Department of Hematology and Oncology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie, 514-8507, Japan.
Department of Hematology, Yokkaichi Municipal Hospital, Yokkaichi, Japan.
Int J Hematol. 2021 Jul;114(1):129-135. doi: 10.1007/s12185-021-03107-9. Epub 2021 Mar 1.
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare subtype of intestinal T-cell lymphoma that occurs mostly in Asia. CHOP-like therapy is usually selected, but the prognosis is very poor. This report concerns a 43-year-old woman with newly diagnosed stage IVA MEITL. The patient obtained a partial response after 4 cycles of GDP (gemcitabine, dexamethasone, cisplatin) and achieved a complete response (CR) after cord blood transplantation (CBT) conditioned with total body irradiation, cyclophosphamide, and cytarabine. Seven months after transplantation, the patient experienced cognitive impairment. Magnetic resonance imaging of the brain showed a high-intensity lesion in the right cerebral peduncle and internal capsule. A cerebrospinal fluid examination confirmed central nervous system (CNS) relapse of MEITL. After 3 cycles of MPV (methotrexate, procarbazine, vincristine) followed by whole-brain radiotherapy, her cognitive impairment improved. Due to disease progression, she died 6 months after CNS relapse. Given the CNS relapse after achieving a CR with GDP and CBT in this patient, CNS prophylaxis during first-line therapy may be beneficial in the treatment of MEITL.
单一表型上皮样肠道 T 细胞淋巴瘤(MEITL)是一种罕见的肠道 T 细胞淋巴瘤亚型,主要发生在亚洲。通常选择 CHOP 样治疗,但预后非常差。本报告涉及一名 43 岁的新诊断为 IVA 期 MEITL 的女性患者。该患者在接受 4 个周期 GDP(吉西他滨、地塞米松、顺铂)治疗后获得部分缓解,并在全身照射、环磷酰胺和阿糖胞苷预处理的脐带血移植后达到完全缓解(CR)。移植后 7 个月,患者出现认知障碍。脑部磁共振成像显示右侧大脑脚和内囊的高强度病变。脑脊液检查证实 MEITL 中枢神经系统(CNS)复发。在接受 3 个周期 MVP(甲氨蝶呤、丙卡巴肼、长春新碱)治疗后行全脑放疗后,其认知障碍得到改善。由于疾病进展,在 CNS 复发后 6 个月,患者死亡。鉴于该患者在 GDP 和 CBT 达到 CR 后发生 CNS 复发,在一线治疗期间进行 CNS 预防可能对 MEITL 的治疗有益。