Immune markers of disease severity and treatment response in pediatric acquired aplastic anemia.
Submitted by Eneida R Nemecek on
Title | Immune markers of disease severity and treatment response in pediatric acquired aplastic anemia. |
Publication Type | Journal Article |
Year of Publication | 2013 |
Authors | Sutton, KS, Shereck, EB, Nemecek, ER, Kurre, P |
Journal | Pediatr Blood Cancer |
Volume | 60 |
Issue | 3 |
Pagination | 455-60 |
Date Published | 2013 Mar |
ISSN | 1545-5017 |
Keywords | Adolescent, Anemia, Aplastic, Antineoplastic Agents, Child, Child, Preschool, Female, Flow Cytometry, Hemoglobinuria, Paroxysmal, Humans, Immunophenotyping, Infant, Killer Cells, Natural, Male, Retrospective Studies, T-Lymphocytes |
Abstract | BACKGROUND: To investigate the immune status among pediatric patients with aplastic anemia (AA) and explore PNH-status, T-regulatory and NK-cell frequency as potential markers of clinical response. METHODS: Data were retrospectively analyzed from twenty-six patients diagnosed with AA. PNH populations, T- and NK-subsets were determined via flow cytometry. RESULTS: At diagnosis, 9/23 patients with severe AA (SAA) versus 1/3 with moderate AA (MAA) were PNH(pos) . Among PNH(pos) patients treated with ATG based immunosuppression, 2/6 had a complete response (CR), while 4/6 had a partial response (PR), similarly 2/6 PNH(neg) patients had a CR and 4/6 had a PR. Lymphocyte subset immunophenotyping revealed that T-regulatory cells represented 7.2% of total lymphocytes at diagnosis. Their frequency varied with disease severity (5.5% for SAA and 14.1% for MAA) and response (8.9% for CR and 1.5% for PR), generally increasing following therapy with IST (14.6%). The NK cell frequency was not substantially different based on disease severity or response. CONCLUSIONS: Neither PNH cell populations, nor NK cell frequency corresponded with disease severity or response. T-regulatory cell frequency, although not statistically significant given the small sample size, corresponded with both severity and response, indicating potential utility as a prognostic tool. |
DOI | 10.1002/pbc.24247 |
Alternate Journal | Pediatr Blood Cancer |
PubMed ID | 22811079 |