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 [18–21] 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.