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Th activation in congenital hypoplastic anemia.

J H Herman, R S Shirey, B Smith

    Transfusion
    |May 1, 1987
    PubMed
    Summary

    Persistent T-helper (Th) cell activation was found in most children with Fanconi's anemia or Diamond-Blackfan syndrome. This Th activation may serve as a red cell developmental marker in congenital hypoplastic anemias.

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    Area of Science:

    • Hematology
    • Immunology
    • Pediatrics

    Background:

    • Fanconi's anemia and Diamond-Blackfan syndrome are congenital hypoplastic anemias.
    • T-helper (Th) cell activation is a marker of immune response.
    • The role of Th activation in congenital anemias is not well understood.

    Purpose of the Study:

    • To investigate the presence and significance of Th activation in children with Fanconi's anemia or Diamond-Blackfan syndrome.
    • To determine if Th activation is a specific marker for these conditions or a broader red cell developmental marker.

    Main Methods:

    • Flow cytometry was used to detect Th activation in patients and control groups.
    • Analysis included children with Fanconi's anemia, Diamond-Blackfan syndrome, other bone marrow dysfunctions, healthy children, blood donors, and cord blood specimens.
    • One patient was re-evaluated after bone marrow transplantation.

    Main Results:

    • Persistent Th activation was identified in 71.4% of children with Fanconi's anemia or Diamond-Blackfan syndrome.
    • Th reactivity resolved in one patient post-bone marrow transplantation.
    • Low incidence of Th activation was observed in family members, other bone marrow dysfunction patients, healthy children, blood donors, and patients with hemolytic/hypoplastic conditions.
    • Th reactivity was detected in 13.5% of cord blood specimens.

    Conclusions:

    • Th activation may be a red cell developmental marker.
    • This marker appears to be present in congenital hypoplastic anemias like Fanconi's anemia and Diamond-Blackfan syndrome.
    • Th activation is also expressed on newborn red cells.

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