Successful treatment of Langerhans cell histiocytosis with 2‐chlorodeoxyadenosine

Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder which frequently involves the lungs of affected adults. Recent evidence suggests it is a clonal neoplastic disorder. Prognosis in this disease is variable, but in its multisystem form or when associated with progressive respiratory dysfunction, prognosis is poor. Recent case reports and a phase II trial of the antimonocyte drug 2-chlorodeoxy-adenosine (2CDA) have described success in treating LCH. We used 2CDA to treat a young Australian man with LCH involving lungs and bone. A complete symptomatic remission was achieved with no evidence of recurrence some 5 years after completion of chemotherapy.

Adult pulmonary Langerhans' cell histiocytosis

“…There is no evidence that these agents are beneficial in adults with isolated PLCH. Although 2-chlorodeoxyadenosine has been shown to be useful in cases of refractory systemic LCH, an improvement in lung function in patients with PLCH has not been clearly demonstrated [70][71][72].…”

Respiratory failure in interstitial lung disease

“…Interferon-␥ has been used to manage steroid-resistant delayed pulmonary toxicity syndrome (interstitial pneumonia and pulmonary fibrosis after high-dose carmustine) [79]. Pulmonary and multisystem Langerhans cell histiocytosis is treated with steroids, 2-chlorodeoxy-adenosine [80], and lung transplantation.…”

Une toux chronique chez une patiente infectée par le VIH

“…La vinblastine, traitement essentiel des formes systémiques, n'a pas fait preuve d'efficacité dans l'HLP. Quelques cas d'amélioration de la fonction respiratoire sous cladribine (2CDA) ont été décrits [3,4] [5,7]. Le rôle potentiel du VIH dans le développement de l'HLP est difficile à déterminer.…”