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Pulmonary Langerhans Cell Histiocytosis-Cladribine Treatment
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A1526 - Pulmonary Langerhans Cell Histiocytosis-Cladribine Treatment
Author Block: E. Radzikowska, E. Wiatr, K. Blasinska-Przerwa, K. Roszkowski-Sliz; National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland.
Purpose: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder, mainly young adult smokers, caused by proliferation of CD1a-positive myeloid derived dendritic cells forming granulomas within lung parenchyma. It is resulted with cystic lung destruction and may be the cause of the respiratory insufficiency. The course of the disease is unpredictable. In some patients only smoking cessation can induce regression of the disease but in others progression in spite of the chemotherapy is noticed. Cladribine has been proved as effective treatment of multi-system Langerhans cell histiocytosis and isolated PLCH, however the number of cases is still small.
Patients and method: This retrospective analysis included 12 patients (7 females and 5 males; aged mean 40.08 ± 6.7; range 19-60 years) with pulmonary Langerhans cell histiocytosis treated with cladribine in our Department in years 2010 to 2017. Eight patients had multi system and 4 isolated pulmonary LCH. Patients received 2 to 6(mean 5.1) courses of cladribine, as a single agent in a dose of 0.15 mg/kg per day/ iv. for 5 consecutive days at monthly intervals.
Results: Treatment resulted in improvement or stabilisation of pulmonary function parameters in 5(36%) and 7(64%) patients respectively. Two patients with bone lesions, one with abdominal changes and one woman with infiltration in vertebra and in periaortic space experienced partial regression and disease was not active. Treatment related toxicities were: upper respiratory tract infections grade 2 in 7(64%) patients, and grade 3 in 2(18%), leukopenia grade 1 in 3(25%), grade 2 in 4 (33%), grade 3 in 1(9%) patients, lymphopenia grade 1 in 3(25%), grade 2 in 7(64%), and grade 3 in 2(18%) patients, thrombocytopenia grade 1 in 2(17%), grade 2 in 1(9%), grade 4 in one (9 %) patient, anaemia grade 4 and 2 in one (9%) patient respectively. There was no case of LCH progression. The mean follow-up period was 111.5 ± 59.22 months. During the time of observation one patient suddenly died with unknown cause, other one developed chronic myelogenic leukaemia. No reactivation of the disease was noticed.
Conclusion: Cladribine as a single agent is an effective therapy in adult patients with progressive PLCH but was mainly resulted in stabilization of pulmonary function. Myelosuppression, and pulmonary infections were the most important adverse events.
Author Block: E. Radzikowska, E. Wiatr, K. Blasinska-Przerwa, K. Roszkowski-Sliz; National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland.
Purpose: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder, mainly young adult smokers, caused by proliferation of CD1a-positive myeloid derived dendritic cells forming granulomas within lung parenchyma. It is resulted with cystic lung destruction and may be the cause of the respiratory insufficiency. The course of the disease is unpredictable. In some patients only smoking cessation can induce regression of the disease but in others progression in spite of the chemotherapy is noticed. Cladribine has been proved as effective treatment of multi-system Langerhans cell histiocytosis and isolated PLCH, however the number of cases is still small.
Patients and method: This retrospective analysis included 12 patients (7 females and 5 males; aged mean 40.08 ± 6.7; range 19-60 years) with pulmonary Langerhans cell histiocytosis treated with cladribine in our Department in years 2010 to 2017. Eight patients had multi system and 4 isolated pulmonary LCH. Patients received 2 to 6(mean 5.1) courses of cladribine, as a single agent in a dose of 0.15 mg/kg per day/ iv. for 5 consecutive days at monthly intervals.
Results: Treatment resulted in improvement or stabilisation of pulmonary function parameters in 5(36%) and 7(64%) patients respectively. Two patients with bone lesions, one with abdominal changes and one woman with infiltration in vertebra and in periaortic space experienced partial regression and disease was not active. Treatment related toxicities were: upper respiratory tract infections grade 2 in 7(64%) patients, and grade 3 in 2(18%), leukopenia grade 1 in 3(25%), grade 2 in 4 (33%), grade 3 in 1(9%) patients, lymphopenia grade 1 in 3(25%), grade 2 in 7(64%), and grade 3 in 2(18%) patients, thrombocytopenia grade 1 in 2(17%), grade 2 in 1(9%), grade 4 in one (9 %) patient, anaemia grade 4 and 2 in one (9%) patient respectively. There was no case of LCH progression. The mean follow-up period was 111.5 ± 59.22 months. During the time of observation one patient suddenly died with unknown cause, other one developed chronic myelogenic leukaemia. No reactivation of the disease was noticed.
Conclusion: Cladribine as a single agent is an effective therapy in adult patients with progressive PLCH but was mainly resulted in stabilization of pulmonary function. Myelosuppression, and pulmonary infections were the most important adverse events.
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