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Pulmonary Langerhans cell histiocytosis

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Pulmonary Langerhans cell histiocytosis (PLCH) may be seen as part of widespread involvement in patients with disseminated Langerhans cell histiocytosis or more frequently as a distinct entity in young adult smokers. This article focuses on the latter.

Epidemiology

Pulmonary Langerhans cell histiocytosis is usually identified in young adults (20-40 years of age). A history of current or previous cigarette smoking is identified in up to 95% of cases ^1,4. It is a rare disorder with no well-established gender predilection, which appears to be more common in ~~Caucasian~~White populations ⁴.

Associations

Haematopoietic neoplasms:

acute lymphoblastic leukaemia (ALL) ^11,12
acute myeloid leukaemia (AML) ¹⁰

Clinical presentation

Presentation is usually with dyspnoea or non-productive cough. Other symptoms include constitutional symptoms (fatigue and weight loss), pleuritic chest pain, or spontaneous pneumothorax ^1,4. Up to a quarter of patients are asymptomatic.

Pathology

Langerhans cells proliferate in the bronchiolar and bronchial epithelium, forming granulomas. It is postulated that as these cellular granulomas evolve, peripheral fibrosis forms resulting in traction on the central bronchiole which becomes cyst-like ³. This explains the presumed evolution from a nodule, through cavitating nodule and thick-walled cysts, to the ~~'stable' thin~~"stable" thin-walled cysts ^3,4. An immune-mediated mechanism has been postulated, although an inciting agent has not been isolated ⁴. This proliferation is accompanied by inflammation and granuloma formation. Electron microscopy may reveal characteristic Birbeck granules ^1,2.

More recent evidence suggests that pulmonary Langerhans cell histiocytosis represents a myeloid neoplasm with inflammatory properties ⁹.

Radiographic features

Pulmonary Langerhans cell histiocytosis has variable appearance depending on the stage of the disease, ranging from small peribronchiolar nodular opacities to multiple irregularly-shaped cysts. There is a mid and upper zone predilection ^1,3,4.

Plain radiograph

The earliest change is a diffuse bilateral symmetrical reticulonodular pattern with a predilection for the mid and upper zones. The ill-defined nodules range from 1-10 mm in size. Later, cyst formation may be seen or may mimic a honeycomb appearance due to a summation of air-filled cysts. Cysts can be identified in only 1-15% of cases ¹, and range from 1-3 cm in diameter. There is a preservation of lung volumes or even hyperinflation ^1,3,4. Reduced lung volumes are uncommon and only seen in end-stage fibrotic cases ⁴. Lymph node enlargement visible on chest x~~-rays~~-ray is rare ⁴.

CT

As is usually the case, CT and especially HRCT is superior to plain chest radiography in identifying both the reticulonodular opacities and cysts ^1,3,4. Distribution is the key in differentiating pulmonary Langerhans cell histiocytosis from other cystic lung diseases with a predilection for the mid and upper zones and regional sparing of the costophrenic recesses, anterior right middle lobe and lingula of the left upper lobe ^1,3,4.

nodules
- more pronounced early in the disease
- may range in number from a few to innumerable
- 1-10 mm in diameter (typically 1-5 mm ⁴)
- centrilobular distribution -: may also be peribronchial or peribronchiolar
- usually have irregular margins
- may be cavitary nodules with thick walls, later becoming cysts
- surrounding lung parenchyma appears normal
cysts
- more pronounced later in the disease
- usually less than 10 mm in diameter
- may measure up to 2-3 ~~centimetres~~cm in size
- the extreme bases may be preserved
- usually thin-walled, but on occasion may be up to a few millimetres thick
- confluence of 2 or more cysts results in bizarre shapes
  - bilobed
  - cloverleaf
  - branching
  - internal septations

Other common findings include ^1,3:

ground-glass and/or reticular opacities
- DIP-like change ¹
mosaic attenuation
interlobular septal ~~line~~ thickening
emphysema

In late disease, other findings include:

coalescent cysts
fibrosis
honeycombing

The appearance of new nodules later in the disease (when cystic change is established) indicates disease progression but is a rare finding ³.

Treatment and prognosis

Overall prognosis is generally good with over 50% of patients demonstrating spontaneous resolution or stabilisation even without treatment ³. This is especially the case in patients who stop smoking.

In a minority of patients (~20%) and more frequently in those who continue to smoke, the disease is progressive with deterioration in respiratory function and eventual end-stage pulmonary fibrosis ³.

