[Clinical features of three cases with primary bone marrow lymphoma].

Hidetaka Nakagaki, Takahiro Shima, Reiko Yoneda, Masayasu Hayashi, Sae Utsumi, Seiya Hirakawa, Chiaki Kubara, Ken Takigawa, Akihisa Yoshino, Mariko Minami, Yayoi Matsuo, Takuro Kuriyama, Shuichi Taniguchi, Tetsuya Eto
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Abstract

Primary bone marrow lymphoma (PBML) is a malignant lymphoma characterized by proliferation of lymphoma cells exclusively in the bone marrow without lymphadenopathy. Despite the dismal prognosis of PBML, it is a very rare lymphoma with limited evidence concerning its pathophysiology, making accumulation of cases important. We herein report three cases of PBML at our institution. The first patient was an 80-year-old man who presented with hemophagocytic syndrome and pancytopenia at admission, and died of septic shock during initial chemotherapy. The second patient was a 64-year-old man who achieved complete remission with intensive chemotherapies, but relapsed shortly after completing the final chemotherapy course. The third patient was a 66-year-old woman who underwent chemotherapies and allogeneic hematopoietic stem cell transplantation, only to relapse shortly after transplantation. Although early intervention with chemotherapy is essential for PBML treatment, diagnosis of PBML is very challenging due to the absence of lymph node involvement. Moreover, treatment outcomes of existing chemotherapy and transplantation therapies for PBML are still poor. Further accumulation of cases and development of new treatment strategies are desirable.

【原发性骨髓淋巴瘤3例临床分析】。
原发性骨髓淋巴瘤(PBML)是一种恶性淋巴瘤,其特征是淋巴瘤细胞仅在骨髓中增殖而无淋巴结病变。尽管PBML预后不佳,但它是一种非常罕见的淋巴瘤,有关其病理生理的证据有限,因此病例积累很重要。我们在此报告三例PBML在我们的机构。第一位患者为80岁男性,入院时表现为噬血细胞综合征和全血细胞减少症,在初始化疗期间死于感染性休克。第二位患者是一位64岁的男性,他通过强化化疗获得了完全缓解,但在完成最后的化疗疗程后不久复发。第三例患者是一名66岁的女性,她接受了化疗和异体造血干细胞移植,移植后不久复发。尽管早期化疗干预对PBML治疗至关重要,但由于没有淋巴结累及,PBML的诊断非常具有挑战性。此外,现有的化疗和移植疗法对PBML的治疗效果仍然很差。进一步积累病例和发展新的治疗策略是可取的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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