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Weekly Chest CasesArchive of Old Cases

Case No : 587 Date 2009-01-26

  • Courtesy of Hyun Ju Lee, MD. / Seoul National University Hospital, Korea.
  • Age/Sex 37 / F
  • Chief ComplaintChest PA abnormality / Known calcified mass suggesting granuloma in RUL
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Pulmonary Langerhans Cell Histiocytosis
Radiologic Findings
There are diffuse thin-walled air cysts in both lung fields. Upper lobes are more affected than lower lobes.
Sellar MRI shows thickening and enhancement of pituitary stalk.
Findings are consistent with Langerhans histiocyosis.
Brief Review
Pulmonary Langerhans cell histiocytosis (LCH; histiocytosis X, eosinophilic granuloma) belongs to the spectrum of the diseases that are characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells (LCs).
It is a rare disease most commonly affecting adult cigarette smokers.
Men and women are almost equally affected, although a slightly higher proportion of women has recently been reported, most frequently between the ages of 20 to 40 years.
Pulmonary LCH may present as an isolated form of LCH or it can present simultaneously with other organ involvement.
Chest plain radiograph shows bilateral, symmetrical reticular, and micronodular opacities in upper and middle lung zones, with relative sparing of costophrenic angles. Sometimes mediastinal lymphadenopathy and pneumothorax are demonstrated.
HRCT scan is of utmost importance in the diagnosis of pulmonary LCH. The most characteristic finding is nodules and cysts in the upper lung zones with sparing of the lung bases. Cysts are of varying size, sometimes of bizarreshape.
In the advanced stage, the distortion of the parenchyma is seen, and cysts can fuse and give radiological appearance almost indistinguishable from emphysema.
References
1. Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH. Pulmonary Langerhans?cell histiocytosis.N Engl J Med. 2000;342:1969?8.
2. Youkeles LH, Grizzanti JN, Liao Z, Chang CJ,Rosenstreich DL. Decreased tobacco-glycoprotein-induced lymphocyte proliferation in vitro in pulmonary eosinophilic granuloma. Am J Respir Crit Care Med. 1995;151:145?0.
3. Willman CL, Busque L, Griffith BB, Favara BE, McClain KL, Duncan MH, et al. Langerhans? cell histiocytosis (histiocytosis X) a clonal proliferative disease. N Engl J Med. 1994;331:154?0.
4. Dacic S, Trusky C, Bakker A, Finkelstein SD,Yousem SA. Genotipic analysis of pulmonary Langerhans cell histiocytosis. Hum Pathol. 2003; 34:1345?.
Keywords
Lung, Interstitial lung disease, LCH, smoking related ILD,

