Abstract
To the Editors: Abt–Letterer–Siwe syndrome, which is a disseminated form of Langerhans' cell histiocytosis (LCH), was diagnosed in the patient at the age of 6 months, affecting the inguinal lymph nodes and skin, and recurred at the age of 21 yrs, affecting lungs and arcus pubis 1. After two chemotherapy sessions, including vinblastine, etoposide and corticosteroids, complete remission was reached. At the age of 25 yrs the first high-altitude oedema occurred during a ski vacation at 2,400 m followed by the second episode 3 yrs later at an altitude of 2,800 m, which was confirmed by chest radiograph. After the second episode of high-altitude pulmonary oedema (HAPE) the patient was referred to the Division of Sports Medicine at the University Hospital of Heidelberg, Heidelberg, Germany, where he was assessed as described before 2. Estimated systolic pulmonary artery pressure ( P pa) was normal at rest (25 mmHg), increased to 50 mmHg after 30 min (oxygen saturation 74%) and to 60 mmHg (saturation 65%) after 90 min of exposure to normobaric hypoxia (inspiratory oxygen fraction ( F I,O2) of 12%). Stress Doppler echocadiography revealed systolic P pa values up to 95 mmHg during normoxic exercise of 125 W. The patient had a normal systemic blood pressure at rest and during exercise and no signs of left ventricular dysfunction. Maximum exercise capacity was 1.95 W·kg−1 (70% of normal), maximum oxygen consumption ( V ′O2) was 2.6 L·min−1 (80% of normal), body plethysmography showed patterns of obstructive and restrictive lung disease (airway resistance 158% of normal, forced expiratory volume in 1 s/forced vital capacity (FEV1/FVC) ratio was 60% (70% predicted), vital capacity was 70%, residual volume 166%, total lung capacity 95%) and a reduced diffusion capacity for carbon monoxide (48% pred). Despite shortness of breath, the …
Highlights
To the Editors: Abt–Letterer–Siwe syndrome, which is a disseminated form of Langerhans’ cell histiocytosis (LCH), was diagnosed in the patient at the age of 6 months, affecting the inguinal lymph nodes and skin, and recurred at the age of 21 yrs, affecting lungs and arcus pubis [1]
At the age of 35 yrs, exercise performance had further decreased while lung function and gas exchange were largely unchanged compared with 7 yrs previously
This treatment was tolerated well and improved exercise capacity, right ventricular pump function and Ppa within a follow-up time of 18 months. This is the first report of recurrent episodes of high-altitude pulmonary oedema (HAPE) at moderate altitude in a patient with Abt–Letterer–Siwe syndrome who developed a symptomatic chronic pulmonary hypertension a few years later
Summary
To the Editors: Abt–Letterer–Siwe syndrome, which is a disseminated form of Langerhans’ cell histiocytosis (LCH), was diagnosed in the patient at the age of 6 months, affecting the inguinal lymph nodes and skin, and recurred at the age of 21 yrs, affecting lungs and arcus pubis [1]. Thoracoscopic biopsy of the lung revealed no signs of a relapse of LCH, but residual multifocal scars of the lung tissue and diffuse histological changes of the pulmonary arteries with medial and subintimal wall thickening obstructing the vessels. This is the first report of recurrent episodes of HAPE at moderate altitude in a patient with Abt–Letterer–Siwe syndrome who developed a symptomatic chronic pulmonary hypertension a few years later.
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