Miyashita Tatsuyuki, Yamazaki Susumu, Ohta Hiromitsu, Nakamura Hidetoshi, Nagata Makoto
Department of Respiratory Medicine Saitama Medical University Saitama Japan.
Department of Respiratory Medicine, Saitama Medical Center Jichi Medical University Saitama Japan.
Respirol Case Rep. 2020 Nov 26;9(1):e00697. doi: 10.1002/rcr2.697. eCollection 2021 Jan.
A 65-year-old man presented to our hospital with complaint of acute dyspnoea. He smoked 30 cigarettes per day from age 20 to 52 years. Immunocytochemical findings revealed 6.3% of positive CD1a cells in the cell fraction of bronchoalveolar lavage, thus suggesting a diagnosis of pulmonary Langerhans cell histiocytosis (PLCH), after nine years since the first suspicion of PLCH. Furthermore, he was diagnosed with secondary pulmonary hypertension (PH) caused by progressed PLCH by right heart catheterization. At 59 years of age, he was diagnosed with panhypopituitarism, and persistent hormone replacement therapy was subsequently started by an endocrinologist. After the initiation of oxygen therapy and treatment with a combination of sildenafil and warfarin, an estimated pulmonary artery systolic pressure reduced 97.9 to 64.0 mmHg. We believed this is a rare case of PLCH with irreversible central nervous system (CNS) disorder in whom severe PH developed due to a long-term burden of PLCH in a middle-aged male.
一名65岁男性因急性呼吸困难前来我院就诊。他从20岁至52岁期间每天吸烟30支。免疫细胞化学检查结果显示，支气管肺泡灌洗细胞成分中CD1a阳性细胞占6.3%，这表明在首次怀疑肺朗格汉斯细胞组织细胞增多症（PLCH）九年后确诊为PLCH。此外，经右心导管检查，他被诊断为由进展性PLCH引起的继发性肺动脉高压（PH）。59岁时，他被诊断为全垂体功能减退症，随后内分泌科医生开始对其进行持续的激素替代治疗。在开始氧疗并联合使用西地那非和华法林治疗后，估计肺动脉收缩压从97.9 mmHg降至64.0 mmHg。我们认为这是一例罕见的PLCH病例，该中年男性因长期PLCH负担而出现严重PH，并伴有不可逆的中枢神经系统（CNS）疾病。
