Wells S J, Bray R A, Stempora L L, Farhi D C
Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA.
Am J Clin Pathol. 1996 Aug;106(2):192-5. doi: 10.1093/ajcp/106.2.192.
CD117 is a transmembrane protein receptor encoded by the c-kit proto-oncogene. The CD117 ligand is stem cell factor, an important hematopoietic regulator. CD117 is present on approximately 4% of normal bone marrow mononuclear cells and in acute myelogenous leukemia (AML) and chronic myelogenous leukemia in myeloid blast crisis, but rarely in acute lymphoblastic leukemia (ALL). Initially viewed as a primitive myeloid marker, CD117 has been identified in all FAB subtypes of AML and may predict poor outcome. CD34, a primitive stem cell marker, may also predict poor outcome. The aim of this study was to examine the relationship between CD117 and CD34 expression on leukemic blasts and to determine whether CD117 is related to lymphoid-associated antigen (LAA) expression in AML. Consecutive bone marrow samples were studied from cases of AML (30 cases), myelodysplastic syndromes (MDS) (4 cases), myeloproliferative disorders in blast crisis (MPD-BC) (6 cases), and ALL (5 cases). Cases were diagnosed according to FAB criteria and included M0 (3 cases), M1 (2 cases), M2 (13 cases), M3 (1 case), M4 (6 cases), M5 (3 cases), M6 (1 case), AML NOS (1 case), RAEB (3 cases), and RAEB-T (1 case). CD117 and CD34 were analyzed by multiparameter flow cytometry. Blasts in 10 de novo AML samples were CD117+/CD34+ in 4 cases, CD117+/CD34-in 3 cases, CD117-/CD34+ in 1 case, and CD117-/ CD34- in 2 cases. Blasts in 20 cases of relapsed AML were CD117+/ CD34+ in 13 cases, CD117+/CD34- in 6 cases, and CD117-/CD34+ in 1 case. Blasts in MDS were CD117+/CD34+ in 3 cases, CD117-/ CD34+ in 1 case. Blasts in MPD-BC were CD117+/CD34+ in 4 cases, CD117-/CD34+ in 2 cases. Blasts in ALL were CD117+/CD34+ in 1 case, CD117-/CD34+ in 1 case, CD117-/CD34- in 3 cases. Of 26 cases of CD117+ AML, CD4 was expressed in 15 (58%) cases, CD7 in 7 (27%) cases, and CD2 in 2 (8%) cases. CD117/CD34 expression did not correlate with FAB subtype of AML. CD117 is borne on most leukemic blasts of myeloid origin (in this study, 87% of AML, 80% of MPD-myeloid BC, and 75% of MDS) and does not exclude expression of LAA. Although CD117 is a receptor for stem cell factor, its expression does not appear to correlate with CD34 positivity.
CD117是一种由原癌基因c-kit编码的跨膜蛋白受体。CD117的配体是干细胞因子，一种重要的造血调节因子。CD117存在于约4%的正常骨髓单个核细胞以及急性髓系白血病（AML）和处于髓系原始细胞危象的慢性髓系白血病中，但在急性淋巴细胞白血病（ALL）中很少见。CD117最初被视为一种原始髓系标志物，已在AML的所有FAB亚型中被鉴定出来，并且可能预示预后不良。CD34，一种原始干细胞标志物，也可能预示预后不良。本研究的目的是检测白血病原始细胞上CD117与CD34表达之间的关系，并确定CD117是否与AML中的淋巴系相关抗原（LAA）表达有关。对AML患者（30例）、骨髓增生异常综合征（MDS）患者（4例）、处于原始细胞危象的骨髓增殖性疾病（MPD-BC）患者（6例）和ALL患者（5例）的连续骨髓样本进行了研究。病例根据FAB标准进行诊断，包括M0（3例）、M1（2例）、M2（13例）、M3（1例）、M4（6例）、M5（3例）、M6（1例）、AML未分类（1例）、难治性贫血伴原始细胞过多（RAEB）（3例）和难治性贫血伴原始细胞过多转变型（RAEB-T）（1例）。通过多参数流式细胞术分析CD117和CD34。10例初发AML样本中的原始细胞，4例为CD117+/CD34+，3例为CD117+/CD34-，1例为CD117-/CD34+，2例为CD117-/CD34-。20例复发AML样本中的原始细胞，13例为CD117+/CD34+，6例为CD117+/CD34-，1例为CD117-/CD34+。MDS样本中的原始细胞，3例为CD117+/CD34+，1例为CD117-/CD34+。MPD-BC样本中的原始细胞，4例为CD117+/CD34+，2例为CD117-/CD34+。ALL样本中的原始细胞，1例为CD117+/CD34+，1例为CD117-/CD34+，3例为CD117-/CD34-。在26例CD117+的AML病例中，15例（58%）表达CD4，7例（27%）表达CD7，2例（8%）表达CD2。CD117/CDJ4表达与AML的FAB亚型无关。CD117存在于大多数髓系来源的白血病原始细胞上（在本研究中，87%的AML、80%的MPD-髓系BC和75%的MDS），并且不排除LAA的表达。虽然CD117是干细胞因子的受体，但其表达似乎与CD34阳性无关。
