Further Evidence That Malignant Angioendotheliomatosis Is an Angiotropic Large-Cell Lymphoma

作者：Khalil Sheibani, Hector A. Battifora, Carl D. Winberg, Jerome S. Burke, Jonathan M. Ben‐Ezra, Gary Ellinger, Nelson J. Quigley, Balbino B. Fernandez, Dwight Morrow, Henry Rappaport · 发表于：New England Journal of Medicine · 年份：1986 · DOI：10.1056/nejm198604103141502 · 被引用次数：301 · 研究领域：Lymphoma Diagnosis and Treatment、CNS Lymphoma Diagnosis and Treatment、Vascular Tumors and Angiosarcomas

Malignant angioendotheliomatosis is a rare, generally fatal disease characterized by a multifocal proliferation of neoplastic mononuclear cells within the lumens of small blood vessels. Although the disease primarily involves the vasculature of the skin and central nervous system, vascular involvement of other organs may occur and may produce a variety of clinical findings. Some early investigators concluded that malignant angioendotheliomatosis was a neoplasm of endothelial cells, but recently others have suggested that it is of hematopoietic origin. We have studied three patients with the disease and have characterized the immunophenotype of the neoplasm on cryostat-cut fresh-frozen tissues. A detailed antigenic phenotyping of neoplastic lymphoid cells showed that one patient had the immunophenotype T11+, Leu-1+, Leu-3+, Leu-2+, B1-, B2-, SIg-, LN1-, LN2-, the predominant phenotype for peripheral T-cell lymphoma; the others had T11-, Leu-1-, Leu-3-, Leu-2-, B1+, B2+, SIg+, LN1+, LN2+, consistent with a B-cell-derived lymphoma. On the basis of our results, we suggest that angiotropic (intravascular) large-cell lymphoma would be more appropriate than malignant angioendotheliomatosis as a name for this disease.