Treatment may not be required once smoking has ceased. Corticosteroids are frequently used and appear beneficial. In patients with rapidly progressive disease, no proven therapy has been found. In some selected patients lung transplantation may be an option, provided smoking has ceased. Recurrence in the transplanted lung has been described ⁴.

Complications

cyst rupture
- spontaneous pneumothorax: may be the first presentation
- pneumomediastinum
interstitial fibrosis
- pulmonary arterial hypertension and cor pulmonale
- end-stage pulmonary fibrosis and respiratory failure

Differential diagnosis

Differential depends on whether the nodular or cystic change is the dominant feature.

Early in the disease, when nodules are the dominant feature, consider:

granulomatous disease
- granulomatosis with polyangiitis
- sarcoidosis
metastases
miliary tuberculosis

See differential of multiple pulmonary nodules and differential of miliary opacities for more comprehensive lists.

Later in the disease, when cysts are prominent, consider:

~~lymphangiomyomatosis~~lymphangioleiomyomatosis (LAM)
- diffuse distribution
- regular shaped and sized cysts
cystic bronchiectasis from ABPA
- central distribution
- mucous plugging
centrilobular emphysema
- lack of visible cyst wall ^1-3
Pneumocystis jiroveci pneumonia
idiopathic pulmonary fibrosis
- basal and subpleural distribution
- reduced lung volumes
lymphocytic interstitial pneumonitis (LIP)
- smooth-walled simple cysts
- associated with autoimmune disease

