title: Expression of coagulation factor XIII subunit A in acute promyelocytic leukemia.
abstract: Leukemic cells often express markers, which are not characteristic of their particular cell lineage. In this study, we identified the "A" subunit of coagulation factor XIII (FXIII-A) in leukemic promyelocytes in de novo AML M3 cases. The cytoplasmic presence of factor XIII-A has previously been shown only in platelets/megakaryocytes and monocytes/macrophages. Furthermore, more recently we described the presence of FXIII-A in leukemic lymphoblasts. We studied 14 patients with this rare type of acute leukemia in a period of 4 years and investigated their bone marrow samples by 3-color flow cytometry upon diagnosis, mainly focusing on FXIII-A expression of leukemic cells. We detected FXIII-A also by ELISA, Western-blot, and confocal laser scanning microscopy. This was a homogenous group of AML M3 patients with translocation t(15;17)(q22;q21) detected by fluorescence in situ hybridization (FISH). In 10 out of 14 samples, FXIII-A was detectable by flow cytometry and was coexpressed with markers characteristic for leukemic promyleocytes (CD45dim/CD13+/CD33+/CD117+/cyMPO+ and HLA-DR-/CD34-/CD14-/CD15-). Staining for the markers GPIIb and GPIX were negative, and FXIII-A was identified in the cytoplasm of the cells by confocal microscopy in a relatively high quantity, as measured by ELISA. By Western blot analysis we could identify FXIII-A in the native 82 kDa form and in cleaved forms corresponding to cleavage products observed when purified FXIII-A was treated by human neutrophil elastase. This novel expression site of FXIII-A in AML M3 can be considered as a leukemia associated immunophenotype and may have pathophysiological significance.
authors.affiliation: Department of Laboratory Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary.
authors.forename: Ágnes
authors.initials: Á
authors.lastname: Simon
authors.forename: Zsuzsa
authors.initials: Z
authors.lastname: Bagoly
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Hevessy
authors.forename: László
authors.initials: L
authors.lastname: Csáthy
authors.forename: Éva
authors.initials: É
authors.lastname: Katona
authors.forename: György
authors.initials: G
authors.lastname: Vereb
authors.forename: Anikó
authors.initials: A
authors.lastname: Ujfalusi
authors.forename: László
authors.initials: L
authors.lastname: Szerafin
authors.forename: László
authors.initials: L
authors.lastname: Muszbek
authors.forename: János
authors.initials: J
authors.lastname: Kappelmayer
mesh_terms: D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000378:metabolism; D015153:Blotting, Western; D004797:Enzyme-Linked Immunosorbent Assay; D026122:Factor XIIIa / Q000378:metabolism*; D005260:Female; D005434:Flow Cytometry; D042381:Granulocyte Precursor Cells / Q000378:metabolism; D006801:Humans; D016130:Immunophenotyping; D015473:Leukemia, Promyelocytic, Acute / Q000378:metabolism* / Q000473:pathology; D008297:Male; D018613:Microscopy, Confocal; D008875:Middle Aged; D021122:Protein Subunits / Q000378:metabolism*
chemical_list: D015415:Biomarkers; D021122:Protein Subunits; D026122:Factor XIIIa