title: Enhanced myeloid specificity of CD117 compared with CD13 and CD33.
abstract: The c-kit proto-oncogene encodes a 145 kd tyrosine kinase transmembrane receptor, which plays a key role in haemopoiesis. The c-kit has been classified as CD117 and is especially useful in the differential diagnosis of acute myelogenous leukemia (AML) and acute lymphoblastic leukemia (ALL). We analysed 104 consecutive cases (55 AML, 23 B-cell lineage ALL, three T-cell ALL, 11 blast crisis of chronic myeloproliferative disorders and 12 cases of myelodysplastic syndromes with more than 10% of blasts) referred to our Hospital for immunophenotypic diagnosis and compared the expression pattern of CD13, CD33 and CD117 using the same fluorochrome (phycoerythrin-PE). The recommendations of the EGIL group were followed in order to establish lineage involvement of the blastic population. The threshold used to assign positivity for CD117 was 10%. Bcr/abl, TEL/AML-1 and MLL rearrangements were assessed by molecular methods. CD117 expression was detected in 91% of AML and MDS. All the negative cases corresponded to acute monocytic leukemias. The calculated specificity for myeloid involvement was 0.86 for CD117, 0.36 for CD13 and 0.44 for CD33 (P < 0.005). CD117 was also positive in four cases of ALL. None of these cases showed bcr/abl or MLL rearrangements. In the light of these findings, CD117 expression should yield a higher score, at least one point, in the system currently applied for the diagnosis of biphenotypic acute leukemias (BAL) as its myeloid specificity is greater than that of CD13 and CD33. Moreover, its absence in AML could identify two subgroups of M5b cases. The coexpression of CD117 with cytoplasmic CD79a is often associated with CD7 reactivity, suggesting a stem cell disorder. CD117 should be included on a routine basis for the immunophenotypic diagnosis of acute leukemias.
authors.affiliation: Secció de Marcadors, Department d'Hematologia, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. jfnomde@santpau.es
authors.forename: J F
authors.initials: JF
authors.lastname: Nomdedéu
authors.forename: R
authors.initials: R
authors.lastname: Mateu
authors.forename: A
authors.initials: A
authors.lastname: Altès
authors.forename: A
authors.initials: A
authors.lastname: Llorente
authors.forename: C
authors.initials: C
authors.lastname: Rio
authors.forename: C
authors.initials: C
authors.lastname: Estivill
authors.forename: O
authors.initials: O
authors.lastname: López
authors.forename: J
authors.initials: J
authors.lastname: Ubeda
authors.forename: E
authors.initials: E
authors.lastname: Rubiol
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis* / Q000096:biosynthesis / Q000235:genetics; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis* / Q000096:biosynthesis / Q000235:genetics; D015415:Biomarkers; D018826:CD13 Antigens / Q000032:analysis* / Q000096:biosynthesis / Q000235:genetics; D019070:Cell Lineage; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D007231:Infant, Newborn; D007951:Leukemia, Myeloid / Q000276:immunology* / Q000473:pathology; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000276:immunology* / Q000473:pathology; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000276:immunology* / Q000473:pathology; D000090063:Proto-Oncogene Mas; D019009:Proto-Oncogene Proteins c-kit / Q000032:analysis* / Q000096:biosynthesis / Q000235:genetics; D063268:Sialic Acid Binding Ig-like Lectin 3
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D015415:Biomarkers; C571163:CD33 protein, human; C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D063268:Sialic Acid Binding Ig-like Lectin 3; D019009:Proto-Oncogene Proteins c-kit; D018826:CD13 Antigens