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: Of the 34 patients with MI present at the initial LCH diagnosis, 32 were treated with corticosteroids (prednisone) at a dose of 40 mg/m2/day and vinblastine (6 mg/m2/day), sometimes in association to additional drugs. One patient died before any therapy and one patient was treated with steroid alone. If the patients were included in therapeutic LCH II and LCH III trials, VP16 (N = 2) or methotrexate (N = 4) was administered according to the randomization of the trial. The three patients with MI at relapse were each treated at their initial LCH diagnosis with the same doses of prednisone and vinblastine. At relapse, one patient was treated with vinblastine and prednisone, one with 2-chlorodeoxyadenosine (2-CdA) alone, and one received VP16, cytarabine, and cyclosporine. After 6 weeks of treatment, according to the evaluation criteria of the Histiocyte Society, the disease status was considered as non-active in four patients (11%), active disease better in 29 (78%), including one who had received only steroid treatment, active disease—stable in three (8%) and lastly active disease—worse in one patient (2.7%) who died from the disease. The disease activity score decreased for all patients, except those who died from the disease, however it remained above five in six patients, which can be considered a poor response. All treated patients presented with decreased volume of MI; 15 patients had a residual mass after 6 weeks. Even in cases with poor response to the initial therapy, MI was not considered to be life threatening, or even as a therapeutic target in the latter course of therapy. Sixteen patients experienced at least one disease relapse and the 5-year relapse rate was only 45% (95% CI, 26–62%; Fig. 3B). Three of these relapses involved the thymus in patients who did not have MI at initial diagnosis, at 1 or 3 years after the initial diagnosis. Treatment of refractory disease or relapse was managed according to national guidelines, when available. Four patients received 2-CdA and cytarabine as a second-line therapy, and four patients received monotherapy with 2-CdA.
Affiliation: Service D'hémato Oncologie Pédiatrique, CHU de Strasbourg, Strasbourg, France; Service D'hémato Oncologie Pédiatrique, CHU de Bordeaux, Bordeaux, France.