Aberrant expression of KOR-SA3544 antigen in childhood acute lymphoblastic leukemia predicts TEL-AML1 negativity. The Pediatric Hematology Working Group in the Czech Republic.
The antigen KOR-SA3544 is physiologically expressed exclusively on granulocytes. Aberrant expression of KOR-SA3544 has been invariably found in BCR/ABL-positive acute lymphoblastic leukemia (ALL) and in some BCR/ABL-negative ALL. In an interim analysis of a prospective clinical and cytometric study data of 73 children with newly diagnosed or relapsed ALL with and without TEL/AML1 fusion are presented. KOR-SA3544 expression over 3% was detected in the majority of TEL/AML1-negative patients with newly diagnosed common or preB ALL (19 of 31) and not in TEL/AML1-positive patients (0 of 18, P < 0.0001). The level of expression of KOR-SA3544 was 0.02-90% (median 6.0%) and 0.03-2.4% (median 0.23%) in TEL/AML1-negative and TEL/AML1-positive patients, respectively. All five newly diagnosed patients with DNA index > or =1.16 and <1.6 exhibited high levels of KOR-SA3544 expression. Membrane expression of CD79a was found to correlate with TEL/AML1 negativity, although less significantly than KOR-SA3544 (P = 0.03). Furthermore, our data confirm that TEL/AML1 positivity correlates with non-hyperdiploidy and low presenting age. In conclusion, KOR-SA3544 correlated strongly with TEL/AML1 negativity, it was a better predictor of TEL/AML1 status than other factors tested and was found at high levels in hyperdiploidy. In combination with age, KOR-SA3544 predicted TEL/AML1 status in 86% newly diagnosed preB/cALL patients.
Institute of Immunology, Prague, Czech Republic.
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Hrusák
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Trka
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Zuna
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Housková
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Bartůnková
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Starý
D000367:Age Factors; D000951:Antigens, Neoplasm / Q000096:biosynthesis*; D019677:Artificial Gene Fusion; D002648:Child; D050676:Core Binding Factor Alpha 2 Subunit; D004273:DNA, Neoplasm / Q000032:analysis / Q000235:genetics; D004268:DNA-Binding Proteins / Q000096:biosynthesis / Q000235:genetics*; D006801:Humans; D011003:Ploidies; D016133:Polymerase Chain Reaction; D011233:Precipitin Tests; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics / Q000378:metabolism; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics* / Q000378:metabolism*; D011518:Proto-Oncogene Proteins; D050783:Proto-Oncogene Proteins c-ets; D012097:Repressor Proteins; D014157:Transcription Factors / Q000096:biosynthesis / Q000235:genetics*; D000097764:ETS Translocation Variant 6 Protein
D000951:Antigens, Neoplasm; D050676:Core Binding Factor Alpha 2 Subunit; D004273:DNA, Neoplasm; D004268:DNA-Binding Proteins; D011518:Proto-Oncogene Proteins; D050783:Proto-Oncogene Proteins c-ets; D012097:Repressor Proteins; D014157:Transcription Factors; D000097764:ETS Translocation Variant 6 Protein; C493728:RUNX1 protein, human