000 Pulmonary Langerhans Cell Histiocytosis

Position of Disease
Upper and mid lung field distribution
Ashley Davidoff MD TheCommonvein.net lungs-0772

Structures Involved

Small Airways
Lung Parenchyma
Interstitial Tissue

Imaging Appearances

  • Upper Lung Predominance:
  • Nodules and Cysts:
    •  cysts are often irregularly shaped and
    • may vary in size. 
  • Centrilobular Nodules:
  • Ground-Glass Opacities:
  • Ground-glass opacities may be observed on CT scans, representing areas of increased lung density. These opacities can be associated with inflammation and infiltration of Langerhans cells.

 Early

    • cellular interstitial infiltrates of
    • Langerhans’ cells,
      • Staining with antibodies against CD1a antigen on the cell surface
    • lymphocytes,
    • macrophages,
      eosinophils, plasma cells, and fibroblasts
  • Mid stages
    • infiltrates enlarge to form nodules centered on small airways (peribronchial)
    • often stellate in configuration

Early Phase

  • Figure 9. Nodular infiltrates with a stellate border extending into the surrounding interstitium in a patient with PLCH. (Courtesy of Professor A. Pesci, University of Parma.)

  • next Phase
    • Cavitation within nodules due to
    • either an airway remnant or
    • de novo cavitation due to an enlarging
      inflammatory infiltrate

 

Figure 2. Pulmonary Langerhans cell histiocytosis. Multiple nodules and cysts seen on computed tomography (CT) imaging ( A ) with histology showing cellular nodules ( B ), some with central cavities ( B , *) containing diagnostic Langerhans cell aggregates highlighted by positive immunohistochemical staining for CD1a ( C , brown stain ) typical of the early cellular stage of the disease. Coronal CT image from another patient showing multiple bizarre-shaped cysts in an upper-zone–predominant distribution, with sparing of the costophrenic angles representative of later-stage disease ( D ). Histologic features typical of later disease stages include cystic spaces ( E ) associated with paucicellular stellate fibrosis ( E , arrow ). Accumulations of smoking-related pigmented macrophages ( F , arrowhead ) are frequently seen in the surrounding parenchyma. Original magnifications: 2 3 ( E ); 4 3 ( B ); 40 3 ( C ); 100 3 ( F ).
Gupta et al Diffuse Cystic Lung Disease: Part I  American Journal of Respiratory and Critical Care Medicine 191(12)  April 2015

Cavitation

Cyst Formation Initially Thick Walled

 

Then Thin Walled Cysts

CT UPPER LUNG ZONE PROMINENCE OF SMALL THIN WALLED CYSTS
LANGERHANS HISTIOCYTOSIS
Ashley Davidoff MD

 

53-year-old female with nicotine dependence presents with dyspnea and cough
CXR (PA and Lateral) shows bilateral and extensive reticular nodular changes slightly more prominent in the upper lung zones


CXR (PA and Lateral) shows bilateral and extensive reticular nodular changes slightly more prominent in the upper lung zones
Ashley Davidoff MD

CT scan shows multiple relatively thick-walled cysts predominantly in the upper lobes. The cysts are round and air filled large and are between 5mm-8mm

CT  CORONAL VIEW – UPPER ZONES MORE AFFECTED THAN LOWER LUNG ZONES – LANGERHANS HISTIOCYTOSIS
53-year-old female with nicotine dependence presents with dyspnea and cough
CXR (PA and Lateral) shows bilateral and extensive reticular nodular changes slightly more prominent in the upper lung zones
CT scan from 16 months prior showed multiple relatively thick-walled cysts predominantly in the upper lobes. The cysts are round and air filled large and are between 5mm-8mm
CT scan 9 months later shows improvement in the thickened walls of the cysts but maintenance of diffuse cystic changes predominantly in the upper lobes
A CT scan done 2 years later shows no significant change in the diffuse bilateral cystic changes, dominant in the upper lobes and consistent with Langerhans histiocytosis
Ashley Davidoff MD
CT  CORONAL VIEW – UPPER ZONES MORE AFFECTED THAN LOWER LUNG ZONES – LANGERHANS HISTIOCYTOSIS
53-year-old female with nicotine dependence presents with dyspnea and cough
CXR (PA and Lateral) shows bilateral and extensive reticular nodular changes slightly more prominent in the upper lung zones
CT scan from 16 months prior showed multiple relatively thick-walled cysts predominantly in the upper lobes. The cysts are round and air filled large and are between 5mm-8mm
CT scan 9 months later shows improvement in the thickened walls of the cysts but maintenance of diffuse cystic changes predominantly in the upper lobes
A CT scan done 2 years later shows no significant change in the diffuse bilateral cystic changes, dominant in the upper lobes and consistent with Langerhans histiocytosis
Ashley Davidoff MD
CT AXIAL VIEW  – APEX SHOWS MOST SEVERE DISEASE
LANGERHANS HISTIOCYTOSIS
53-year-old female with nicotine dependence presents with dyspnea and cough
CXR (PA and Lateral) shows bilateral and extensive reticular nodular changes slightly more prominent in the upper lung zones
CT scan from 16 months prior showed multiple relatively thick-walled cysts predominantly in the upper lobes. The cysts are round and air filled large and are between 5mm-8mm
CT scan 9 months later shows improvement in the thickened walls of the cysts but maintenance of diffuse cystic changes predominantly in the upper lobes
A CT scan done 2 years later shows no significant change in the diffuse bilateral cystic changes, dominant in the upper lobes and consistent with Langerhans histiocytosis
Ashley Davidoff MD
CT HILAT REGIONS – LANGERHANS HISTIOCYTOSIS
Ashley Davidoff MD
CT BASAL REGIONS – LANGERHANS HISTIOCYTOSIS
Ashley Davidoff MD

A Second Case

28 -year-old male with only minimal reported nicotine dependence presents with dyspnea and cough
CXR shows no acute cardiopulmonary disease with mild interstitial prominence

LANGERHANS HISTIOCYTOSIS
CXR shows no acute cardiopulmonary disease with mild interstitial prominence

CT scan shows multiple small cysts predominantly in the upper lobes. The cysts are round and air filled large and are between 5mm-8mm
These findings are consistent with Langerhans histiocytosis though the relatively minor smoking history was inconsistent with the diagnosis and thus the person was subjected to multicentric wedge biopsies.

CT UPPER LUNG ZONE PROMINENCE OF SMALL THIN WALLED CYSTS
LANGERHANS HISTIOCYTOSIS
Ashley Davidoff MD
CT UPPER LUNG ZONE PROMINENCE OF SMALL THIN WALLED CYSTS
LANGERHANS HISTIOCYTOSIS
Ashley Davidoff MD
CT LOWER LUNG ZONE DISEASE LESS PROMINENT
LANGERHANS HISTIOCYTOSIS
Ashley Davidoff MD

 

Pathology revealed numerous stellate nodules which suggested “burnout pulmonary Langerhans cell histiocytosis though immunostains including Langerin, CD1a and S100 were negative thus failing to confirm the diagnosis Other findings of histiocytic aggregates raised the possibility of an inhalational hypersensitivity pneumonitis.
Ashley Davidoff MD

Leatherwood D et al Pulmonary Langerhans Cell Histiocytosis RadioGraphicsVol. 27, No. 1