Affiliations: Department of Haematology, Qingdao Central Hospital, Qingdao 266042, Shandong, China
Correspondence:
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Corresponding author: Ling Wang, Department of Haematology, Qingdao Central Hospital, No. 127 of Siu South Road, Shibei district, Qingdao 266042, Shandong, China. Tel.: +86 018660229182; Fax: +86 0532 84863506; E-mail: lingwang54321@21cn.com.
Abstract: OBJECTIVE: This study aims to analyze Chinese patients who developed acute leukemia after being diagnosed and treated for Philadelphia chromosome (Ph)-negative chronic myeloproliferative neoplasms (MPNs), and compare the findings of this series with similar studies from literature. METHODS: Nine patients who progressed to leukemia after being diagnosed with MPN were included into the present study. Clinical data including age, treatment modalities and duration of use in the myeloproliferative phase, latency to leukemic transformation (LT), characteristics of leukemia, chemotherapy administration, and survival after LT were examined. Furthermore, factors associated with leukemia transformation were analyzed. RESULTS: Over a 13-year period, nine patients had LT in 192 Ph-negative MPNs. Among these patients,
two patients had polycythemia vera (PV), three patients had essential thrombocythemia (ET),
and four patients had myelofibrosis (MF). The median age at MPN diagnosis was 51 years old
(range: 42–69 years old), and the median age upon reaching LT was 57 years old (range: 46–72
years old). Furthermore, the median latency to LT was 72.8 months (range: 7–144 months). Five
patients had cytogenetic abnormalities (62.5%), with abnormalities in
chromosomes -5, +8 and -7 being common. Eight patients underwent the JAK2 V617F gene test when diagnosed with MPN. The prognosis of patients with LT was poor, and the average
survival time was 6.7 months. This was not correlated with the treatment. CONCLUSION: LT in Ph-negative MPNs is rare, and has poor prognosis, which has been consistently reported in a number of studies, However, this needs to be further confirmed through larger studies.
