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肥大细胞增多症与并存的非霍奇金淋巴瘤和骨髓增殖性疾病。

Mastocytosis and co-existent non-Hodgkin's lymphoma and myeloproliferative disorders.

作者信息

Hutchinson R M

机构信息

Department of Haematology, Leicester Royal Infirmary, U.K.

出版信息

Leuk Lymphoma. 1992 May;7(1-2):29-36. doi: 10.3109/10428199209053599.

Abstract

Generalised mastocytosis is a rare condition characterised by the clinical features of the release of vasoactive peptides from tissue mast cells infiltrating in the reticuloendothelial tissues. The mast cell however appears to have its origin in the pluripotential bone marrow stem cell committed to a basophil and it is therefore not surprising that myeloproliferative and myelodysplastic disorders commonly co-exist or terminate the clinical phase of mastocytosis. Both abnormal proliferation and maturation of the myeloid committed cells are found. Non-Hodgkin's lymphoma can occur before and after mastocytosis becomes manifest. While this is statistically a random event the relationship between lymphokines and mast cell differentiation and proliferation raises the possibility of a benign reactive lymphoid event eventually becoming malignant.

摘要

全身性肥大细胞增多症是一种罕见疾病,其临床特征为浸润于网状内皮组织中的组织肥大细胞释放血管活性肽。然而,肥大细胞似乎起源于定向分化为嗜碱性粒细胞的多能骨髓干细胞,因此骨髓增殖性疾病和骨髓增生异常综合征通常并存或终结肥大细胞增多症的临床阶段也就不足为奇了。发现髓系定向细胞存在异常增殖和成熟现象。非霍奇金淋巴瘤可在肥大细胞增多症显现之前或之后发生。虽然从统计学角度来看这是一个随机事件,但淋巴因子与肥大细胞分化和增殖之间的关系增加了良性反应性淋巴样事件最终恶变的可能性。

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