Langerhans Cell Histiocytosis Questions & Answers

Updated: Jun 12, 2020
  • Author: Christopher R Shea, MD; Chief Editor: William D James, MD  more...
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Questions & Answers

Overview

What is Langerhans cell histiocytosis (LCH)?

What is the pathophysiology of Langerhans cell histiocytosis (LCH)?

What causes Langerhans cell histiocytosis (LCH)?

What is the prevalence of Langerhans cell histiocytosis (LCH)?

What are the racial predilections of Langerhans cell histiocytosis (LCH)?

What are the sexual predilections of Langerhans cell histiocytosis (LCH)?

Which age groups have the highest prevalence of Langerhans cell histiocytosis (LCH)?

What is the prognosis of Langerhans cell histiocytosis (LCH)?

Presentation

Which clinical history findings are characteristic of Langerhans cell histiocytosis (LCH)?

Which physical findings are characteristic of Langerhans cell histiocytosis (LCH)?

Which physical findings are characteristic of chronic unifocal Langerhans cell histiocytosis (LCH)?

Which physical findings are characteristic of classic multifocal Langerhans cell histiocytosis (LCH)?

Which physical findings are characteristic of acute disseminated Langerhans cell histiocytosis (LCH)?

Which physical findings are characteristic of congenital self-healing histiocytosis?

What are the possible complications of Langerhans cell histiocytosis (LCH)?

DDX

Which conditions are included in the differential diagnoses of Langerhans cell histiocytosis (LCH)?

What are the differential diagnoses for Langerhans Cell Histiocytosis?

Workup

What is the role of blood testing in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of liver function tests in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of urinalysis in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of chest radiography in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of skeletal radiograph survey in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of CT scanning or MRI in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of FDG-PET scanning in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of pulmonary function testing in the workup of Langerhans cell histiocytosis (LCH)?

When is a small bowel series indicated in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of hormonal studies in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of visual, neurologic and auditory testing in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of cerebrospinal fluid biomarkers in the workup of Langerhans cell histiocytosis (LCH)?

What is the role of biopsy in the workup of Langerhans cell histiocytosis (LCH)?

Which histologic findings are characteristic of Langerhans cell histiocytosis (LCH)?

How is Langerhans cell histiocytosis (SS-LCH) staged?

Treatment

How is Langerhans cell histiocytosis (LCH) treated?

How is single-system Langerhans cell histiocytosis (LCH) treated?

How is multisystem Langerhans cell histiocytosis (LCH) treated?

What is the efficacy of chemotherapeutic agents used to treat Langerhans cell histiocytosis (LCH)?

How is refractory Langerhans cell histiocytosis (LCH) treated?

What is the role of intense MACOP-B therapy in the treatment of Langerhans cell histiocytosis (LCH)?

Which drugs have been proposed for use in the treatment of Langerhans cell histiocytosis (LCH)?

What is the role of vemurafenib in the treatment of Langerhans cell histiocytosis (LCH)?

Which specialist consultations are beneficial to patients with Langerhans cell histiocytosis (LCH)?

Medications

What is the goal of drug treatment for Langerhans cell histiocytosis (LCH)?

Which medications in the drug class Bisphosphonate derivatives are used in the treatment of Langerhans Cell Histiocytosis?

Which medications in the drug class Phototherapy agents are used in the treatment of Langerhans Cell Histiocytosis?

Which medications in the drug class Antineoplastic agents are used in the treatment of Langerhans Cell Histiocytosis?

Which medications in the drug class Nonsteroidal anti-inflammatory drugs are used in the treatment of Langerhans Cell Histiocytosis?

Which medications in the drug class Corticosteroids are used in the treatment of Langerhans Cell Histiocytosis?