Thymus and mediastinal node involvement in childhood Langerhans cell histiocytosis: long-term follow-up from the French national cohort.
Bottom Line: Thirteen cases (35%) were diagnosed because of MI-related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10).The overall 5-year survival was 87.1%, and immunodeficiency was not detected as a sequel.MI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications.
Affiliation: Service D'hémato Oncologie Pédiatrique, CHU de Strasbourg, Strasbourg, France; Service D'hémato Oncologie Pédiatrique, CHU de Bordeaux, Bordeaux, France.Show MeSH
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Mentions: At diagnosis, six patients had lymphopenia with deficiency of CD4 and CD8 T lymphocytes, which was profound in two cases among the 14 patients assessable for these criteria. Figure 1 depicts the lymphocyte status of patient UPN 1506627, both at diagnosis and during therapy; this patient's iconography is reported in Figure 2A,B. One child had hypogammaglobulinemia. In the other patients, the immunological control was normal or not checked.
Affiliation: Service D'hémato Oncologie Pédiatrique, CHU de Strasbourg, Strasbourg, France; Service D'hémato Oncologie Pédiatrique, CHU de Bordeaux, Bordeaux, France.