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2200-01-01
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- IFF - Artigos de Periódicos [1300]
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LYMPHOPROLIFERATIVE DISEASE IN PATIENTS WITH WISKOTT-ALDRICH SYNDROME: ANALYSIS OF THE FRENCH REGISTRY OF PRIMARY IMMUNODEFICIENCIES
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Abstract in Portuguese
Através de um estudo retrospectivo no registro nacional do CEREDIH, o Centro Nacional de Referência da França para imunodeficiência primária (consulte a seção Métodos no Repositório Online deste artigo em www.jacionline.org para obter mais detalhes), identificamos 13 pacientes com WAS / XLT que tiveram LPD antes do transplante alogênico de células-tronco (alloSCT) entre os 189 pacientes com SAF e 46 pacientes com XLT tratados entre 1988 e outubro de 2017. Considerando que pacientes com SAF / XLT estavam em risco antes de serem submetidos ao alloSCT, a taxa bruta e a taxa de incidência de LPD em pacientes não transplantados foram 8,4% e 511 por 1 × 105 pacientes-ano (IC 95%, 272–873 por 1 × 105 pacientes-ano), respectivamente, o que é maior do que na população geral (7,5 por 1 × 105 pacientes-ano). anos [IC95% 7,1-7,8]; P <0,001). Taxas brutas (4,3% [IC95%, 0,5% a 14%] para a coorte XLT e 5,8% [IC95%, 2,9% a 10,2%] para a coorte WAS) e taxas de incidência (334 [IC95%, 40 –1200] e 531 por 1 × 105 pacientes-ano [IC95%, 265–943], respectivamente) de LPD foram semelhantes nos pacientes com XLT e naqueles com SAF. A incidência cumulativa de LPD foi de 1,9% aos 10 anos e 9,9% aos 30 anos, como mostra a análise de risco competitiva. Após os 40 anos de idade, o risco de LPD persiste em pacientes não transplantados com EVA / XLT, pois três casos ocorreram com 43,2, 50,3 e 50,6 anos (Figura 1, A). A mediana da idade no diagnóstico de WAS / XLT foi semelhante em toda a coorte e no subgrupo de pacientes que desenvolveram LPD (1 ano; intervalo interquartil, 0-3 anos).
Abstract
Through a retrospective study within the national registry of CEREDIH, the French National Reference Center for primary immune deficiency, we identified 13 patients with WAS/XLT who had LPD before allogeneic stem cell transplantation (alloSCT) among the 189 patients with WAS and 46 patients with XLT treated between 1988 and October 2017. Considering that patients with WAS/XLT were at risk before undergoing alloSCT, the crude rate and incidence ratio of LPD in untransplanted patients were 8.4% and 511 per 1 × 105 patient-years (95% CI, 272–873 per 1 × 105 patient-years), respectively, which is greater than in the general population (7.5 per 1 × 105 patient-years [95%CI 7.1–7.8]; P < .001). Crude rates (4.3% [95% CI, 0.5% to 14%] for the XLT cohort and 5.8% [95% CI, 2.9% to 10.2%] for the WAS cohort) and incidence ratios (334 [95% CI, 40–1200] and 531 per 1 × 105 patient-years [95% CI, 265–943], respectively) of LPD were similar in patients with XLT and those with WAS. The cumulative incidence of LPD was 1.9% at age 10 years and 9.9% at age 30 years, as shown by competing risk analysis. After age 40 years, the risk of LPD persists in untransplanted patients with WAS/XLT as 3 cases occurred at age 43.2, 50.3, and 50.6 years (Fig 1, A). The median age at diagnosis of WAS/XLT was similar in the entire cohort and in the subgroup of patients who developed LPD (1 year; interquartile range, 0–3 years).
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