Expression of the Tn antigen on erythroid cells from a patient with Tn syndrome

Kamitani, Yoko; Kajii, Eiji; Suda, Toshio; Ikemoto, Shigenori

doi:10.1007/bf01883318

Cited by 3 publications

(2 citation statements)

References 23 publications

(24 reference statements)

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“…The correlation with hematologic disorders is not surprising since Tn syndrome is caused by somatic mutations affecting a hematopoietic cell giving rise to a clonal population of Tn antigen expressing RBCs 17 . In vitro studies indicate that the Tn RBCs may have a growth advantage over normal RBCs, 18 leading to preferential expansion. Incidentally, though the overall percentage of Tn RBC can vary between individuals, they can constitute >90% of red cells in some individuals 3…”

Section: Discussionmentioning

confidence: 99%

A case of Tn polyagglutination discovered by an ABO blood group discrepancy

et al. 2022

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Background: Tn syndrome is an acquired form of polyagglutination arising from somatic mutations of hematopoietic stem cells. Tn red blood cells (RBCs) are agglutinable by naturally occurring anti-Tn antibodies in most adult sera. Current ABO typing reagents are monoclonal and do not detect polyagglutination on forward typing. However, herein we describe a case of Tn activation that was suspected due to cross-reactivity with a monoclonal anti-A reagent. Study Design and Methods: A 63-year-old man with myeloproliferative neoplasm, who historically typed as group O, demonstrated unexpected mixed field reactivity with anti-A reagent using a gel-based method. However, manual tube testing was consistent with the patient's historical group O type.Results: Lectin testing demonstrated reactivity with Salvia sclarea and Glycine soja, but not Arachis hypogea. The patient's RBCs produced positive crossmatches with healthy donor sera, but reactivity was eliminated by ficin pretreatment of the RBCs. Ficin treatment also resolved typing discrepancies on gel-based typing. No reactivity was noted using cord blood sera, and N antigen expression was diminished upon phenotyping. Tn activation was confirmed by detection of a novel 4-nucleotide deletion (c.395-398del) in exon 3 of C1GALT1C1 resulting in a truncated glycosyltransferase. Conclusion: This case of acquired Tn polyagglutination due to a novel mutation was first suspected from an ABO phenotyping discrepancy. It highlights the crossreactivity of anti-A reagent with Tn antigen when tested on a common gel-based method. Furthermore, the case demonstrates that review of patient history and test information can clarify discrepancies and guide resolution.

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Section: Discussionmentioning

confidence: 99%

A case of Tn polyagglutination discovered by an ABO blood group discrepancy

et al. 2022

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“…Previous results from our group by flow cytometric analysis showed the mixed-field polyagglutination in this patient, and its Tn RBCs were scored, of which 65.9 and 45.7% were The zeta potentials of peak I and peak II were -13 .32 mV and -4.73 mV, respectively. labeled by polyclonal anti-Tn antibody and lectin V. villosa respectively, which recognizes only the Tn antigen, (Kamitani et al 1992). In this D.E.L.S.…”

Section: Discussionmentioning

confidence: 99%

The Electrokinetic Behavior of Red Blood Cells from a Patient with Tn Syndrome by Doppler Electrophoretic Light Scattering Analysis.

Omi

Kajii

Ikemoto

1994

Tohoku J. Exp. Med.

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Tn-syndrome

Berger

1999

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Diseas

117

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The idiopathic Tn-syndrome, formerly called 'permanent mixed-field polyagglutinability', is a rare hematological disorder characterized by the expression of the Tn-antigen on all blood cell lineages. The immunodominant epitope of the Tn-antigen is terminal alpha-N-acetylgalactosamine, O-glycosidically linked to protein. Normally this residue is 3'-substituted by 5-galactose thereby forming the core 1 structure known as the Thomsen-Friedenreich (TF) antigen (Galbeta1 ==> 3GalNAcalpha1 ==> Thr/Ser). The cause of the exposure of the Tn-antigen appears to be due to the silencing of the gene expression of beta1,3galactosyltransferase, since treatment of deficient Tn(+) lymphocyte T clones with 5'azacytidine or Na butyrate leads to reexpression of enzyme activity and the sialylated TF-antigen. The Tn-syndrome is acquired and permanent and affects both sexes at any age. Its origin is unknown. Pluripotent stem cells are affected since all lineages are involved but each one to a variable extent. Therefore, normal cells co-exist with Tn-transformed cells. Clinically, patients suffering from the Tn-syndrome appear healthy. Laboratory findings usually reveal moderate thrombocyto- and leukopenia and some signs of hemolytic anemia not warranting any treatment.

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Expression of the Tn antigen on erythroid cells from a patient with Tn syndrome

Cited by 3 publications

References 23 publications

A case of Tn polyagglutination discovered by an ABO blood group discrepancy

A case of Tn polyagglutination discovered by an ABO blood group discrepancy

The Electrokinetic Behavior of Red Blood Cells from a Patient with Tn Syndrome by Doppler Electrophoretic Light Scattering Analysis.

Tn-syndrome

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