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Literature Review of Intravascular Lymphomatosis Shigeru Koyano 1 , Shunta Hashiguchi 2 , Fumiaki Tanaka 1 1Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine 2Department of Neurology, Saiseikai Yokohama-shi Nanbu Hospital Keyword: 血管内リンパ腫 , 大細胞B型リンパ腫 , 皮膚ランダム生検 , リツキシマブ , intravascular lymphomatosis , large B-cell lymphoma , random skin biopsy , rituximab pp.927-946
Published Date 2014/8/1
DOI https://doi.org/10.11477/mf.1416101863
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Abstract

Intravascular lymphoma (IVL) is a rare form of malignant lymphoma characterized by the selective growth of lymphoma cells within the lumina of vessels, without the involvement of adjacent parenchymal tissue. IVL is predominantly of B-cell lineage, but cases of T-cell or natural killer cell lineage have been described occasionally, predominantly involving the skin. IVL usually affects elderly patients with a poor performance status, elevated serum lactic dehydrogenase levels, anemia, and B symptoms. The clinical presentation varies in different geographical areas, particularly between patients diagnosed in Europe and Asia. In European countries, the Western variant of IVL mainly involves the central nervous system and skin; in particular, there is a "cutaneous variant" limited to the skin. In Asian countries, the Asian variant of IVL predominantly accompanies hemophagocytic syndrome. Identification of this disease is difficult because it presents with non-specific clinical symptoms. Although organ biopsies are mandatory for accurate IVL diagnosis, no standard procedure has been established. An additional random skin biopsy may be useful to diagnose IVL at an early stage. Early diagnosis and treatment can improve the outcome of IVL patients following treatment with rituximab-containing chemotherapy.


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電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院

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