Email this article Autoimmune diseases in children with acquired aplastic anemia
- Authors: Goronkova O.V.1, Kalinina I.I.1, Salimova T.Y.1, Suntsova E.V.1, Petrova U.N.1, Baydildina D.D.1, Мaschan M.A.2, Novichkova G.A.2, Мaschan A.A.2
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Affiliations:
- Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
- Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Department of Hematology, Oncology and Radiotherapy Russian National Research Medical University named after N.I. Pirogov
- Issue: Vol 17, No 1 (2018)
- Pages: 23-28
- Section: Статьи
- Submitted: 09.08.2018
- Published: 09.02.2018
- URL: https://hemoncim.com/jour/article/view/23
- DOI: https://doi.org/10.24287/1726-1708-2018-17-1-23-28
- ID: 23
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Abstract
The association of aplastic anemia (AA) with other autoimmune diseases (AID) is rarely described and so far not systematically evaluated. We have analysed the literature data and present our own experience. We assessed the incidence and the outcome of concomitant AID in a retrospective, single-center study of 370 patients with AA treated between 1996 and 2014 with either immunosuppression (296) or hematopoietic stem cell transplantation (74) and a median follow-up time of 7 years (0.5–18). Results: Clinically manifest AID were observed in 10 out of 370 (2.7%) patients. Age at diagnosis of AA was similar in patients without AID compared to patients with AID (median, 10,9 versus 10 years; p > 0.05). In 3 patients where the diagnosis of AID was done before AA therapy, response to antithymocyte globulin was good for AA (2 out of 3) but not for AID (0 out of 3). In 7 patients in which AID occurred after first-line therapy, the median time to the AID was 6 months (range 2 months–13.4 years). Response to antithymocyte globulin was good for AA (7 out of 7), but treatment of AID was successful in 43% (3 out of 7) patients. Conclusions: The association of AA with AID in children is very rare. Most cases of AA in association with AID benefitted from IST, but IST was not influence to outcome of concomitant AID.
About the authors
O. V. Goronkova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
Author for correspondence.
Email: goronkova@yandex.ru
ORCID iD: 0000-0001-8571-5395
MD, Department of hematopoiesis depressions, myeloid leukemias, rare and inherited diseases
Russia 117997, Moscow, Samory Mashela st., 1
+7 (495) 287-6570, ext. 5527
I. I. Kalinina
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0002-0813-5626
Russian Federation
T. Yu. Salimova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0002-2958-1705
Russian Federation
E. V. Suntsova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0001-8404-1800
Russian Federation
U. N. Petrova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0002-1258-8281
Russian Federation
D. D. Baydildina
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyRussian Federation
M. A. Мaschan
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyDepartment of Hematology, Oncology and Radiotherapy Russian National Research Medical University named after N.I. Pirogov
Russian Federation
G. A. Novichkova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyDepartment of Hematology, Oncology and Radiotherapy Russian National Research Medical University named after N.I. Pirogov
ORCID iD: 0000-0002-2322-5734
Russian Federation
A. A. Мaschan
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyDepartment of Hematology, Oncology and Radiotherapy Russian National Research Medical University named after N.I. Pirogov
ORCID iD: 0000-0002-0016-6698
Russian Federation
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