骨髓增生异常综合征并发恶性淋巴瘤1例尸检报告。

T. Kageyama, H. Morikawa, H. Ohyabu, T. Takubo, Y. Shibayama, K. Hashimoto, Ryuichi Yamamoto, K. Nakata
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引用次数: 0

摘要

报告1例骨髓增生异常综合征(MDS)合并恶性淋巴瘤。患者男,68岁,因严重贫血入院,经血液学检查及骨髓活检诊断为MDS。类固醇激素、合成代谢激素制剂和输血治疗均不成功,6个月后死于感染。尸检发现骨髓发育不全,母细胞分化很差,巨核细胞数量减少,红细胞细胞核异常。全身性淋巴结病变(气管旁、肠系膜旁、胰腺旁及主动脉旁)。淋巴结组织学检查显示中等大小淋巴细胞弥漫性增生,有B细胞标记,呈浆细胞样分化。淋巴瘤组织中大型单核吞噬细胞的星空星座与伯基特淋巴瘤的组织学非常相似。有广泛性隐球菌病和念珠菌病的证据。恶性淋巴瘤合并MDS是非常罕见的,但本病例的存在提示MDS中发生了多能造血干细胞的克隆异常。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Myelodysplastic Symdrome Complicated by Malignant Lymphoma : Report of an Autopsy Case.
A case of myelodysplastic syndrome (MDS) complicated by malignant lymphoma is reported. The patient was a 68-year-old male who was admitted to hospital with severe anemia and was diagnosed as having MDS on the basis of hematological examinations and the findings of bone marrow biopsy. Treatment with steroid hormones, anabolic hormone preparations, and blood transfusion proved unsuccessful and he died of infection six months later.At autopsy, the bone marrow was hypoplastic and blast cells showed very poor differentiation, the megakaryocytes were decreased in number, and the erythroblasts had abnormal nuclei. Systemic lymphadenopathy (paratracheal, mesenteric, parapancreatic and paraaortic) was found. Histological examination of the lymph-nodes revealed diffuse proliferation of medium sized lymphocytes which had B cell markers and showed plasmacytoid differentiation.The starry sky constellation of large mononuclear phagocytes in the lymphoma tissue was quite similar to the histology of Burkitt's lymphoma. There was evidence of generalized cryptococcosis and candidiasis. Malignant lymphoma complicating MDS is very rare, but the exsistence of this case suggests that a clonal abnormality of the pluripotent hematopoietic stem cells occurs in MDS.
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