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Weekly Chest CasesCases by Disease Category

Case No : 1365 Date 2023-12-19

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  • Courtesy of Chan Seop Kim, Jin Young Yoo, Sung Jin Kim / Chungbuk National University Hospital
  • Age/Sex 47 / M
  • Chief ComplaintRadiologic abnormality, current smoker
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7
  • Figure 8
  • Figure 9

Diagnosis With Brief Discussion

Diagnosis
Pumonary Langerhans cell histiocytosis
Radiologic Findings
Fig 1. Chest PA shows multiple small nodule in both lungs with CPA sparing.
Fig 2-5. Chest CT scans multiple centrilobular nodules and small air cysts with upper and middle lung predominance. Basal lung was spared.
Fig. 6-9: before 3 months, cystic lesions were not evident.
Fig 10, 11: newly appeared cystic lesions are more evident on side-by-side comparison
Brief Review
Pulmonary Langerhans cell histiocytosis (PLCH) is a diffuse lung disease that usually affects young adult smokers with a peak incidence between the ages of 20 and 40 years. Current consensus is that PLCH represents a myeloid neoplasm with inflammatory properties.
As standard chest radiography has limited value as multiple lesions may be small, high-resolution computed tomography plays a major role in the diagnosis of PLCH. Typical findings of early PLCH are centrilobular nodules, nodules with or without a lacuna, and initially thick-walled cysts of various shapes. As the disease progresses, the cysts become larger and thin-walled and may show bizarre shapes. Lesions have a characteristic distribution, with predominance in the upper and middle parts of the lungs and costophrenic angles are usually spared. In some patients, different degrees of pneumothorax are presents. The types of lung lesions vary with disease duration; in early disease, nodules and cavitated nodules are more dominant while advanced disease is often cystic in appearance.
Definite diagnosis of PLCH requires adequate clinical presentation and the identification of Langerhans cells in biopsy specimens that exhibit either CD207 (langerin) or CD1a.
Prognosis of PLCH are unpredictable, varies from spontaneous regression and/or stabilization of the disease after smoking cessation to progression of the disease, developing impaired pulmonary function over time.
References
1. Vassallo R, Harari S, Tazi A. Current understanding and management of pulmonary Langerhans cell histiocytosis. Thorax 2017; 72:937
2. Elzbieta Radzikowska. Update on Pulmonary Langerhans Cell Histiocytosis. Front Med (Lausanne). 2020; 7: 582581.
Keywords
LCH,

No. of Applicants : 88

▶ Correct Answer : 66/88,  75.0%
  • - Wakayama Red Cross Hospital , Japan TOMOAKI OTANI
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan SUZUNE TSUKAMOTO
  • - , Japan HIROAKI ARAKAWA
  • - The University of Tokyo Hospital , Japan JUN KANZAWA
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - , Japan KYOKO NAGAI
  • - Chonnam National University Hwasun Hospital , Korea (South) SEONGWOO CHO
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - Oita university , Japan AYUMI KAMEI
  • - Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
  • - , Italy PAOLO BALDASSARI
  • - Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - , Korea (South) JIN YOUNG LEE
  • - Secomedic Hospital , Japan FUMINORI MIYOSHI
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - , Korea (South) KANGHWI LEE
  • - Qatif Central Hospital , Saudi Arabia HANI MAKKY ALSALAM
  • - Osaka University , Japan AKINORI HATA
  • - Ajou University Hospital , Korea (South) HAEIN LEE
  • - Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
  • - Ajou University Hospital , Korea (South) HYUNSEUNG LEE
  • - OITA UNIVERSITY , Japan FUMIKA ISHITOBI
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Ajou University Hospital , Korea (South) MINJI KIM
  • - Inje University Pusan Paik Hospital , Korea (South) JIYEON HAN
  • - Ajou University Hospital , Korea (South) JAYOUNG MOON
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - , Japan SHUNJIRO NOGUCHI
  • - , Korea (South) HEONSEOK LEE
  • - Tokyo Medical and Dental University , Japan MIKIYA FUJII
  • - Mie university , Japan MIYUKO FUJITA
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Mie university , Japan SHIKO OKABE
  • - Kyoto university , Japan HIROKAZU SAWAMURA
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Seoul National University Hospital , Korea (South) SUNGHO HONG
  • - Holy Family Hospital , India RAJESH GOTHI
  • - Others , India PURUSHOTHAMA RAO TUMMALA
  • - , China MING LU
  • - Chonnam National University Hospital , Korea (South) KIM SANG GYUN
  • - Mie University Hospital , Japan KENSUKE DOMAE
  • - , Algeria SAMIR SALAH AIECH
  • - Kyoto University , Japan SHO KOYASU
  • - Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Fukuoka university , Japan KEISUKE SATO
  • - Samsung Medical Center , Korea (South) SEONGUK KIM
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - Sotiria Hospital, Athens , Greece VASILIOS TZILAS
  • - , Japan YUMI MAEHARA
  • - Toyota Kosei Hospital , Japan YUKI HAYASHI
  • - The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
  • - , Korea (South) WONJU HONG
  • - Kyoto university , Japan AKIHIKO SAKATA
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Other , Korea (South) YONG HO JANG
  • - , Korea (South) JIN WOO YOON
  • - , China LI GUO
  • - Juntendo University , Japan YUTAKA IKENOUCHI
▶ Correct Answer as Differential Diagnosis : 6/88,  6.8%
  • - Shiga University of Medical Science , Japan AKITOSHI INOUE
  • - Affilitated Hospital of Jining Medical college , China JIANG SHENG HUA
  • - Nankai medical center , Japan NORITAKA KAMEI
  • - , Japan KENTARO KOTANI
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
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