The information on this website is not intended for diagnosis or treatment. Please consult your physician for health concerns.

+90 850 321 50 00

Advanced Pulmonary Langerhans Cell Histiocytosis (PLCH)

BRAF/MEK-targeted therapy and transplantation for advanced pulmonary Langerhans cell histiocytosis.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Göğüs Hastalıkları department. Book Appointment →

What is Advanced Pulmonary Langerhans Cell Histiocytosis (PLCH)?

Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related cystic lung disease in which clonal CD1a-positive Langerhans cells accumulate in airways, often harboring BRAF V600E or MAP2K1 mutations.

Patients are typically young to middle-aged adult smokers who present with cough, dyspnea, recurrent spontaneous pneumothorax and characteristic upper- and mid-lobe nodules and bizarrely shaped cysts on HRCT.

Advanced disease can progress to severe cystic destruction, pulmonary hypertension and respiratory failure; modern management includes complete smoking cessation, BRAF/MEK inhibitors (vemurafenib, dabrafenib + trametinib) for multisystem BRAF V600E disease, vinblastine-based chemotherapy in selected cases, and lung transplantation in end-stage disease.

Symptoms

Persistent cough
Progressive dyspnea
Recurrent spontaneous pneumothorax
Constitutional symptoms (fatigue, weight loss)
Hemoptysis in severe cases
Bone pain (multisystem disease)
Diabetes insipidus (hypothalamic involvement)

Risk Factors

Current or heavy past smoking
Young to middle adult age
BRAF V600E or MAP2K1 mutations
Multisystem Langerhans cell histiocytosis
Family history of LCH (rare)
Smoking-related coexistent lung disease
Pulmonary hypertension as advanced complication

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Cystic lung disease in smoker
  • Recurrent pneumothorax in young adult
  • New diabetes insipidus with lung disease
  • Progressive dyspnea despite smoking cessation
  • Pulmonary hypertension on echocardiography
  • Need for BRAF/MEK testing
  • Pre-transplant evaluation

Treatment Methods

01
Complete and sustained smoking cessation
02
HRCT, lung biopsy and BRAF V600E mutation testing
03
Vemurafenib or dabrafenib + trametinib in BRAF-positive multisystem disease
04
Vinblastine-prednisone or cytarabine-based regimens in select multisystem cases
05
Treatment of pulmonary hypertension
06
Pleurodesis for recurrent pneumothorax
07
Lung transplantation in end-stage disease

Which Department to Visit?

You can visit our Göğüs Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Göğüs Hastalıkları Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

Book AppointmentContact Us

Related Health Topics

Other articles from the same department you may want to explore.

Asthma

Göğüs Hastalıkları

Asthma is characterized by wheezing, coughing and shortness of breath attacks; with proper treatment it can be kept under control.

COPD (Chronic Obstructive Pulmonary Disease)

Göğüs Hastalıkları

COPD is an irreversible lung disease characterized by shortness of breath and chronic cough; quitting smoking slows its progression.

Pneumonia

Göğüs Hastalıkları

Pneumonia presents with high fever, cough and shortness of breath; the vast majority recover with appropriate antibiotic treatment.

Tuberculosis (TB)

Göğüs Hastalıkları

Tuberculosis presents with weeks-to-months of cough, fever, and night sweats; early diagnosis and treatment lead to full recovery.

Pleural Effusion

Göğüs Hastalıkları

Pleural effusion is the accumulation of excess fluid in the pleural space, resulting from imbalances in fluid production and removal, and represents a manifestation of diverse cardiopulmonary, infectious, and malignant disorders.

Pneumothorax

Göğüs Hastalıkları

Pneumothorax is the presence of air in the pleural space resulting in partial or complete lung collapse, classified as spontaneous (primary/secondary), traumatic, or iatrogenic, with tension pneumothorax representing a life-threatening emergency.

Bronchitis (Acute and Chronic)

Göğüs Hastalıkları

Acute bronchitis is mostly viral and resolves spontaneously, while chronic bronchitis is a smoking-related component of COPD.

Bronchiectasis

Göğüs Hastalıkları

Bronchiectasis is a chronic respiratory disease characterized by permanent, abnormal dilation of bronchi with associated destruction of muscular and elastic components of airway walls, resulting in impaired mucociliary clearance and recurrent infection.

Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.