Histiocytosis X and right pulmonaryallograft - Thoracic Imaging

Title: Thoracic Imaging - Histiocytosis X and right pulmonaryallograft
Diagnosis: Histiocytosis X and right pulmonaryallograft
Author: Martins Martina
Comment: Howarth Nigel
Hospital: HUG
Department: Département de Radiologie
State: Terminée
Date: 21.03.2003
: 3379
KeyWords: Histiocytosis X, allograft; interstitium
Anatomy: Thorax
Chapter: Interstitial pneumopathy
ACR: 60.66

Clinical Presentation: Male smoker with a unilateral rightallograft.
Comment: The generic term of histiocytosis X, or Langerhansíscell histiocytosis,
includes three diseases, differentiated by theage of onset, localisation and prognosis:
Letterer-Siwe disease,Handís disease and eosinophilic granuloma. They are
oftenassociated with a pneumothorax, which can be bilateral, recurrent,due to the rupture
of subpleural cysts. In the initial stages,chest X-rays show ground glass opacities, or non
alveolaropacities, perihilar, shaped like butterfly wings (similar to anacute pulmonary
edema). These findings are caused by aninflammatory reaction and diffuse alveolar filling
with Langerhansícells, macrophages, eosinophils, histiocytes. This stage is rarelyseen,
unlike the nodular stage when initial lesions have collectedinto nodular clumps (of various
sizes), which also includeinflammatory cells. This condition is mostly seen in young
malesmokers. Its etiology is unknown. No specific effective treatmenthas yet been found.
Giving up smoking is mandatory. The course isunpredictable and includes: improvement
with a regression of theradiological signs (50% of cases), stabilisation, or
progressiontowards obstructive respiratory insufficiency. In some cases, alung transplant
may be necessary.
Description: The chest X-ray shows surgical clips at the rightpulmonary apex and
a metal endoprosthesis in the right mainbronchus. In addition, a poorly defined, spiculated
opacity is seenat the left apex with a diffuse lucency in the left upperhemithorax. Normal
aspect of the right lung (pulmonary graft) onthe CT images. Stellar opacity in the left apex
with multiple,centimetric lung cysts, separated by thin walls, some confluentwith bizarre
shapes. This "geographic" pattern is typical ofadvanced histiocytosis X. Pathology:
Section 1 ñ polymorphousinflammatory infiltrate. Section 2 ñ positive for protein
S100antibodies Electron microscope ñ Langerhansí cells. Note thepresence of indented
nuclei (arrow).

Order: 0
Dislocation: 0
Polytrauma: 0
Open: 0
Pathologic: 0
Operation: 00.00.00
Graft: 0

References: Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH.Clinical
outcomes of pulmonary Langerhans' cell histiocytosis inadults. N Engl J Med
2002;346:484-90. Vassallo R, Ryu JH, Colby TV,Hartman T, Limper AH. Pulmonary
Langerhans' cell histiocytosis. NEngl J Med 2000; 342:1969-78. Arens R, Kramer SS.
Images inclinical medicine. Pulmonary Langerhans' cell granulomatosis(histiocytosis X). N
Engl J Med 2000;342:1959.

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