No. of Applicants : 117

▶ Correct Answer : 30/117,  25.6%
  • - PingTung Christian Hospital ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - yashodha hospital , India pravin mahadevappa
  • - Clinic 1 , Russian Federation Lepikhina Darya
  • - Osaka University , Japan Osamu Honda
  • - Gaziantep Kad Hastallarve Dom Hastanesi , Turkey Tay Bakar
  • - john h stroger hsp , United States dheeraj reddy gopireddy
  • - second hospital xian jiaotong university , China li ming
  • - POPOVO HOSPITAL , Bulgaria VLADISLAV RUSINOV
  • - Hanmaeum Hospital , Korea (South) Kwon Hyoung Kim
  • - hospital Sao Paulo , Brazil israel missrie
  • - Shinchon Severance Hospital , Korea (South) Ho-Joon Lee
  • - CHRU Lille , France Benjamin Damarey
  • - CH Sud Rnion , Reunion jean-baptiste Noel
  • - chest hospital , Egypt assaad assaad
  • - UZ GASTHUISBERG , Belgium Johan COOLEN
  • - Mackay Memorial Hospital , Taiwan Shen-Yeh Shen
  • - wonju christial hospital , Korea (South) Daekeon Lim
  • - Academic hospital Free university of Brussel , Belgium nezar gadalla
  • - Asan Medical Center , Korea (South) Seong Yun Kim
  • - Trakya Univercity School of Medicine , Turkey Armagan Sarac
  • - Chung-Ang University Medical center , Korea (South) Changkeun Sung
  • - Trakya University School of Medicine , Turkey Umut Ugur
  • - Armed Forces Chun-Cheon Hospital , Korea (South) Chaehun Lim
  • - Mediacl IT Consulting , Japan Kazumasa Nishimura
  • - Soonchunhyang University Cheonan Hospital , Korea (South) Park Sang Hyun
  • - Ruby Hall Clinic , India sudarshan rawat
  • - ASL BOLOGNA - MAGGIORE HOSPITAL- ITALY , Italy MARCELLINO BURZI
  • - ROHINI Scans. Ambattur , India Umapathi Mahesh
  • - PITTWATER RADIOLOGY , Australia Julie Arora
  • - PITTWATER RADIOLOGY , Australia Saurabh Khandelwal
▶ Correct Answer as Differential Diagnosis : 48/117,  41.0%
  • - Shiga University of Medical Science , Japan Norihisa Nitta
  • - kims,narkatpally , India k bhaskar
  • - McGill University Health Centre , Canada Amr Ajlan
  • - Father Muller Medical College , Mangalore , India nabil mahmood
  • - Louisiana State University - Shreveport , United States Carlos Previgliano
  • - Chungnam National University hospital , Korea (South) Kyoung Jin Oh
  • - KMC,Manipal , India satish maddukuri
  • - The Armed Forces HamPyeong Hospital , Korea (South) Bae Geun Oh
  • - HOSPITAL SOTIRIA , Greece AIKATERINI DELAVERI
  • - Soonchunhyang university Bucheon hospital , Korea (South) Minhee Lee
  • - Konkuk university hospital , Korea (South) Jeong Geun Yi
  • - Soonchunhyang university hospital, Bucheon , Korea (South) Hyelin Kim
  • - CHRU lille , France manuel toledano
  • - Lilavati Hospital & Research Centre , India Mahavir Swami
  • - Trakya Univercity School of Medicine, , Turkey BELMA KIYMAZASLAN
  • - National Taiwan University Hospital , Taiwan Chin-Chung Shu
  • - Das Allgemeine Krankenhaus , Germany Davis Chiramel
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - KONARK DIAGNOSTIC CENTRE , India AHAMAD MUKARRAB
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Dongguk University International Hospital , Korea (South) Ah-young Kim
  • - Jaseng medical clinic , Korea (South) Ki Taek Hong
  • - BOLLINENI HOSPITAL NELLORE , India jignesh dubal
  • - Ondokuz Mayis University , Turkey Cetin Celenk
  • - Seoul National University Hospital , Korea (South) Kwang Nam Jin
  • - Samsung Medical Center , Korea (South) Eun Kim
  • - IRSA , France jean - luc BIGOT
  • - CHR ANNECY , France Olivier Segall
  • - EKH-Berlin , Germany Holger Hartmann
  • - Private Clinic , Bahamas Trupti Dabholkar
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Dana Farber cancer Institute , United States jyothi jagannathan
  • - Yashoda Super Speciality hospital , India Ramesh Pandey
  • - Homs National Hospital , Syria Rami Abou Zalaf
  • - Yonsei University college of medicine Severance Hospital , Korea (South) Hua Sun Kim
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - Kashan University of Medical Sciences, kashan , Iran Ebrahim Razi
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - NASA SCANS , India RAKESH BHATIA
  • - Priv><br> <input type=hidden name=id17691 value= , Belgium Emmanuel Agneessens
  • - E-DA Hospital / I-Shou University , Taiwan Yu-Feng Wei
▶ Semi-Correct Answer : 35/117,  29.9%
  • - NONE , India rekha gupta
  • - seoul national university hospital , Korea (South) Ijin Joo
  • - AIIMS , India tariq matin
  • - batra hospital new delhi , India shiva rastogi
  • - Ruby Hall Clinic , India john joseph
  • - Uzunkopru Hospital, Edirne , Turkey Derya Karabulut
  • - Samsung Medical Center , Korea (South) Dae Kun Oh
  • - Kasturba Medical College Manipal , India Paresh Desai
  • - Goodmorning hospital , Korea (South) Kwang Keun Lee
  • - DCA, , India Rajesh Gothi
  • - Retd. Prof Thoracic Surgery B.J.MEDIDCAL COLLEGE Punr India , India Bankim Amin
  • - Mon Hospital , France sebastien LARIVE
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - Cheongshim international medical center , Korea (South) Hae Jum Yang
  • - govt royapettah hospital , India gopinathan kathirvelu
  • - EKH-Berlin , Germany Michael Weber
  • - Cairo University , Egypt Mamdouh Eissa
  • - Shinsegye Rad Clinic, Daegu , Korea (South) Gi Beom Kim
  • - All India Institute of Medical Sciences , India puneet kochar
  • - Command Hospital (Eastern Command) , India Vivek Sharma
  • - al watany hosp , Syria mohamad jader
  • - IRCCS S.Luca Hosp. , Italy filippo casolo
  • - NOUR EL-ISLAM RADIOLOGY CENTER , Egypt elsayed mousa
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - KASTURBA MEDICAL COLLEGE , India NAVEEN KULKARNI
  • - Apollo Gleneagles hosp , India salim parvez
  • - homs national hospital , Syria basem hasen
  • - Dongguk University International Hospital , Korea (South) Hee Seok Choi
  • - Changhua Christian Hospital , Taiwan Chia-Fu Tsai
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - Hopital universitaire Lausanne , Switzerland abed kourhani
  • - Trakya universitesi , Turkey Garip KUTLUTK
  • - chp st martin , France Mariotte benoit
  • - Institute of Chest Diseases,Calicut , India sijith raghavan
  • - Vitalife Clinics , Pune , India. , India Rahul Deshmukh
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