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: