Lymphomatoid granulomatosis--a single institute experience: pathologic findings and clinical correlations.
Lymphomatoid granulomatosis--a single institute experience: pathologic findings and clinical correlations.
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DOI：
10.1097/pas.0000000000000328
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发表时间：
2015-02
期刊：
影响因子：
--
通讯作者：
Jaffe ES
中科院分区：
文献类型：
--
作者：
Song JY;Pittaluga S;Dunleavy K;Grant N;White T;Jiang L;Davies-Hill T;Raffeld M;Wilson WH;Jaffe ES
Lymphomatoid granulomatosis (LYG) is a rare angiocentric and angiodestructive EBV-associated B-cell lymphoproliferative disorder. It is hypothesized that these patients have dysregulated immune surveillance of EBV. We reviewed the biopsies of 55 patients with LYG who were referred for a prospective trial at the NCI (1995–2010) and evaluated the histologic, immunohistochemical, in situ hybridization, and molecular findings of these biopsies in conjunction with clinical information. Grading of the lesions was based on morphologic features and the number of EBV-positive B cells. The median age was 46 years (M:F 2.2:1). Clinically, all patients had lung involvement (100%) with the next most common site being the central nervous system (38%). No patient had nodal or bone marrow disease. All patients had past EBV exposure by serology but with a low median EBV viral load. We reviewed 122 biopsies; the most common site was lung (73%) followed by skin/subcutaneous tissue (17%); other sites included kidney, nasal cavity, gastrointestinal tract, conjunctiva, liver, and adrenal gland. Histologically the lesions showed angiocentricity, were rich in T cells, had large atypical B cells, and were positive for EBV. Grading was performed predominantly on the lung biopsy at diagnosis; they were distributed as follows: LYG grade 1 (30%), grade 2 (22%), and grade 3 (48%). Necrosis was seen in all grades with a greater degree in high-grade lesions. Immunoglobulin gene rearrangement studies were performed and a higher percentage of clonal rearrangements were seen in LYG grade 2 (50%) and grade 3 (69%) as compared to grade 1 (8%). LYG is a distinct entity that can usually be differentiated from other EBV-associated B-cell lymphoproliferative disorders based on the combination of clinical presentation, histology, and EBV studies. Grading of these lesions is important because it dictates the treatment choice.