-<p><strong>Pulmonary Langerhans cell histiocytosis (PLCH)</strong> may be seen as part of widespread involvement in patients with <a href="/articles/langerhans-cell-histiocytosis">disseminated Langerhans cell histiocytosis</a> or more frequently as a distinct entity in young adult smokers. This article focuses on the latter. </p><h4>Epidemiology</h4><p>Pulmonary Langerhans cell histiocytosis is usually identified in young adults (20-40 years of age). A history of current or previous cigarette smoking is identified in up to 95% of cases <sup>1,4</sup>. It is a rare disorder with no well-established gender predilection, which appears to be more common in Caucasian populations <sup>4</sup>.</p><h5>Associations</h5><p>Haematopoietic neoplasms:</p><ul>
+<p><strong>Pulmonary Langerhans cell histiocytosis (PLCH)</strong> may be seen as part of widespread involvement in patients with <a href="/articles/langerhans-cell-histiocytosis">disseminated Langerhans cell histiocytosis</a> or more frequently as a distinct entity in young adult smokers. This article focuses on the latter. </p><h4>Epidemiology</h4><p>Pulmonary Langerhans cell histiocytosis is usually identified in young adults (20-40 years of age). A history of current or previous cigarette smoking is identified in up to 95% of cases <sup>1,4</sup>. It is a rare disorder with no well-established gender predilection, which appears to be more common in White populations <sup>4</sup>.</p><h5>Associations</h5><p>Haematopoietic neoplasms:</p><ul>
-</ul><h4>Clinical presentation</h4><p>Presentation is usually with dyspnoea or non-productive cough. Other symptoms include constitutional symptoms (fatigue and weight loss), <a href="/articles/pleuritic-pain">pleuritic chest pain</a>, or spontaneous <a href="/articles/pneumothorax">pneumothorax</a> <sup>1,4</sup>. Up to a quarter of patients are asymptomatic.</p><h4>Pathology</h4><p><a href="/articles/langerhans-cells">Langerhans cells</a> proliferate in the bronchiolar and bronchial epithelium, forming <a href="/articles/granuloma">granulomas</a>. It is postulated that as these cellular granulomas evolve, peripheral fibrosis forms resulting in traction on the central bronchiole which becomes cyst-like <sup>3</sup>. This explains the presumed evolution from a nodule, through cavitating nodule and thick-walled cysts, to the 'stable' thin-walled cysts <sup>3,4</sup>. An immune-mediated mechanism has been postulated, although an inciting agent has not been isolated <sup>4</sup>. This proliferation is accompanied by inflammation and granuloma formation. Electron microscopy may reveal characteristic <a href="/articles/birbeck-granules">Birbeck granules</a> <sup>1,2</sup>.</p><p>More recent evidence suggests that pulmonary Langerhans cell histiocytosis represents a myeloid neoplasm with inflammatory properties <sup>9</sup>.</p><h4>Radiographic features</h4><p>Pulmonary Langerhans cell histiocytosis has variable appearance depending on the stage of the disease, ranging from small peribronchiolar nodular opacities to multiple irregularly-shaped cysts. There is a mid and upper zone predilection <sup>1,3,4</sup>. </p><h5>Plain radiograph </h5><p>The earliest change is a diffuse bilateral symmetrical <a href="/articles/reticulonodular-interstitial-pattern">reticulonodular pattern</a> with a predilection for the mid and upper zones. The ill-defined nodules range from 1-10 mm in size. Later, cyst formation may be seen or may mimic a <a href="/articles/honeycombing-lungs">honeycomb</a> appearance due to a summation of air-filled cysts. Cysts can be identified in only 1-15% of cases <sup>1</sup>, and range from 1-3 cm in diameter. There is a preservation of lung volumes or even hyperinflation <sup>1,3,4</sup>. Reduced lung volumes are uncommon and only seen in end-stage fibrotic cases <sup>4</sup>. Lymph node enlargement visible on chest x-rays is rare <sup>4</sup>.</p><h5>CT </h5><p>As is usually the case, CT and especially HRCT is superior to plain chest radiography in identifying both the <a href="/articles/reticulonodular-interstitial-pattern">reticulonodular opacities</a> and cysts <sup>1,3,4</sup>. Distribution is the key in differentiating pulmonary Langerhans cell histiocytosis from other cystic lung diseases with a predilection for the mid and upper zones and regional sparing of the costophrenic recesses, anterior right middle lobe and lingula left upper lobe <sup>1,3,4</sup>. </p><ul>
+</ul><h4>Clinical presentation</h4><p>Presentation is usually with dyspnoea or non-productive cough. Other symptoms include constitutional symptoms (fatigue and weight loss), <a href="/articles/pleuritic-pain">pleuritic chest pain</a>, or spontaneous <a href="/articles/pneumothorax">pneumothorax</a> <sup>1,4</sup>. Up to a quarter of patients are asymptomatic.</p><h4>Pathology</h4><p><a href="/articles/langerhans-cells">Langerhans cells</a> proliferate in the bronchiolar and bronchial epithelium, forming <a href="/articles/granuloma">granulomas</a>. It is postulated that as these cellular granulomas evolve, peripheral fibrosis forms resulting in traction on the central bronchiole which becomes cyst-like <sup>3</sup>. This explains the presumed evolution from a nodule, through cavitating nodule and thick-walled cysts, to the "stable" thin-walled cysts <sup>3,4</sup>. An immune-mediated mechanism has been postulated, although an inciting agent has not been isolated <sup>4</sup>. This proliferation is accompanied by inflammation and granuloma formation. Electron microscopy may reveal characteristic <a href="/articles/birbeck-granules">Birbeck granules</a> <sup>1,2</sup>.