[An unusual cause of pancytopenia: intravascular lymphoma].

Annales de medecine interne Pub Date : 2003-09-01
Elisabeth Parisi, Marie-Claire Tortel, Marie-Pierre Chenard, Pascale Raby, Isabelle Mazurier, Philippe Moskovtchenko
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Abstract

We report an observation of intravascular lymphoma occurring in a 69-year-old woman. This relatively rare disease presents polymorphic clinical features that render diagnosis difficult. Cutaneous and central nervous system signs and symptoms are frequently observed and should be recognized as suggestive of intravascular lymphoma. However, they are not always observed, in our case only pancytopenia was present. Pronostic is generally unfavorable but good response to chemotherapy has been described with early diagnosis. A large number of pathogenic hypotheses have been put forward: abnormality of cellular receptor, role of Epstein-Barr virus or transformation from lymphoma. Intravascular lymphoma should be included in the differential diagnosis of pancytopenia to increase chances of good response.

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【全血细胞减少症的不寻常原因:血管内淋巴瘤】。
我们报告一例发生在69岁女性的血管内淋巴瘤。这种相对罕见的疾病表现出多形态的临床特征,使诊断变得困难。皮肤和中枢神经系统的体征和症状经常被观察到,应该被认为是血管内淋巴瘤的提示。然而,它们并不总是被观察到,在我们的病例中只有全血细胞减少症存在。前列腺增生通常是不利的,但早期诊断对化疗反应良好。人们提出了大量的致病假说:细胞受体异常、eb病毒作用或淋巴瘤转化。血管内淋巴瘤应列入全血细胞减少症的鉴别诊断,以增加良好反应的机会。
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