Fatal Bronchiolitis Obliterans with Very Severe Subcutaneous Emphysema and Pneumomediastinum after Unrelated BMT / 대한소아혈액종양학회지

ABSTRACT

Bronchiolitis obliterans (BO) is a particularly severe complication that occurs in 10% to 15 % of the patients with extensive graft-versus-host disease (GVHD) and is often refractory to treatment. We report herewith a child with chronic GVHD and BO after unrelated BMT. A 8 year-old girl with Ph ALL (Philadelphia-chromosome positive acute lymphoblastic leukemia) underwent an unrelated BMT in the first complete remission. Engraftment was uneventful. Acute GVHD of the skin developed, and was treated with methylprednisolone. No evidence of GVHD flare was documented, and immunosuppression was tapered off by 7 months posttransplant. On 9 months posttransplant, chronic GVHD involving skin, liver, mouth, eyes, gastrointestinal tract, and lungs developed. Despite conservative managements, the patient developed pneumomediastinum and subcutaneous emphysema secondary to severe BO. Her condition continued to deteriorate, and she died of respiratory failure 10 months after transplant. Further studies are required to identify risk factors and to develop newer methods of effective treatment for this rare complication.