Acute myeloid leukemia with 17p abnormality in untreated essential thrombocythemia

Hernandez, JA; Florensa, Lourdes; Solé, Francisco; Ma, Baojin; Espinet, Blanca

doi:10.1038/sj.leu.2402176

Cited by 4 publications

(2 citation statements)

References 5 publications

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“…29 The overall findings from the current study support our earlier contention that LT is a natural sequelae of ET whose occurrence is dependent more on intrinsic disease biology rather than on the type and number of cytoreductive agents used for treatment. 30 In this regard, it is important to note that current indications for cytoreductive therapy in ET are biased towards those patients with an increased underlying risk for LT (i.e., presence of extreme thrombocytosis).…”

Section: Prognosis In Essential Thrombocythemiasupporting

confidence: 87%

Risk stratification for survival and leukemic transformation in essential thrombocythemia: a single institutional study of 605 patients

et al. 2006

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Unlike the case with thrombosis, prognostic models for survival and leukemic transformation (LT) in essential thrombocythemia (ET) are not available. Among 605 patients with ET seen at our institution and followed for a median of 84 months, 155 died and LT was documented in 20 patients (3.3%). In a multivariable analysis, hemoglobin level below normal (females o120 g/l; males o135 g/l) was identified as an independent risk factor for both inferior survival and LT. Additional risk factors for survival included age X60 years, leukocyte count X15 Â 10 9 /l, smoking, diabetes mellitus and thrombosis. For LT, platelet count X1000 Â 10 9 /l but not cytoreductive therapy was flagged as an additional independent risk factor. In fact, four of the 20 patients (20%) with LT were untreated previously. We used the above information to construct prognostic models that effectively discriminated among low-, intermediate-and high-risk groups with respective median survivals of 278, 200 and 111 months (Po0.0001), and LT rates of 0.4, 4.8 and 6.5% (P ¼ 0.0009) respectively. Presence of JAK2V617F did not impact either survival or LT and mutational frequency was similar among the different risk groups.

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Section: Prognosis In Essential Thrombocythemiasupporting

confidence: 87%

Risk stratification for survival and leukemic transformation in essential thrombocythemia: a single institutional study of 605 patients

et al. 2006

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“…In fact, monosomy or deletion of chromosome 5 or 7 are commonly observed in patients treated with alkylating agents [27], whereas 17p has been observed following hydroxyurea [21]. These abnormalities, however, have been found at the time of BP also in untreated patients [28,29]. Most patients showed more than a single abnormality or a complex karyotype.…”

Section: Discussionmentioning

confidence: 99%

Blast phase of essential thrombocythemia: A single center study

et al. 2009

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Blast phase (BP) may occur as a late event in essential thrombocythemia (ET). This study includes 19 patients with post-ET BP diagnosed and followed in a single institution. At BP, 63% of patients had leukocytosis (white blood cell count >10 3 10 9 /L), 74% had anemia (hemoglobin value <10 g/dL), 74% had thrombocytopenia (platelet count <100 3 10 9 /L), and 84% were over 65 years of age. Cytogenetic analysis was available in 10 patients: six had karyotype aberrations. According to cytogenetic-based risk stratification of de novo acute leukemia (AL), all patients had an unfavorable profile. JAK2 (V617F) mutational status was evaluated in five patients. In two of them, the JAK2 mutation was undetectable in blast cells (one with JAK2-positive ET), whereas in three both granulocytes and blast cells displayed the mutation. Treatment of BP was patient-based according to the performance status and co-morbidities and consisted of palliation in 14 patients, and of induction of remission in five. Median survival was 2.3 months (range 0.2-22.3), irrespective of the treatment received. In conclusion, this study indicates that AL evolved from ET has unfavorable clinical and biological features. JAK2 (V617F)-positive ET may evolve in few instances into JAK2-negative leukemia. The outcome of patients is poor whatever the treatment used. Am. J. Hematol. 84:641-644, 2009. V

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