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Vol. 61. Issue 2.
Pages 124-130 (1 August 2004)
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Vol. 61. Issue 2.
Pages 124-130 (1 August 2004)
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Enfermedad pulmonar obstructiva tras trasplante alogénico de progenitores hematopoyéticos en niños
Obstructive lung disease after allogenic stem cell transplantation in children
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R. Alonso Riofríoa,
Corresponding author
r.alonso@saludalia.com

Correspondencia: Hospital Infantil Universitario Niño Jesús. Avda. Menéndez Pelayo, 65. 28009 Madrid. España
, J.R. Villa Asensia, A. Sequeiros Gonzáleza, M.A. Díaz Pérezb, M. González Vicentb, L. Madero Lópezb
a Secciones de Neumología Pediátrica. Hospital Infantil Universitario Niño Jesús. Madrid. España
b Secciones de Hematología y Oncología Pediátrica. Hospital Infantil Universitario Niño Jesús. Madrid. España
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Introducción

La bronquiolitis obliterante está considerada como una complicación pulmonar grave que aparece a partir de los 3 meses del trasplante de médula ósea

Objetivo

Determinar la incidencia y evolución clínica de la enfermedad pulmonar obstructiva (EPO) en una población de niños que han recibido trasplante alogénico de células madre hematopoyéticas

Pacientes y métodos

Se ha revisado una muestra de 110 pacientes que recibieron trasplante alogénico entre enero de 1992 y junio de 2002. En los 77 pacientes que sobrevivieron más de 100 días después del trasplante se ha calculado la incidencia de EPO. El diagnóstico de EPO se basó en la presencia de hallazgos clínicos sin evidencia de infección respiratoria, pruebas de función pulmonar (volumen espiratorio forzado en el primer segundo/capacidad vital forzada [FEV1/FVC] < 80% y FEV1 < 80% del valor teórico) y datos de la tomografía computarizada (TC)

Resultados

Presentaron EPO, 8 pacientes (10,4 %) con una mediana del tiempo de inicio de 184 días después del trasplante (rango, 100–1.735). Todos los pacientes con EPO presentaron síntomas respiratorios. La obstrucción de la vía respiratoria fue diagnosticada en el primer año tras el trasplante en 6 de los 8 pacientes. Todos presentaban enfermedad de injerto contra huésped (EICH) crónica (p < 0,01), la incidencia de EPO entre los 23 afectados de EICH crónica fue del 34,8 %. Dos (25 %) de los 8 pacientes presentaron mejoría completa de la EPO con la intensificación del tratamiento de la EICH crónica con inmunosupresores. La FEV1 disminuyó rápidamente en 3 (37,5 %) pacientes, los cuales fallecieron por fallo respiratorio. Presentaron mejoría parcial 2 pacientes (25%), aunque persistía afectación de la función pulmonar. Un paciente (12,5 %) presenta obstrucción grave estable

Conclusiones

La EPO tras el trasplante presenta un momento de inicio y una progresión variable, está estrechamente relacionada con la EICH crónica y su incidencia depende del número de pacientes con este proceso

Palabras clave:
Bronquiolitis obliterante
Trasplante de células madre hematopoyéticas
Obstrucción de la vía aérea
Enfermedad injerto contra huésped
Niños
Introduction

Bronchiolitis obliterans is recognized as a life-threatening pulmonary complication that can develop 3 months after bone marrow transplantation

Objective

To determine the incidence and clinical progression of obstructive lung disease (OLD) in a population of children who had undergone allogenic hematopoietic stem cell transplantation (HSCT)

Patients and methods

We examined a sequential sample of 110 patients who received allogeneic HSCT between January 1992 and June 2002. The incidence of OLD in the 77 children who survived for more than 100 days after transplantation was analyzed. The diagnosis of OLD was based on clinical findings with no evidence of infection, pulmonary function test (FEV1/FVC less than 80 % and FEV1 less than 80 % of predicted value) and computed tomography scan

Results

Eight patients (10.4 %) developed OLD at a median time of onset of 184 days after allogenic HSCT (range: 100–1735 days). All patients with OLD had respiratory symptoms. In six out of eight patients airflow obstruction was diagnosed within 1 year of transplantation. All patients showed chronic graft-versus-host disease (GVHD) (p < 0.01). The incidence of OLD in the 23 patients with chronic GVHD was 34.8 %. Two patients (25 %) had a complete response to intensified treatment of chronic GVHD with immunosuppressant therapy. FEV1 declined rapidly in three patients (37.5 %) who died of respiratory failure. Two patients (25 %) had partial reversal but pulmonary function continued below normal values. In one patient (12.5 %) severe obstructive disease was stable

Conclusions

The time of onset and form of progression of OLD after HSCT may vary. OLD is strongly associated with chronic GVHD and its incidence depends on the number of patients with chronic GVHD

Key words:
Bronchiolitis obliterans
Hematopoietic stem cell transplant
Airflow obstruction
Graft-versus-host disease
Children
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Copyright © 2004. Asociación Española de Pediatría
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