Clinical and biologic features of CD4+CD56+ malignancies
Clinical and biologic features of CD4+CD56+ malignancies
复制标题
DOI：
10.1182/blood.v99.5.1556
复制
发表时间：
2002-03-01
期刊：
影响因子：
20.3
通讯作者：
Béné, MC
中科院分区：
文献类型：
--
作者：
Feuillard, J;Jacob, MC;Béné, MC
CD4(+)CD56（+）恶性肿瘤是一种罕见的血液肿瘤，最近被证明与所谓的2型树突状细胞（DC2）或浆细胞样树突状细胞相对应。本研究介绍了一系列23例此类病例的生物学和临床特征，这些病例选择了定义DC2白血病对应物的最小免疫表型标准，即在缺乏B、T和髓系标记物的情况下，CD4和CD56的共表达。临床表现典型为皮肤结节伴淋巴结病或脾肿大，或两者兼而有之。细胞减少症频繁发生。循环恶性细胞常被检出。23例患者中有20例（87%）出现大量骨髓浸润。大多数肿瘤细胞的细胞核具有花边染色质，具有胚性，质膜旁有大的细胞质液泡或微液泡，细胞质扩张似假足。其他免疫表型特征包括阴性（CD16、CD57、CD116和CD117）和阳性（CD36、CD38、低水平CD45、CD45RA、CD68、CD123和HLA DR）标记物。在没有化疗的情况下，预后是迅速致命的。23例患者中有18例（78%）在多次化疗后获得完全缓解。大多数患者在2年内复发，主要复发于骨髓、皮肤或中枢神经系统。考虑到这些临床和生物学特征，结论是CD4(+)CD56（+）恶性肿瘤构成了一个真正的同质实体。此外，还明确了一些治疗方案。最后，讨论了单纯皮肤惰性形式的疾病与急性白血病的关系，以及与CD4(+)CD56（+）恶性细胞的淋巴/髓系起源的关系。(C) 2002年由美国血液病学会出版。
CD4(+)CD56(+) malignancies are rare hematologic neoplasms, which were recently shown to correspond to the so-called type 2 dendritic cell (DC2) or plasmacytoid dendritic cells. This study presents the biologic and clinical features of a series of 23 such cases, selected on the minimal immunophenotypic criteria defining the DC2 leukemic counterpart, that is, coexpression of CD4 and CD56 in the absence of B, T, and myeloid lineage markers. Clinical presentation typically corresponded to cutaneous nodules associated with lymphadenopathy or spleen enlargement or both. Cytopenia was frequent. Circulating malignant cells were often detected. Massive bone marrow infiltration was seen in 20 of 23 (87%) patients. Most tumor cells exhibited nuclei with a lacy chromatin, a blastic aspect, large cytoplasm-containing vacuoles or microvacuoles beside the plasma membrane, and cytoplasmic expansions resembling pseudopodia. Other immunophenotypic characteristics Included both negative (CD16, CD57, CD116, and CD117) and positive (CD36, CD38, CD45 at low levels, CD45RA, CD68, CD123, and HLA DR) markers. The prognosis was rapidly fatal in the absence of chemotherapy. Complete remission was obtained in 18 of 23 (78%) patients after polychemotherapy. Most patients had a relapse In less than 2 years, mainly in the bone marrow, skin, or central nervous system. Considering these clinical and biologic features, the conclusion is made that CD4(+)CD56(+) malignancies constitute a genuine homogeneous entity. Furthermore, some therapeutic options were clearly identified. Finally, relationships between the pure cutaneous indolent form of the disease and acute leukemia as well as with the lymphoid/myeloid origin of the CD4(+)CD56(+) malignant cell are discussed. (C) 2002 by The American Society of Hematology.