Outcome of autoimmune cytopenia after hematopoietic cell transplantation in primary immunodeficiency.
J Allergy Clin Immunol
; 146(2): 406-416, 2020 08.
Article
em En
| MEDLINE
| ID: mdl-32442647
ABSTRACT
BACKGROUND:
Post hematopoietic cell transplantation (HCT) autoimmune cytopenia (AIC) is a potentially life-threatening complication, but studies focusing on large cohorts of patients transplanted for primary immunodeficiency are lacking.OBJECTIVES:
This study sought to determine the incidence, risk factors, and outcomes of post-HCT AIC and B-lymphocyte function following rituximab.METHODS:
We retrospectively studied 502 children with primary immunodeficiency who were transplanted at our center between 1987 and 2018.RESULTS:
Thirty-six patients (9%) developed post-HCT AIC, with a median onset of 6.5 months post-HCT. On univariate analysis, pre-HCT AIC, mismatched donor, alemtuzumab, anti-thymocyte antiglobulin, and acute and chronic graft versus host disease were significantly associated with post-HCT AIC. After multivariate analysis, alemtuzumab (subdistribution hazard ratio, 9.0; 95% CI, 1.50-54.0; P = .02) was independently associated with post-HCT AIC. Corticosteroid and high-dose intravenous immunoglobulin achieved remission in 50% (n = 18), additional rituximab led to remission in 25% (n = 9), and the remaining 25% were treated with a combination of various modalities including sirolimus (n = 5), bortezomib (n = 3), mycophenolate mofetil (n = 2), splenectomy (n = 2), and second HCT (n = 3). The mortality of post-HCT AIC reduced from 25% (4 of 16) prior to 2011 to 5% (1 of 20) after 2011. The median follow-up of 5.8 years (range, 0.4 to 29.1 years) showed that 26 of 30 survivors (87%) were in complete remission, and 4 were in remission with ongoing sirolimus and low-dose steroids. Of the 17 who received rituximab, 7 had B-lymphocyte recovery, 5 had persistent low B-lymphocyte count and remained on intravenous immunoglobulin replacement, 2 had second HCT, and 3 died.CONCLUSIONS:
The frequency of post HCT AIC in our cohort was 9%, and the most significant risk factors for its occurrence were the presence of graft versus host disease and the use of alemtuzumab.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Complicações Pós-Operatórias
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Linfócitos B
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Transplante de Células-Tronco Hematopoéticas
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Sirolimo
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Anticorpos Monoclonais Humanizados
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Rituximab
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Doenças da Imunodeficiência Primária
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Doença Enxerto-Hospedeiro
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Fatores Imunológicos
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article