Contributions
Abstract: EP1117
Type: E-Poster Presentation
Session title: Myeloproliferative neoplasms - Clinical
Background
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis that typically affect many organs including lung and pleura. However, studies concerning pulmonary involvement are limited in ECD patients.
Aims
The aim of the study is to describe the clinical manifestations, radiological findings and treatment outcomes of ECD pulmonary involvement, explore its prognostic value, as well as compare the pulmonary involvement of our ECD cohort with the adult LCH patients in our center.
Methods
We performed a retrospective study of the clinical features, radiological characteristics and treatment outcomes of 54 consecutive ECD patients at Peking Union Medical College Hospital between January 2013 and October 2020. Data of adult Langerhans cell histiocytosis (LCH) patients with pulmonary involvement diagnosed from January 1990 to November 2019 in our center were also retrospectively analyzed. Clinical and radiological characteristics between these two cohorts were compared.
Results
The median age of diagnosis was 48 years (range 9-66 years) for the 54 ECD patients. 49 patients (91%) had pulmonary involvement, while 14 (25.9%) had respiratory symptoms. Chest computed tomography (CT) scans revealed lung involvement in 49 (91%) patients and pleura involvement in 34 (63%). 33 (61%) patients had interstitial lung disease (ILD) with varying degrees of interlobular septal thickening (57.4%), micronodules (55.6%), and ground-glass opacities (48.1%). Diffusion dysfunction was the most frequent pattern in pulmonary function test and the median diffusion lung capacity for carbon monoxide was 65.1% predicted (37.5-111% predicted). Patients with pulmonary involvement between ECD and LCH show significant differences in smoking status (10.3% VS 59.3%, P<0.001), respiratory symptoms (28.6% VS 57.5%, P=0.001) like cough, pneumothorax (0 VS 23.9%, P<0.001), and the radiological findings, including cysts (P<0.001), opacities (P<0.001), and pleural thickening (P<0.001). With the median follow-up duration of 24 months (range, 1–84 months), the estimated 3-year OS of this cohort were 90.2%. Patients with ILD tended to have worse PFS than those with no ILD (P = 0.29).
Conclusion
Pulmonary involvement is usually asymptomatic and therefore underestimated among ECD patients. Typical findings on CT scans include interlobular septal thickening, micronodules, ground-glass opacities, and pleural thickening. The pulmonary involvement of ECD is quite different from LCH in clinical and radiological manifestations.
Keyword(s): Myeloid malignancies, Pulmonary
Abstract: EP1117
Type: E-Poster Presentation
Session title: Myeloproliferative neoplasms - Clinical
Background
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis that typically affect many organs including lung and pleura. However, studies concerning pulmonary involvement are limited in ECD patients.
Aims
The aim of the study is to describe the clinical manifestations, radiological findings and treatment outcomes of ECD pulmonary involvement, explore its prognostic value, as well as compare the pulmonary involvement of our ECD cohort with the adult LCH patients in our center.
Methods
We performed a retrospective study of the clinical features, radiological characteristics and treatment outcomes of 54 consecutive ECD patients at Peking Union Medical College Hospital between January 2013 and October 2020. Data of adult Langerhans cell histiocytosis (LCH) patients with pulmonary involvement diagnosed from January 1990 to November 2019 in our center were also retrospectively analyzed. Clinical and radiological characteristics between these two cohorts were compared.
Results
The median age of diagnosis was 48 years (range 9-66 years) for the 54 ECD patients. 49 patients (91%) had pulmonary involvement, while 14 (25.9%) had respiratory symptoms. Chest computed tomography (CT) scans revealed lung involvement in 49 (91%) patients and pleura involvement in 34 (63%). 33 (61%) patients had interstitial lung disease (ILD) with varying degrees of interlobular septal thickening (57.4%), micronodules (55.6%), and ground-glass opacities (48.1%). Diffusion dysfunction was the most frequent pattern in pulmonary function test and the median diffusion lung capacity for carbon monoxide was 65.1% predicted (37.5-111% predicted). Patients with pulmonary involvement between ECD and LCH show significant differences in smoking status (10.3% VS 59.3%, P<0.001), respiratory symptoms (28.6% VS 57.5%, P=0.001) like cough, pneumothorax (0 VS 23.9%, P<0.001), and the radiological findings, including cysts (P<0.001), opacities (P<0.001), and pleural thickening (P<0.001). With the median follow-up duration of 24 months (range, 1–84 months), the estimated 3-year OS of this cohort were 90.2%. Patients with ILD tended to have worse PFS than those with no ILD (P = 0.29).
Conclusion
Pulmonary involvement is usually asymptomatic and therefore underestimated among ECD patients. Typical findings on CT scans include interlobular septal thickening, micronodules, ground-glass opacities, and pleural thickening. The pulmonary involvement of ECD is quite different from LCH in clinical and radiological manifestations.
Keyword(s): Myeloid malignancies, Pulmonary