Lai Binbin, Mu Qitian, Zhu Huiling, Wang Yi, Zhang Yi, Xu Kaihong, Sheng Lixia, Ouyang Guifang
Department of Hematology Laboratory of Stem Cell Transplantation, Ningbo First Hospital, Ningbo, PR China.
Medicine (Baltimore). 2018 Apr;97(14):e0294. doi: 10.1097/MD.0000000000010294.
Philadelphia chromosome-positive mixed phenotype acute leukemia (Ph+ MPAL) is a rare type of leukemia with poor prognosis. Tyrosine kinase inhibitors (TKIs) in combination with chemotherapy have significantly improved its remission rate. However, relapse remains the major obstacle to achieve long survival. Lenalidomide is a second-generation oral immunomodulatory drug that has been broadly applied in the treatment of various hematological malignancies.
A 54-year-old Chinese male patient who complained of chest pain and fatigue for 20 days. Bone marrow aspirate examination revealed hypercellularity with 70% blast cells. Flow cytometry analysis revealed that the blast cells exhibit both myeloid and lymphoid lineage antigens. Chromosomal analysis reveals t(9;22)(q34;q11) translocation. Minor BCR-ABL fusion gene was positive.
Philadelphia chromosome-positive mixed phenotype acute leukemia.
After relapsed from routine chemotherapy plus imatinib, the therapy was switched to oral therapy with nilotinib and lenalidomide due to his feeble condition.
He successfully achieved long survival after oral therapy with nilotinib and lenalidomide.
Combination of TKIs with lenalidomide may be an effective maintenance treatment regimen for Ph+ MPAL patients with minimal side effect.
费城染色体阳性混合表型急性白血病（Ph+ MPAL）是一种预后较差的罕见白血病类型。酪氨酸激酶抑制剂（TKIs）联合化疗显著提高了其缓解率。然而，复发仍然是实现长期生存的主要障碍。来那度胺是一种第二代口服免疫调节药物，已广泛应用于各种血液系统恶性肿瘤的治疗。
一名54岁中国男性患者，主诉胸痛和疲劳20天。骨髓穿刺检查显示细胞增多，原始细胞占70%。流式细胞术分析显示原始细胞同时表达髓系和淋巴系抗原。染色体分析显示t(9;22)(q34;q11)易位。微小BCR-ABL融合基因呈阳性。
费城染色体阳性混合表型急性白血病。
在常规化疗加伊马替尼治疗后复发，由于患者身体虚弱，治疗改为尼洛替尼和来那度胺口服治疗。
口服尼洛替尼和来那度胺治疗后，他成功实现了长期生存。
TKIs与来那度胺联合使用可能是Ph+ MPAL患者有效的维持治疗方案，且副作用最小。
