研究概要
该患者接受了基于移植后环磷酰胺的单倍体移植,截至诊断后 2 年的末次随访检查时仍保持无复发。
中文摘要
髓系/自然杀伤(NK)细胞前体急性白血病(MNKPL)是一种罕见白血病亚型,可能起源于NK细胞前体。该病预后不良,治疗资料有限。我们报告首例46岁MNKPL女性患者病例:该患者对两线AML类型的强化化疗无应答,但接受维奈克拉联合阿扎胞苷(VEN/AZA)后治疗成功。患者依据幼稚淋巴母细胞样形态、无髓过氧化物酶反应、CD7/CD33/CD34/CD56/HLA-DR阳性表型及髓外病灶确诊MNKPL。疾病对柔红霉素联合阿糖胞苷(DNR/Ara-C)诱导治疗及米托蒽醌、依托泊苷联合阿糖胞苷(MEC)再诱导治疗均耐药。两线诱导化疗后出现大量心包及胸腔积液,怀疑为髓外病灶;患者发生心脏压塞,需要心包穿刺引流。因此,采用VEN/AZA作为三线治疗。经过两个周期VEN/AZA,心包和胸腔积液消失,患者达到完全缓解。随后患者接受基于移植后环磷酰胺的单倍体移植;确诊两年后的末次随访时仍未复发。
展开英文摘要原文
Myeloid/natural killer (NK) cell precursor acute leukemia (MNKPL) is a rare leukemia subtype that possibly originates from precursor NK cells. The disease has a poor prognosis, and information on its treatment is lacking. We herein report the first case of a 46-year-old woman with MNKPL who was refractory to two lines of acute myeloid leukemia (AML)-type intensive chemotherapy but was successfully treated with venetoclax and azacytidine (VEN/AZA). She was diagnosed with MNKPL based on the conformations of immature lymphoblastoid morphology without myeloperoxidase reactivity that showed a CD7/CD33/CD34/CD56/HLA-DR positive phenotype and extramedullary regions. The disease was refractory to induction therapy with daunorubicin and cytarabine (DNR/Ara-C) and to reinduction therapy with mitoxantrone, etoposide, and cytarabine (MEC). After two lines of induction chemotherapy, massive pericardial and pleural effusion was found, and was suspected to be extramedullary lesions. The patient developed cardiac tamponade and required pericardiocentesis. Thus, VEN/AZA was administered as third-line therapy. After two cycles of VEN/AZA, the pericardial and pleural effusion disappeared, and complete remission was achieved. The patient received post-transplant cyclophosphamide-based haploidentical transplantation and has stayed relapse-free as of her last follow-up examination 2 years after diagnosis.
论文信息
- 作者
- Shiomi I、Nakako S、Nakane T、Ogawa Y、Araki T、Fujitani Y、Yamamura R、Hino M
- 第一作者单位
- Department of Hematology, Osaka Saiseikai Nakatsu Hospital, 2-10-39, Shibata, Kita-Ku, Osaka, 530-0012, Japan.Japan
- 通讯作者单位
- Department of Hematology, Osaka Saiseikai Nakatsu Hospital, 2-10-39, Shibata, Kita-Ku, Osaka, 530-0012, Japan. t_nakane@nakatsu.saiseikai.or.jp.Japan
- 文献类型
- 病例报告
- 期刊
- International journal of hematology2024 Jan