Progression of primary myelofibrosis to polycythemia vera

Yan Guo, XU Wen-wei, Dong Lin, Ning Huang, Kehong Bi
In: Medicine · 2017 · vol. 96(28) , pp. e7464 · doi:10.1097/md.0000000000007464 · PMID:28700486 · W2734329509
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This case report details a patient with primary myelofibrosis who progressed to polycythemia vera, identified by new chromosomal abnormalities, but ultimately showed improved quality of life and prognosis.

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Abstract

RATIONALE: This case report describes the progression of primary myelofibrosis (PMF) to polycythemia vera (PV), and discuss its potential mechanisms. PATIENT CONCERNS: The patient was admitted because of abdominal discomfort and enlarged spleen for 19 months. DIAGNOSIS: A case of PMF progressed to PV was retrospectively analyzed. There were 19 months between the diagnosis of PMF and PV. The JAK2 V617F mutation was positive before and after the diagnosis of PV; however, new chromosomal abnormalities were detected during the progression. INTERVENTIONS: For treatment of PMF, the danazol, calcitriol, and thalidomide were given. Then, the use of thalidomide and calcitriol was stopped, and hydroxyurea was started. For treatment of PV, interferon treatment was given, whereas hydroxyurea was continued. OUTCOMES: After 30 months of the progression (at the recent follow-up), this patient had no obvious symptoms or thrombosis. LESSONS: PMF rarely progresses to PV, however, the progression will significantly improve the quality of life and prognosis.

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