Langerhans' Cell Histiocytosis

A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001)

Symptoms of Langerhans' Cell Histiocytosis

The following features are indicative of Langerhans' Cell Histiocytosis:
  • trouble speaking
  • inability to walk or balance
  • tremors of their hands with difficulty writing
  • lethargy
  • fever
  • weight loss
  • rash
  • diabetes insipidus
It is possible that Langerhans' Cell Histiocytosis shows no physical symptoms and still is present in a patient.

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Common Causes of Langerhans' Cell Histiocytosis

The following are the most common causes of Langerhans' Cell Histiocytosis:
  • genetic mutation in the v-raf murine sarcoma viral oncogene homolog B1 gene
  • family history
  • smoking
  • genetic mutation in the mitogen-activated protein kinase (MAP2K) gene

Risk Factors for Langerhans' Cell Histiocytosis

The following factors may increase the likelihood of Langerhans' Cell Histiocytosis:
  • smoking
  • family history
  • being Hispanic

Prevention of Langerhans' Cell Histiocytosis

No, it is not possible to prevent Langerhans' Cell Histiocytosis.
  • genetic defect

Occurrence of Langerhans' Cell Histiocytosis

Number of Cases

The following are the number of Langerhans' Cell Histiocytosis cases seen each year worldwide:
  • Not common between 50K - 500K cases

Common Age Group

Langerhans' Cell Histiocytosis can occur at any age.

Common Gender

Langerhans' Cell Histiocytosis can occur in any gender.

Lab Tests and Procedures for Diagnosis of Langerhans' Cell Histiocytosis

The following lab tests and procedures are used to detect Langerhans' Cell Histiocytosis:
  • Neurological exam: To examine the nerve, brain and spinal cord function
  • Liver function test: To measure the blood levels of certain substances released by the liver
  • Complete blood count (CBC) with differential: To measure the amount of the hemoglobin, white blood cells, red blood cells and platelets
  • Physical exam and history: To examine the general signs of health
  • Blood chemistry studies: To measure the unusual amount of a substance released into the body by organs and tissues in the body
  • BRAF gene testing: To detect the mutations of the BRAF gene
  • Water deprivation test: To diagnose diabetes insipidus
  • Urinalysis: To examine the color of urine and its contents
  • Bone marrow aspiration and biopsy: To evaluate the signs of Langerhans Cell Histiocytosis (LCH)
  • Magnetic resonance imaging (MRI): To view the detailed pictures of areas inside the body
  • Computed tomography (CT) scan: View the organs or tissues more clearly
  • Bone scan: To examine if there are rapidly dividing cells in the bone

Doctor for Diagnosis of Langerhans' Cell Histiocytosis

Patients should visit the following specialists if they have symptoms of Langerhans' Cell Histiocytosis:
  • Hematologist

Complications of Langerhans' Cell Histiocytosis if untreated

Yes, Langerhans' Cell Histiocytosis causes complications if it is not treated. Below is the list of complications and problems that may arise if Langerhans' Cell Histiocytosis is left untreated:
  • spinal cord damage
  • fractures of long bones
  • compression of vertebrae
  • pulmonary hypertension
  • pulmonary fibrosis

Procedures for Treatment of Langerhans' Cell Histiocytosis

The following procedures are used to treat Langerhans' Cell Histiocytosis:
  • Surgery: Treats the bone lesions
  • Chemotherapy: Destroy the growth of cancer cells

Patient Support for Treatment of Langerhans' Cell Histiocytosis

The following actions may help Langerhans' Cell Histiocytosis patients:
  • Join support organization: Helps in coping with the disease by connecting with other patients and families

Time for Treatment of Langerhans' Cell Histiocytosis

While time-period of treatment for each patient may vary, below is the typical time-period for Langerhans' Cell Histiocytosis to resolve if treated properly under an expert supervision:
  • More than 1 year

Last updated date

This page was last updated on 2/04/2019.
This page provides information for Langerhans' Cell Histiocytosis.
Langerhans' Cell Histiocytosis

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