</p><p>More recent evidence suggests that pulmonary Langerhans cell histiocytosis represents a myeloid neoplasm with inflammatory properties <sup>9</sup>.</p><h4>Radiographic features</h4><p>Pulmonary Langerhans cell histiocytosis has variable appearance depending on the stage of the disease, ranging from small peribronchiolar nodular opacities to multiple irregularly-shaped cysts. There is a mid and upper zone predilection <sup>1,3,4</sup>. </p><h5>Plain radiograph </h5><p>The earliest change is a diffuse bilateral symmetrical <a href="/articles/reticulonodular-interstitial-pattern">reticulonodular pattern</a> with a predilection for the mid and upper zones. The ill-defined nodules range from 1-10 mm in size. Later, cyst formation may be seen or may mimic a <a href="/articles/honeycombing-lungs">honeycomb</a> appearance due to a summation of air-filled cysts. Cysts can be identified in only 1-15% of cases <sup>1</sup>, and range from 1-3 cm in diameter. There is a preservation of lung volumes or even hyperinflation <sup>1,3,4</sup>. Reduced lung volumes are uncommon and only seen in end-stage fibrotic cases <sup>4</sup>. Lymph node enlargement visible on chest x-ray is rare <sup>4</sup>.</p><h5>CT </h5><p>As is usually the case, CT and especially HRCT is superior to plain chest radiography in identifying both the <a href="/articles/reticulonodular-interstitial-pattern">reticulonodular opacities</a> and cysts <sup>1,3,4</sup>. Distribution is the key in differentiating pulmonary Langerhans cell histiocytosis from other cystic lung diseases with a predilection for the mid and upper zones and regional sparing of the costophrenic recesses, anterior right middle lobe and lingula of the left upper lobe <sup>1,3,4</sup>. </p><ul>
~~-<a href="/articles/centrilobular-region">centrilobular distribution</a> - may also be peribronchial or peribronchiolar</li>~~
+<a href="/articles/centrilobular-region">centrilobular distribution</a>: may also be peribronchial or peribronchiolar</li>
~~-<li>may measure up to 2-3 centimetres in size</li>~~
+<li>may measure up to 2-3 cm in size</li>
~~-<li><a href="/articles/septal-line-thickening">septal line thickening</a></li>~~
+<li><a title="Interlobular septal thickening" href="/articles/interlobular-septal-thickening">interlobular septal thickening</a></li>
-</ul><p>The appearance of new nodules later in the disease (when cystic change is established) indicates disease progression but is a rare finding <sup>3</sup>.</p><h4>Treatment and prognosis</h4><p>Overall prognosis is generally good with over 50% of patients demonstrating spontaneous resolution or stabilisation even without treatment <sup>3</sup>. This is especially the case in patients who stop smoking.</p><p>In a minority of patients (~20%) and more frequently in those who continue to smoke, the disease is progressive with deterioration in respiratory function and eventual end-stage pulmonary fibrosis <sup>3</sup>.</p><p>Treatment may not be required once smoking has ceased. Corticosteroids are frequently used and appear beneficial. In patients with rapidly progressive disease, no proven therapy has been found. In some selected patients lung transplantation may be an option, provided smoking has ceased. Recurrence in the transplanted lung has been described <sup>4</sup>.</p><h5>Complications</h5><ul>
+</ul><p>The appearance of new nodules later in the disease (when cystic change is established) indicates disease progression but is a rare finding <sup>3</sup>.</p><h4>Treatment and prognosis</h4><p>Overall prognosis is generally good with over 50% of patients demonstrating spontaneous resolution or stabilisation even without treatment <sup>3</sup>. This is especially the case in patients who stop smoking.</p><p>In a minority of patients (~20%) and more frequently in those who continue to smoke, the disease is progressive with deterioration in respiratory function and eventual end-stage <a href="/articles/pulmonary-fibrosis">pulmonary fibrosis</a> <sup>3</sup>.</p><p>Treatment may not be required once smoking has ceased. Corticosteroids are frequently used and appear beneficial. In patients with rapidly progressive disease, no proven therapy has been found. In some selected patients lung transplantation may be an option, provided smoking has ceased. Recurrence in the transplanted lung has been described <sup>4</sup>.</p><h5>Complications</h5><ul>
~~-<li>~~
~~-<p>granulomatous disease</p>~~
~~-<ul>~~
+<li>granulomatous disease<ul>
~~-<a href="/articles/lymphangioleiomyomatosis-1">lymphangiomyomatosis (LAM)</a><ul>~~
+<a href="/articles/lymphangioleiomyomatosis-1">lymphangioleiomyomatosis (LAM)</a><ul>
~~-<li>cystic bronchiectasis from <a href="/articles/allergic-bronchopulmonary-aspergillosis">ABPA</a><ul>~~
+<li>
+<a href="/articles/cystic-bronchiectasis">cystic bronchiectasis</a> from <a href="/articles/allergic-bronchopulmonary-aspergillosis">ABPA</a><ul>
~~-<li><a href="/articles/langerhans-cell-histiocytosis-skeletal-manifestations-1">eosinophilic granuloma (EG)</a></li>~~
+<li><a href="/articles/langerhans-cell-histiocytosis-skeletal-manifestations-1">eosinophilic granuloma</a></li>

