Escribano L, Ocqueteau M, Almeida J, Orfao A, San Miguel J F
Department of Hematology, Hospital Ramón y Cajal, Universidad de Alcalá de Henares, Madrid, Spain.
Leuk Lymphoma. 1998 Aug;30(5-6):459-66. doi: 10.3109/10428199809057558.
The c-kit proto-oncogen (CD 117) has been shown to be present in several cell types including normal and neoplastic hemopoietic cells. Among normal BM cells, CD117 expression has been found in about half of the CD34+ precursors including progenitors committed to the erythroid, granulo-monocytic, and megakaryocytic cell lineages. In addition, strong CD117 expression is detected in bone marrow mast cells as well as in a small subset of NK cells displaying strong reactivity for CD56, and in a relatively important proportion of CD3 /CD4 /CD8 prothymocytes. These results suggest that CD117 expression can be detected in both myeloid and lymphoid lineages although for the lymphoid lineage it would be restricted to a small NK-cell subset and early T-cell precursors. In acute leukemias CD117 expression was initially associated with AML. Nevertheless, at present it is well established that CD 117 expression may also be found in a relatively important proportion of T-ALL while it is usually absent in B-lineage ALL. Moreover, recent studies have shown that in about one-third of multiple myeloma cases and patients with monoclonal gammopathy of undetermined significance plasma cells display reactivity for CD1117. The prognostic influence of CD117 expression has not yet been clearly established. The analysis of this marker may also be of value for the investigation of minimal residual disease (MRD). It has been suggested that CD117 in combination with other antigens may be of great help for the identification of leukemia-associated phenotypes that could be used to monitor MRD in both acute myeloid leukemias and multiple myeloma patients achieving morphological complete remission.
原癌基因c-kit(CD117)已被证明存在于多种细胞类型中,包括正常和肿瘤性造血细胞。在正常骨髓细胞中,已发现约一半的CD34+前体细胞表达CD117,这些前体细胞包括定向于红系、粒-单核系和巨核系细胞谱系的祖细胞。此外,在骨髓肥大细胞以及一小部分对CD56有强烈反应性的NK细胞中,以及在相对较大比例的CD3 /CD4 /CD8原胸腺细胞中,均检测到强CD117表达。这些结果表明,虽然对于淋巴系来说,CD117表达仅限于一小部分NK细胞亚群和早期T细胞前体,但在髓系和淋巴系中均可检测到CD117表达。在急性白血病中,CD117表达最初与急性髓系白血病(AML)相关。然而,目前已明确,在相对较大比例的T细胞急性淋巴细胞白血病(T-ALL)中也可发现CD117表达,而在B系急性淋巴细胞白血病(B-ALL)中通常不存在。此外,最近的研究表明,在约三分之一的多发性骨髓瘤病例和意义未明的单克隆丙种球蛋白病患者中,浆细胞对CD117有反应性。CD117表达的预后影响尚未明确确立。该标志物的分析对于微小残留病(MRD)的研究可能也有价值。有人提出,CD117与其他抗原联合使用,可能有助于识别白血病相关表型,可用于监测急性髓系白血病和多发性骨髓瘤患者形态学完全缓解后的MRD。