Intravascular Large B-Cell Lymphoma

被引:61
作者
Orwat, Dennis E. [1 ]
Batalis, Nicholas I. [1 ]
机构
[1] Med Univ S Carolina, Dept Pathol & Lab Med, Charleston, SC 29425 USA
关键词
MALIGNANT ANGIOENDOTHELIOMATOSIS; CLINICAL PRESENTATION; HEMOPHAGOCYTOSIS; MANAGEMENT; DIAGNOSIS; NEOPLASM; VARIANT;
D O I
10.5858/arpa.2010-0747-RS
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
A rare type of diffuse large B-cell lymphoma, intravascular large B-cell lymphoma primarily affects the middle-aged to elderly population, with a slight predominance in men. By the time of presentation, most patients have advanced, disseminated disease, and often the diagnosis is made at autopsy. Patients may present with any of a myriad of symptoms, with any tissue potentially being infiltrated. Central nervous system and cutaneous involvement is common, as is the presence of B symptoms including fever, weight loss, and night sweats. Morphologically, growth of neoplastic cells is restricted to the lumen of small vessels. The cells are large, with 1 or more prominent nucleoli, scant cytoplasm, and frequent mitotic figures, and are commonly positive for cluster of differentiation markers 79a, 20, and 19, as well as MUM1/IRF4 and Bcl-2. Intravascular large B-cell lymphoma is aggressive, and without treatment is rapidly fatal. (Arch Pathol Lab Med. 2012;136:333-338; doi:10.5858/arpa.2010-0747-RS)
引用
收藏
页码:333 / 338
页数:6
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