SESSION TITLE: Interstitial Lung Disease Cases
SESSION TYPE: Case Reports
PRESENTED ON: Saturday, March 22, 2014 at 09:00 AM - 10:00 AM
INTRODUCTION: The course of adult Pulmonary Langerhans cell Histiocytosis (PLCH) is variable and unpredictable . Conventional therapy is mainly based on smoking cessation. If the patient is severely symptomatic or the disease shows radiological or physiological deterioration after a short observation period, a course of steroids should be given. Chemotherapeutic drugs,like 2-chlorodeoxyadenosine, may be effective in childhood forms of disseminated LCH, but there is far too little experience with these agents in adult pulmonary LCH patient .
CASE PRESENTATION: A 45 year-old man, heavy smoker, was admitted in the hospital with recurrent right spontaneous pneumothoraces. A lung biopsy confirmed pulmonary PLCH, and pleurodesis was carried out. Six months after, the patient had a new left pneumothorax, with air leaking. Throughout a ten month period, our patient had several relapses of pneumothoraces in both lungs. Bilateral thoracotomy with pleurodesis was carried out due to the persistence of leakage. After these procedures, he continued with a poor performance despite a course of sterorids and after given up smoking. Echocardiography did not detected pulmonary hypertension. Intravenous Cladribine was given every four weeks up to three cycles, the only side effect observed was thrombocytopenia on the second cycle. Nine months after the therapy the oxygen saturation at rest and arterial blood gases showed a sustained improvement. The patient reported a clear symptomatic improvement.
DISCUSSION: Our patient had an advanced pulmonary disease despite a course of steroids. He was in such a severe condition that we made a proposal for lung transplantation but he was rejected. After being absolutely sure that the patient had given up smoking, we considered a trial of cladribine. The role of immunosuppressive therapy in the care of patients with adverse prognostic, indicators is not clear, since such treatment has not been objectively demonstrated to improve lung function or reduce long-term mortality. Thus cytotoxic drugs should only be given to rapidly progressive cases of isolated pulmonary disease not responding to corticosteroids. .
CONCLUSIONS: There are several case reports and small case series about adult patients who responded favorably to 2-CDA despite of having multysistemic progressive disease. Few of the patients reported in these case series had pulmonary involvement, but none had isolated PLCH. Still, the effect of 2-CDA to treat isolated PLCH remain unclear. . Our findings suggest that the therapy with cladribine may be a sensible option when patients with progressive PLCH have not responded to other conventional therapeutic measures.
Reference #1: Vasallo R, Ryu JH, Schroeder Dr, et al. Clinical outcome of Pulmonary Langerhans cell Histiocytosis in adults. N Engl J Med 2002;346:484-490.
Reference #2: Michelle R. Aerni, Robert Vasallo. 2-Chlorodeoxyadenosine treatment in pulmonary lesions of LCH. Chest 2006;130:301s-302s.
DISCLOSURE: The following authors have nothing to disclose: Juan Cruz Rueda, Julián Ceballos Gutiérrez, Ana Fulgencio, Jose Manuel Gonzalez de Vega, Ana Dolores Romero Ortiz
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