Petrella Tony, Wechsler Janine, Courville Philippe, de Muret Anne, Bosq Jacques, Déchelotte Pierre, Feuillard Jean, Durlach Anne, Vergier Béatrice
Centre de Pathologie, 33 rue Nicolas Bornier, BP189, 21005, Dijon Cedex, France.
Ann Pathol. 2004 Jun;24(3):241-55; quiz 227. doi: 10.1016/s0242-6498(04)93959-4.
Hematodermic CD4/CD56 neoplasm is a recently described entity. This name has been initially proposed by the French Study Group on Cutaneous Lymphomas which established the primary anatomoclinical and pathogenic and cytogenic bases of the disease in 1999. This descriptive and provisional name allowed conceptualizing the entity by its main clinical and phenotypical characteristics. The first case in the literature goes back to 1994. Since that time, several other cases have been published. The expression of CD56 led most of the authors to propose an NK-cell lineage origin. In the last WHO classification of lymphomas, the entity was indexed under the name of "blastic NK-cell lymphoma". However, the authors underlined that there were currently no clues to the etiology of blastic NK-cell lymphoma and that the precise lineage of this disease was still unresolved. At the clinical level the main characteristics of the disease are the skin tropism and the occurrence of a leukemic phase at any time during the course of the disease. The median age is 59 but pediatric cases do exist. At the morphological level skin biopsy shows a monomorphous cell proliferation simulating a pleomorphic T cell cutaneous lymphoma. The diagnosis is based on phenotypic criteria which require frozen tissue. Currently, the main characteristics are the expression of CD4 and CD56 antigens while the main defined lineage specific markers are negative (B-cell, T-cell, NK-cell and myeloid-cell lineages). The origin of the tumor cells still remains uncertain but the plasmacytoid dendritic cell is presently a very serious candidate. The tumor cells share a great phenotypical homology and particularly the expression of the CD123 antigen. Functional homologies have also been demonstrated with tumor cells in vitro. Outcome of CD4/CD56 hematodermic neoplasms is very bad. The median time of survival is 14 months irrespective of the treatment given. Conventional chemotherapies used for the treatment of aggressive lymphomas or acute myeloid leukemias are quickly inefficient.
皮肤CD4/CD56肿瘤是一种最近才被描述的疾病实体。这个名称最初是由法国皮肤淋巴瘤研究小组提出的，该小组于1999年确立了该病的主要解剖临床、致病和细胞遗传学基础。这个描述性的临时名称使其能够根据其主要临床和表型特征来界定该疾病实体。文献中报道的首例病例可追溯到1994年。从那时起，又有其他几例病例被发表。CD56的表达使得大多数作者认为其起源于NK细胞系。在世界卫生组织最新的淋巴瘤分类中，该疾病实体被归为“母细胞性NK细胞淋巴瘤”。然而，作者们强调，目前尚无母细胞性NK细胞淋巴瘤病因的线索，其确切来源仍未明确。在临床方面，该病的主要特征是皮肤嗜性以及在病程中的任何时候都可能出现白血病期。中位年龄为59岁，但也有儿科病例。在形态学上，皮肤活检显示单形性细胞增殖，类似多形性T细胞皮肤淋巴瘤。诊断基于表型标准，这需要冷冻组织。目前，其主要特征是CD4和CD56抗原的表达，而主要的明确谱系特异性标志物均为阴性（B细胞、T细胞、NK细胞和髓细胞系）。肿瘤细胞的起源仍然不确定，但浆细胞样树突状细胞目前是一个很受关注的候选来源。肿瘤细胞具有很大的表型同源性，尤其是CD123抗原的表达。体外研究也证实了肿瘤细胞之间的功能同源性。皮肤CD4/CD56肿瘤的预后非常差。无论接受何种治疗，中位生存时间为14个月。用于治疗侵袭性淋巴瘤或急性髓细胞白血病的传统化疗很快就会失效。
