Current clinical strategies and emergent treatment landscapes in leukemic transformation of Philadelphia-negative myeloproliferative neoplasms

Abruzzese, Elisabetta; Niscola, Pasquale

doi:10.1080/17474086.2020.1850251

Cited by 2 publications

(3 citation statements)

References 84 publications

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“…MPN-BP is usually seen as a terminal event with a reported median survival of 3.6 months. 4 The MPN-BP prognosis is related to karyotype, platelet count < 100 × 10 9 /L, transfusion dependence and age. 5,6 Clinical factors associated to MPN-BP in ET are low hemoglobin level and the presence of platelets > 1 × 10 12 /L, and it is independent of JAK2 V617F status.…”

Section: Discussionmentioning

confidence: 99%

“…The treatment in this patient was a dilemma after the third cycle of consolidation (allo-SCT or surveillance). Generally, MPD-BP is seen as a terminal event that needs treatment with allo-SCT as the only way to cure the disease with a great risk of morbidity and mortality 4 . We opted by monitoring NPM1 MRD to detect preclinical relapse as ELN guidelines recommended.…”

Section: Discussionmentioning

confidence: 99%

“…1 The AML transformation usually is a multistep process associated to secondary mutations including mutations of TP53 2 and is associated to bad prognosis. 3,4 We describe a case of ET transformed to an AML with NPM1+/ FLT3-ITD-.…”

Section: Introductionmentioning

confidence: 99%

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NPM1+ /FLT3- Acute Myeloid Leukemia after JAK2-V617F+ Essential Thrombocythemia. Management and Prognosis

Moriano¹,

Barragán²,

Ferre³

et al. 2021

Clin Med�Insights�Blood�Disord

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NPM1+ AML after Essential Thrombocythemia (ET) or Myeloproliferative neoplasms is extremely rare. Only 2 cases have been previously reported after Primary Myelofibrosis. Given the extremely poor prognosis of the Blastic phase of Myeloproliferative neoplasms (MPN-BP), the only curative treatment of these patients is allogeneic stem cell transplantation (allo-SCT) after achieving Complete Response (CR). De novo NPM1+/FLT3- AML is considered a good prognosis entity in which allo-SCT is not contemplated as the first option. A 41 year old diagnosed of ET JAK2 V617F+ 4 years before the diagnosis of AML with normal karyotype and NPM1+/FLT3- was treated with conventional AML induction with Cytarabine and Idarubicine and 3 cycles of high dose Cytarabine. At the diagnosis of AML other 2 mutations were noted: EZH2 and IKZ1. After treatment of AML, NPM1+ clone disappeared, and JAK2 V617F clone reappeared. We opted to treat NPM1+ AML as a de novo AML and we decided to follow up during 2 years without allo-SCT. The patient remains in complete response with NPM1 minimal residual disease negative during the follow up. This case exemplifies the nature of NPM1+ AML secondary to MPD as an extremely sensitive disease to induction therapy plus high dose cytarabine and makes that these type of patients perhaps could be managed without the use of allo-SCT.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%