromosome.

No. Age/Sex CMPD JAK2 V617F Chromosomal translocation Initial treatment for the CMPD Transformation to CML (duration in years) Chromosomal translocation CML treatment Response for CML treatment Outcome Ref.
1 58/F ET ND Negative HU 18 Positive Interferon alpha, UR-PBSCT CCyR Improve [20]
2 65/F ET ND Negative HU 6 Positive Imatinib mesylate PCyR Improve [17]
3 73/M ET ND Negative HU 12 Positive Imatinib mesylate PCyR Progress [18]
4 38/F ET ND Negative Monosomy 6,22 Aspirin 13 Positive + monosom7 Imatinib mesylate + RIST CCyR Improve [19]
5 82/M ET ND Negative HU 9 Positive double Ph1 Imatinib mesylate Nilotinib CCyR Progress [21]
6 49/F ET ND Negative Aspirin 7 Positive HU + Nilotinib, after three months, Nilotinib replaced with IM CCyR Improve Current case

F – Female; M – Male; ET – essential thrombocythemia; HU – hydroxyurea; ND – not detected; IM – imatinib mesylate; CCyR – complete cytogenetic remission; RIST – reduced intensity allogeneic stem cell transplant; UR-PBSCT – unrelated donor peripheral blood stem cell transplantation; CML – chronic myelogenous leukemia. The first reported case [17] was treated initially with HU. Anagrelide was then used to treat the increasing platelet counts, while the CML was managed with imatinib. Almost all reported cases [1821] have been treated with HU and imatinib. The time between initial diagnosis of ET and transformation to CML was six, 12, 13, nine, and 18 years, respectively. Our case had a history that covered a period of seven years.