Yang Liqing, Huang Meijuan, Chen Yuanzhong, Wu Yong
Department of Hematology, Fujian Medical University Union Hospital, Fujian Medical University, Fuzhou, Fujian, China.
Fujian Provincial Key Laboratory on Hematology, Fujian Institute of Hematology, Fujian Medical University Union Hospital, Fuzhou, Fujian, China.
Front Oncol. 2025 Jul 25;15:1623528. doi: 10.3389/fonc.2025.1623528. eCollection 2025.
Mixed-phenotype acute leukemia (MPAL) is a rare type of acute leukemia with an incidence of less than 5% and Philadelphia chromosome-positive (Ph+) represents a distinct subtype.
An 18-year-old female complained of recurrent fever with fatigue and chills for one month, and a week of growing lymphadenectasis. Bone marrow examination revealed two distinct populations of blast cells and the presence of fusion gene, leading to a diagnosis of Ph+ MPAL. The patient received induction chemotherapy of DVAP regimen combined with tyrosine kinase inhibitors (TKIs), and underwent allogeneic hematopoietic stem cell transplantation after achieving complete remission. To date, the patient has maintained sustained hematological and molecular complete remission.
A literature review of 59 cases revealed that Ph+ MPAL is more common in adult, male patients and primarily manifests as B/myeloid subtype. Higher leukocyte counts and chromosome -7 abnormalities have been identified as poor prognostic markers. Acute lymphoblastic leukemia-type therapy is considered more effective for patients with MPAL, and in the TKI era Ph+ has become a subtype with a better prognosis.
混合表型急性白血病(MPAL)是一种罕见的急性白血病,发病率低于5%,费城染色体阳性(Ph+)代表一种独特的亚型。
一名18岁女性主诉反复发热伴乏力、寒战1个月,以及进行性淋巴结肿大1周。骨髓检查发现两种不同的原始细胞群,并存在融合基因,从而诊断为Ph+ MPAL。患者接受了DVAP方案联合酪氨酸激酶抑制剂(TKIs)的诱导化疗,并在完全缓解后接受了异基因造血干细胞移植。迄今为止,患者一直保持持续的血液学和分子学完全缓解。
对59例病例的文献综述显示,Ph+ MPAL在成年男性患者中更为常见,主要表现为B/髓系亚型。较高的白细胞计数和染色体-7异常已被确定为不良预后标志物。对于MPAL患者,急性淋巴细胞白血病类型的治疗被认为更有效,并且在TKI时代,Ph+已成为预后较好的亚型。