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骨髓纤维化合并T细胞淋巴瘤,随后发生白血病转化

[Myelofibrosis complicated by T-cell lymphoma followed by leukemic transformation].

作者信息

Nakase K, Hasegawa M, Suzuki Y, Tamaki S, Tanigawa M, Ikeda T, Tsuji K, Miyanishi E

机构信息

Department of Internal Medicine, Yamada Red Cross Hospital, Japan.

出版信息

Rinsho Ketsueki. 1995 Nov;36(11):1284-8.

PMID:8691569
Abstract

A 37-year-old man was admitted because of pancytopenia with leukoerythroblastosis and anisocytosis in January 1986. Bone marrow aspiration resulted in a dry tap and biopsy showed marked myelofibrosis. Three months after admission, generalized lymph node swelling and multiple skin tumors were recognized. A biopsied lymph node revealed lymphoblastic lymphoma. The surface markers of lymphoma cells showed an immature T-cell phenotype, whereas T-cell receptor beta and gamma chain genes showed germ line configuration. The patient was treated with combination chemotherapy in June 1986. A month later, he developed leukemic transformation with features of acute myelocytic leukemia and he died of pneumonia. Autopsy disclosed extramedullary hematopoiesis in the liver and spleen. Primary myelofibrosis complicated by T-cell lymphoma is extremely rare.

摘要

一名37岁男性于1986年1月因全血细胞减少伴幼稚粒-幼红细胞血象和红细胞大小不均而入院。骨髓穿刺呈干抽,活检显示明显的骨髓纤维化。入院三个月后,发现全身淋巴结肿大和多处皮肤肿瘤。淋巴结活检显示为淋巴母细胞淋巴瘤。淋巴瘤细胞的表面标志物显示为不成熟的T细胞表型,而T细胞受体β和γ链基因显示为种系构型。该患者于1986年6月接受联合化疗。一个月后,他发生了急性髓细胞白血病特征的白血病转化,并死于肺炎。尸检发现肝脏和脾脏有髓外造血。原发性骨髓纤维化合并T细胞淋巴瘤极为罕见。

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