Blood Disorders

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Langerhan Histiocytosis

What is Langerhans Cell Histiocytosis?

Langerhan cell histiocytosis is a rare blood disease that is caused by an excess of white blood cells called histiocytes, which cluster together and attack the skin, bones, lung, liver, spleen, gums, ears, eyes, and/or brain. The disease can range from limited involvement that spontaneously regresses to progressive multi-organ involvement that can be chronic and debilitating. In some cases, the disease can be life-threatening.

Who gets LCH? 

Histiocytosis is more common in children than adults and affects 1 in 200,000 children born each year in the United States, though it can affect adults of any age. The cause is in most cases is not known.  However, in cases where only the lungs are involved, smoking has been strongly implicated.

Symptoms and Diagnosis of LCH

LCH can present increased thirst and urination (diabetes insipidus) due to involvement of an area in the brain called the pituitary gland. Other symptoms may be bone pain, particular lower jaw, skin rash, cough or shortness of breath, weight loss, fever, and enlarged lymph nodes or spleen. The diagnosis of LCH requires a biopsy of an affected area. The biopsy is best reviewed by large academic centers since it is rare and the findings can be subtle.

Treatment of LCH

LCH is best treated at large institution like Seattle Cancer Care Alliance where there will be doctors who have experience in treating this rare disease. Since it is so rare in adults, there is little research about its cause and treatment and no standard treatment is available. Many adult treatments are based on what has been successful in treating children, but the results have not been good. When the disease is localized to one or two areas, surgery and/or radiation therapy have been used. Patients with mild symptoms may be managed with steroids and some will improve without treatment. When only the lungs are involved, smoking cessation has resulted in some cures. For patients with more extensive disease and/or severe symptoms, chemotherapy is required. Hematopoietic stem cell transplant  should also be considered for patients who have a poor response to chemotherapy.