Clinical and biological characteristics of adult biphenotypic acute leukemia in comparison with that of acute myeloid leukemia and acute lymphoblastic leukemia: a case series of a Chinese population

Abstract

BackgroundBiphenotypic acute leukemia is a rare disorder that is difficult to diagnose. It displays features of both myeloid and lymphoid lineage. There is still a lack of studies in biphenotypic acute leukemia in a Chinese population. We present here a comprehensive investigation of the clinical and biological characteristics, and outcome of biphenotypic acute leukemia in our hospital in over a seven year period. Design and MethodsWe retrospectively analyzed 452 adult acute leukemia patients diagnosed according to French-American-British (FAB) classification and biphenotypic acute leukemia diagnosed according to European Group for the Immunological Characterization of Leukemias (EGIL) classification, respectively. Biological characteristics, response to treatment, and outcome were examined in biphenotypic acute leukemia patients and compared with that in acute myeloid leukemia and acute lymphoblastic leukemia patients with complete follow-up profiles diagnosed in the same period. ResultsOf 452 acute leukemia patients, 21 cases (4.6%) were diagnosed as biphenotypic acute leukemia. Among them, 14 (66.7%) were B lymphoid and myeloid, 5 (23.8%) were T lymphoid and myeloid, one (4.8%) was T/B lymphoid and one (4.8%) was trilineage differentiation. When compared with acute myeloid leukemia and acute lymphoblastic leukemia, patients with biphenotypic acute leukemia showed significantly higher incidence of CD34 antigen expression, unfavorable karyotypes, and extramedullary infiltration (p<0.05). In this cohort of patients with biphenotypic acute leukemia, t(9;22) was the most common abnormality in chromosome structure. The median disease-free survival and overall survival in biphenotypic acute leukemia patients was five months and ten months, respectively, significantly shorter than those in acute myeloid leukemia and acute lymphoblastic leukemia patients (p<0.05). ConclusionsThe prognosis of biphenotypic acute leukemia patients is poor when compared with de novo acute myeloid leukemia or acute lymphoblastic leukemia. Biphenotypic acute leukemia patients showed a much higher incidence of CD34 antigen expression, complex abnormal karyotype, extramedullary infiltration, relapse, and resistance to therapy after relapse.Key words: biphenotypic leukemia, immunophenotype, cytogenetics, prognosis. Haematologica 2009;94:919-927.doi:10.3324/haematol.2008 This is an open-access paper.

Mixed-phenotype acute leukemia: historical overview and a new definition

“…However, Killick et al 34 found that 86% of their patients with t(9;22) were dead at follow-up as compared with 40% without this translocation, which suggests that the presence of this translocation correlates with a worse outcome (P ¼ 0.03). Similarly, Xue et al 36 found that Philadelphia-positive biphenotypic leukemia had a worse prognosis when compared with other cases. In their study, Killick et al 34 found that there was no apparent difference in survival between Philadelphia-positive biphenotypic leukemia and Philadelphia-positive ALL.…”

“…Killick et al 34 showed that in their adult group, the survival of patients with biphenotypic leukemia was worse when compared with matched controls with AML or ALL. Similarly, Xue et al 36 found that adult patients with biphenotypic leukemia had a worse overall survival and disease-free survival than those with either AML or ALL. Legrand et al 40 also found that adult patients with biphenotypic acute leukemia had a worse prognosis in terms of achieving complete remission and 4-year overall survival when compared with AML or ALL patients.…”

“…35 This tends to occur more commonly in children and is more frequent in infancy. 36 These cases also tend to present with a dimorphic blast population and the lymphoblasts have a CD19-positive, CD10-negative, B-precursor immunophenotype and are frequently positive for CD15. The prognosis of MPAL patients with MLL rearrangement is poor.…”

Classification of Acute Leukemia

“…EGIL Scoring system for biphenotypic acute leukemia Biphenotypic acute leukemia is defined when scores are >2 for the myeloid lineage and >1 for the lymphoid lineage. In some T-ALL cases, clonality of TCR alphabeta rearrangements can now be assessed cytoflourmetrically (Langerak 2001, Xu XQ et al 2009. The prognosis of biphenotypic acute leukemia patients is poor when compared with de novo acute myeloid leukemia or acute lymphoblastic leukemia.…”

Treatment of acute mixed-cell leukemia with autologous hematopoietic SCT followed by allogeneic hematopoietic stem cell micro-transplantation

“…1 The prognosis of AMAL is poor, with an average OS of only 10 months in patients treated by chemotherapy alone, which is far shorter than that for patients with acute leukemias with single lineage origins. 2 Although allogeneic hematopoietic SCT (allo-HSCT) can significantly prolong patient survival, 3 intensified preconditioning is required to ensure long-term survival, 4 which usually is not possible in elderly patients. However, in contrast to traditional allo-HSCT, related allogeneic hematopoietic stem cell micro-transplantation has been shown to be feasible and effective in elderly patients with AML.…”