[Successful treatment with EPOCH followed by mogamulizumab for angioimmunoblastic T-cell lymphoma with myelofibrosis and pure red cell aplasia].

Ayako Yamamura, Kisato Nosaka, Hiro Tatetsu, Jun-Ichirou Yasunaga
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Abstract

The patient was a 70-year-old woman. She visited a doctor for sore throat, facial edema, and lymphadenopathy. Lymph node biopsy was performed, and led to a diagnosis of AITL. When the patient was admitted to our department for treatment, she had severe anemia with Hb 4.2 g/dl, and bone marrow aspiration resulted in a dry tap. Bone marrow biopsy revealed infiltration of tumor cells, a severe decrease in erythroblastic cells, and bone marrow fibrosis. Although lymph node size was reduced by THP-COP, bone marrow biopsy showed residual infiltration of tumor cells. In addition, since low reticulocyte counts and anemia persisted, weekly red blood cell transfusions were required. Therefore, the treatment regimen was changed to EPOCH. After two cycles of EPOCH, anemia improved, and red cell transfusions were no longer required. Bone marrow examination after four cycles showed disappearance of lymphoma cells and no evidence of bone marrow fibrosis or erythroblasts. However, due to residual bone marrow infiltration of lymphoma cells, treatment with mogamulizumab was started. Complete remission was maintained for five years. This indicates that EPOCH and mogamulizumab are treatment options for AITL patients with bone marrow involvement, PRCA, and myelofibrosis.

患者是一名 70 岁的妇女。她因咽喉疼痛、面部水肿和淋巴结病就诊。医生对其进行了淋巴结活检,诊断为 AITL。患者入住我科接受治疗时,出现严重贫血,血红蛋白为 4.2 g/dl,骨髓穿刺结果为干结。骨髓活检显示肿瘤细胞浸润,红细胞严重减少,骨髓纤维化。虽然 THP-COP 使淋巴结缩小,但骨髓活检显示肿瘤细胞仍在浸润。此外,由于网织红细胞计数低和贫血持续存在,每周都需要输红细胞。因此,治疗方案改为 EPOCH。经过两个周期的 EPOCH 治疗后,贫血有所改善,不再需要输红细胞。四个周期后的骨髓检查显示淋巴瘤细胞消失,没有发现骨髓纤维化或红细胞。然而,由于骨髓中仍有淋巴瘤细胞浸润,患者开始接受莫干单抗治疗。完全缓解维持了五年。这表明,EPOCH和mogamulizumab是骨髓受累、PRCA和骨髓纤维化的AITL患者的治疗选择。
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