FURTHER EVIDENCE THAT MALIGNANT ANGIOENDOTHELIOMATOSIS IS AN ANGIOTROPIC LARGE-CELL LYMPHOMA
FURTHER EVIDENCE THAT MALIGNANT ANGIOENDOTHELIOMATOSIS IS AN ANGIOTROPIC LARGE-CELL LYMPHOMA
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DOI：
10.1056/nejm198604103141502
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发表时间：
1986-04-10
影响因子：
158.5
通讯作者：
RAPPAPORT, H
中科院分区：
文献类型：
--
作者：
SHEIBANI, K;BATTIFORA, H;RAPPAPORT, H
恶性血管内皮瘤病是一种罕见的、通常是致命的疾病，其特征是小血管腔内肿瘤性单核细胞的多灶性增殖。虽然本病主要累及皮肤和中枢神经系统的脉管系统，但也可累及其他器官的血管，并可产生各种临床表现。一些早期研究者认为恶性血管内皮瘤病是一种内皮细胞肿瘤，但最近其他人认为它是造血起源。我们研究了三名患者的疾病，并在冷冻器切割的新鲜冷冻组织上描述了肿瘤的免疫表型。肿瘤淋巴样细胞的详细抗原表型显示，1例患者的免疫表型为T11+、Leu-1+、Leu-3+、Leu-2+、B1-、B2-、Sig-、LN1-、LN2-，这是周围t细胞淋巴瘤的主要表型；其余为T11-、Leu-1-、Leu-3-、Leu-2-、B1+、B2+、Sig+、LN1+、LN2+，符合B细胞源性淋巴瘤。根据我们的研究结果，我们认为血管性（血管内）大细胞淋巴瘤比恶性血管内皮瘤病更适合作为这种疾病的名称。
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.