Tuesday, September 22, 2009

Langerhans Cell Histiocytosis




Langerhans Cell Histiocytosis is a rare disease that occurs when there are too many of a certain white blood cell. These cells function normally within the body and their job is to help fight infections. LCH occurs when too many of these cells accumulate in an area of the body. Scientists are still unsure as to why this occurs to some people. The most common place to see this is in the head and neck, sternum, ribs, long bones, and vertebrae. This disorder most often affects children 10 and under.

Common symptoms include skin rash, tenderness/pain around the area involved, loose or lost teeth, swollen gums, vision problems and weakness to name a few. To diagnose this disease a CBC is drawn, CT or MRI done and even a biopsy can be necessary.

Treatments vary widely from simply taking medication and surgery to radiation therapy and chemotherapy. Chemotherapy is the most serious treatment that is considered as this mode of therapy can’t differentiate between cancerous cells and healthy cells. Remember that the majority of those diagnosed with this disease are children.

The long-term outlook for a child with this condition is good. Survival rate is high, but many have long-term chronic ailments.

The image above is taken from the textbook, Maxillofacial Imaging and demonstrates this disease in a one year child. The image shows a soft tissue mass that has eroded the mastoid cortex and complete opacification/destruction of the mastoid air cells.


Susan Brumley

1 comment:

  1. Common symptoms include skin rash, tenderness/pain around the area involved, loose or lost teeth, swollen gums, vision problems and weakness to name a few. To diagnose this disease a CBC is drawn, CT or MRI done and even a biopsy can be necessary.

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