B﹣cell acute lymphoblastic leukemia with E2A﹣HLF fusion gene: report of two cases and review of literature / 白血病·淋巴瘤

ABSTRACT

Objective To explore the role of E2A﹣HLF fusion gene in the prognosis evaluation of B﹣cell acute lymphoblastic leukemia (B﹣ALL), and to improve the accuracy of stratified treatment. Methods The clinical characteristics, treatment effect and survival time of two B﹣ALL patients with E2A﹣HLF fusion gene who were admitted to the Second Hospital of Shanxi Medical University were retrospectively analyzed, and the related literature was reviewed. Results The first patient was an 8 years old girl, developed with fever, abdominal pain, and slightly increased white blood cells (WBC), and also accompanied by hypercalcemia. Another patient was a 27 years old man, developed with jaw pain and anemia, WBC was normal. Precursor B﹣ALL (pre﹣B﹣ALL) was identified by flow cytometry (FCM) in the two cases. E2A﹣HLF fusion gene was screened out at first diagnosis for the girl, but found after relapse for the man. Both patients early received intensive treatment with high﹣dose methotrexate after the first complete remission, but relapsed after 3 and 6 months respectively. The girl did not receive allogeneic hematopoietic stem cell transplantation (allo﹣HSCT) after relapse and died of severe infection. The man received allo﹣HSCT complete remission, and maintained complete remission within 5 months after HSCT, E2A﹣HLF fusion gene was also negative, but eventually died of multiple transplantation﹣related complications. Conclusions E2A﹣HLF fusion gene occurs mostly in the pre﹣B﹣ALL patients with hypercalcemia, and it shows extremely poor prognosis with a high multidrug resistance rate and high early recurrence rate. The allo﹣HSCT can increase the leukemia﹣free survival rate, and the relapse and overall survival may be improved when these patients received allo﹣HSCT in the first complete remission.