References changed:

1. David P. Naidich, Nestor L. Müller, W. Richard Webb. Computed Tomography and Magnetic Resonance of the Thorax. (2007) ISBN: 9780781757652 - <a href="http://books.google.com/books?vid=ISBN9780781757652">Google Books</a>
2. Schmidt S, Eich G, Geoffray A et al. Extraosseous Langerhans Cell Histiocytosis in Children. Radiographics. 2008;28(3):707-26. <a href="https://doi.org/10.1148/rg.283075108">doi:10.1148/rg.283075108</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/18480480">Pubmed</a>
3. Brauner M, Grenier P, Tijani K, Battesti J, Valeyre D. Pulmonary Langerhans Cell Histiocytosis: Evolution of Lesions on CT Scans. Radiology. 1997;204(2):497-502. <a href="https://doi.org/10.1148/radiology.204.2.9240543">doi:10.1148/radiology.204.2.9240543</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9240543">Pubmed</a>
4. Abbott G, Rosado-de-Christenson M, Franks T, Frazier A, Galvin J. From the Archives of the AFIP. Radiographics. 2004;24(3):821-41. <a href="https://doi.org/10.1148/rg.243045005">doi:10.1148/rg.243045005</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15143231">Pubmed</a>
5. Moore A, Godwin J, Müller N et al. Pulmonary Histiocytosis X: Comparison of Radiographic and CT Findings. Radiology. 1989;172(1):249-54. <a href="https://doi.org/10.1148/radiology.172.1.2787035">doi:10.1148/radiology.172.1.2787035</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/2787035">Pubmed</a>
6. Leatherwood D, Heitkamp D, Emerson R. Pulmonary Langerhans Cell Histiocytosis. Radiographics. 2007;27(1):265-8. <a href="https://doi.org/10.1148/rg.271065044">doi:10.1148/rg.271065044</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/17235012">Pubmed</a>
7. Juvet S, Hwang D, Downey G. Rare Lung Diseases III: Pulmonary Langerhans' Cell Histiocytosis. Can Respir J. 2010;17(3):e55-62. <a href="https://doi.org/10.1155/2010/216240">doi:10.1155/2010/216240</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/20617216">Pubmed</a>
8. Tazi A. Adult Pulmonary Langerhans' Cell Histiocytosis. Eur Respir J. 2006;27(6):1272-85. <a href="https://doi.org/10.1183/09031936.06.00024004">doi:10.1183/09031936.06.00024004</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16772390">Pubmed</a>
9. Vassallo R, Harari S, Tazi A. Current Understanding and Management of Pulmonary Langerhans Cell Histiocytosis. Thorax. 2017;72(10):937-45. <a href="https://doi.org/10.1136/thoraxjnl-2017-210125">doi:10.1136/thoraxjnl-2017-210125</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28689173">Pubmed</a>
10. Blakley M, Dutcher J, Wiernik P. Pulmonary Langerhans Cell Histiocytosis, Acute Myeloid Leukemia, and Myelofibrosis in a Large Family and Review of the Literature. Leuk Res. 2018;67:39-44. <a href="https://doi.org/10.1016/j.leukres.2018.01.011">doi:10.1016/j.leukres.2018.01.011</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29428448">Pubmed</a>
11. Egeler R, Neglia J, Aricò M, Favara B, Heitger A, Nesbit M. Acute Leukemia in Association with Langerhans Cell Histiocytosis. Med Pediatr Oncol. 1994;23(2):81-5. <a href="https://doi.org/10.1002/mpo.2950230204">doi:10.1002/mpo.2950230204</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/8202046">Pubmed</a>
12. Naeem M, Ballard D, Jawad H, Raptis C, Bhalla S. Noninfectious Granulomatous Diseases of the Chest. Radiographics. 2020;40(4):1003-19. <a href="https://doi.org/10.1148/rg.2020190180">doi:10.1148/rg.2020190180</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/32501738">Pubmed</a>
1. Computed tomography and magnetic resonance of the thorax. editors, David P. Naidich.. [et al.]; contributing author, Monvadi B. Srichai. Philadelphia : Wolters Kluwer/Lippincott Williams & Wilkins, c2007. <a href="http://www.worldcat.org/wcpa/isbn/0781757657">ISBN:0781757657</a> <a href="http://www.amazon.com/gp/product/0781757657?ie=UTF8&tag=radiopaediaor-20&linkCode=as2&camp=1789&creative=9325&creativeASIN=0781757657">(find it at amazon.com)</a>
2. Schmidt S, Eich G, Geoffray A et-al. Extraosseous langerhans cell histiocytosis in children. Radiographics. 28 (3): 707-26. <a href="http://dx.doi.org/10.1148/rg.283075108">doi:10.1148/rg.283075108</a> [<a href="http://www.ncbi.nlm.nih.gov/pubmed/18480480">pubmed citation</a>]
