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Immune markers of disease severity and treatment response in pediatric acquired aplastic anemia
Author(s) -
Sutton Kathryn S.,
Shereck Evan B.,
Nemecek Eneida R.,
Kurre Peter
Publication year - 2013
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.24247
Subject(s) - medicine , immune system , immunosuppression , immunophenotyping , aplastic anemia , immunology , anemia , lymphocyte , disease , gastroenterology , sickle cell anemia , flow cytometry , bone marrow
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. Pediatr Blood Cancer 2013; 60: 455–460. © 2012 Wiley Periodicals, Inc.