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Langerhans cell histiocytosis

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Langerhans cell histiocytosis
Pathology
Type

Autoimmune

Cause(s)

Uncontrolled growth of a certain type of cell in the immune system

Symptoms

Bone swelling, rash, enlargement of liver, spleen or lymph nodes, excessive urination and thirst due to associated diabetes insipidus, chronic coughing, shortness of breath.

Mortality Rate

About 10%

Treatments

Surgery, radiation therapy, chemotherapy

Show Information
Appearances

Merry Little Christmas

  [Source]


CT of periorbital Langerhans cell histiocytosis

CT scan of a growth due to Langerhans cell histiocytosis in the skull near the eye, courtesy Countincr via Wikipedia Commons

Langerhans cell histiocytosis, also known as histiocytosis X, is a type of autoimmune disease related to uncontrolled production of certain types of cells of the immune system called Langerhans cells, which play a role in transporting antibodies to the skin. The production of these cells goes on unabated and the body responds with general inflammation that results in the symptoms of the disease.

Although not classified as a type of cancer, the uncontrolled growth of cells is typical of cancer and LCH will respond to the same therapy. If the inflammation is localized, surgery is generally effective. Otherwise, radiation therapy and chemotherapy are indicated. Patients generally respond well to treatment although patients where the disease is widespread will generally have to be treated throughout their lives.

Diagnosis is usually with a biopsy, which shows the distinctive shape of the Langerhans cells.

LCH is rare and generally presents in children under the age of 10. It is even rarer in adults and is almost unheard of in the elderly.

Histiocytosis at NIH

Langerhans cell histiocytois at Wikipedia

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