3. Brauner MW, Grenier P, Tijani K et-al. Pulmonary Langerhans cell histiocytosis: evolution of lesions on CT scans. Radiology. 1997;204 (2): 497-502. <a href="http://radiology.rsna.org/content/204/2/497.abstract">Radiology (abstract)</a> [<a href="http://www.ncbi.nlm.nih.gov/pubmed/9240543">pubmed citation</a>]
4. Abbott GF, Rosado-de-Christenson ML, Franks TJ et-al. From the archives of the AFIP: pulmonary Langerhans cell histiocytosis. Radiographics. 24 (3): 821-41. <a href="http://dx.doi.org/10.1148/rg.243045005">doi:10.1148/rg.243045005</a> [<a href="http://www.ncbi.nlm.nih.gov/pubmed/15143231">pubmed citation</a>]
5. Moore AD, Godwin JD, Müller NL et-al. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology. 1989;172 (1): 249-54. <a href="http://radiology.rsna.org/content/172/1/249.abstract">Radiology (abstract)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/2787035">Pubmed citation</a><div class="ref_v2"></div>
6. Leatherwood DL, Heitkamp DE, Emerson RE. Best cases from the AFIP: Pulmonary Langerhans cell histiocytosis. Radiographics : a review publication of the Radiological Society of North America, Inc. 27 (1): 265-8. <a href="https://doi.org/10.1148/rg.271065044">doi:10.1148/rg.271065044</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/17235012">Pubmed</a> <span class="ref_v4"></span>
7. Juvet SC, Hwang D, Downey GP. Rare lung diseases III: pulmonary Langerhans' cell histiocytosis. Canadian respiratory journal. 17 (3): e55-62. <a href="https://www.ncbi.nlm.nih.gov/pubmed/20617216">Pubmed</a> <span class="ref_v4"></span>
8. Tazi A. Adult pulmonary Langerhans' cell histiocytosis. The European respiratory journal. 27 (6): 1272-85. <a href="https://doi.org/10.1183/09031936.06.00024004">doi:10.1183/09031936.06.00024004</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16772390">Pubmed</a> <span class="ref_v4"></span>
9. Vassallo R, Harari S, Tazi A. Current understanding and management of pulmonary Langerhans cell histiocytosis. Thorax. 72 (10): 937. <a href="https://doi.org/10.1136/thoraxjnl-2017-210125">doi:10.1136/thoraxjnl-2017-210125</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28689173">Pubmed</a> <span class="ref_v4"></span>
10. Blakley MP, Dutcher JP, Wiernik PH. Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature. (2018) Leukemia research. 67: 39-44. <a href="https://doi.org/10.1016/j.leukres.2018.01.011">doi:10.1016/j.leukres.2018.01.011</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/29428448">Pubmed</a> <span class="ref_v4"></span>
11. Egeler RM, Neglia JP, Aricò M, Favara BE, Heitger A, Nesbit ME. Acute leukemia in association with Langerhans cell histiocytosis. (1994) Medical and pediatric oncology. 23 (2): 81-5. <a href="https://www.ncbi.nlm.nih.gov/pubmed/8202046">Pubmed</a> <span class="ref_v4"></span>
12. Naeem M, Ballard DH, Jawad H, Raptis C, Bhalla S. Noninfectious Granulomatous Diseases of the Chest. (2020) Radiographics : a review publication of the Radiological Society of North America, Inc. 40 (4): 1003-1019. <a href="https://doi.org/10.1148/rg.2020190180">doi:10.1148/rg.2020190180</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/32501738">Pubmed</a> <span class="ref_v4"></span>

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7","thumbnail":"https://prod-images-static.radiopaedia.org/images/55442257/93e953232f4a1a3b7a3158da136134087b081abae09ac2717021373d99951399_big_gallery.jpeg","isStack":true,"diagnosticCertainty":"confirmed_unsubstantiated"},{"imageId":53886551,"studyId":99502,"caseSlug":"pulmonary-langerhans-cell-histiocytosis-34","caption":"Case 8","thumbnail":"https://prod-images-static.radiopaedia.org/images/53886551/I0000186_0_0.dcm_big_gallery.jpeg","isStack":true,"diagnosticCertainty":"confirmed_unsubstantiated"},{"imageId":2803020,"studyId":21019,"caseSlug":"pulmonary-langerhans-cell-histiocytosis-6","caption":" Case 9","thumbnail":"https://prod-images-static.radiopaedia.org/images/2803020/c04504468250d5c909c925c7c9522d_big_gallery.jpg","isStack":false,"diagnosticCertainty":"confirmed_unsubstantiated"},{"imageId":7470392,"studyId":30420,"caseSlug":"pulmonary-langerhans-cell-histiocytosis-13","caption":"Case 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