Clinical Analysis of 208 Patiets with BCR/ABL Negative Myeloproliferative Neoplasms Objective: To analyze the incidence,hemogram,genetics,clinical manifestations,therapeutic efficacy and outcome of patients w ith myeloproliferative neoplasms( M PN) so as to provide much more therapeutic basis for clinically studying the pathogenesis,diagnosis,and treatment as w ell as evaluating the prognosis of M PN patients.Methods: The clinical data and related laboratory test results in 208 cases of BCR/ABL fusion gene regative M PN w ere collected and analyzed retrospectively. Results: The M PN could occur at any age,but the highest incidence w as observed in patients aged 40-79. Among 208 patients w ith M PN,the patients w ith essential thrombocythemia( ET)accounted for 48. 56%( 101/208),the patients w ith polycythemia vera( PV) accounted for 25. 96%( 54/208),and the patients w ith primary myelofibrosis( PM F) accounted for 25. 48( 53/208). The clinical manifestation of M PN varied,the first manifestations w as no-specific,onset of disease presented slow. The JAK2V617 F gene mutation existed in 130 out of 208 patients w ith M PN,total mutation rate w as 62. 5%; JAK2V617 F mutation rate in PV patients w as 81. 5%( 44/54),while that in ET and PMF patients was 58. 4%( 59/101) and 50. 9%( 27/53) respectively,the detected rate of this mutation in PV patients w as significantly higher than that in ET and PM F patients( P 0. 05). In PV group,the WBC count of JAK2V617 F positive patients w as significantly enhanced( P 0. 05); in ET and PM F groups,the JAK2V617 F positive patients had a higher WBC count and hemoglobin level( P 0. 05). The most common vascular event in patients w ith M PN w as ischemic cerebrovascular disease. The JAK2V617 F mutation related w ith risk of thrombosis( OR = 2. 222,95% CI = 1. 101 to 4. 486). The difference in the incidence of vascular event betw een ET and PV patients w as no statistically significant( P > 0. 05),but the incidence of vascular event in ET and PV patients w as higher than that in PM F patients( P < 0. 05). The disease conversion much more easily happened in JAK2V617 F positive patients. After treatment,the M PN could be controlled,yet the maintained treatment is needed. Conclusion: The M PN can occur almost at any age,but more commonly occures in middle-aged and elderly persons. The onset of M PN varies,the clinical manifestation w as similar,a high detected rate of JAK2V617 F mutation is observed in M PN patients and relates closely w ith onset of M PN; moreover,JAK2V617 F mutation rate relates w ith type of M PN. The M PN patients w ith JAK2V617 F mutation have higher WBC count and higher incidence of thrombosis. After treatment,the M PN can be better controlled,and need maintenance treatment. So as to avoid the reccurence of disease,control the complications and obtain the long-term survival.
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