Histiocytosis

What is histiocytosis?
Histiocytosis is a disorder of the white blood cells called histiocytes. This disease resembles cancer and is treated by oncologists in much the same way. It is a disease that mostly affects children, but may be found in people of all ages.
 
There are three major types of histiocytosis:
• Langerhans cell histiocytosis, also called histiocytosis X
• Malignant histiocytosis syndrome (known as T-cell lymphoma)
• Non-Langerhans cell histiocytosis  (known as hemophagocytic syndrome)
 
The most common is Langerhans cell histiocytosis (LCH)
 
What causes histiocytosis?
The cause of some forms of the disease is genetic, while others remain unknown to researchers.

What are the symptoms of histiocytosis?
The symptoms in adults usually are:
• Cough
• Fever
• Bone pain
• Chest pain
• Weight loss
• Excessive thirst
• Excessive urination
• Rash, or other abnormal skin lesions
• Shortness of breath
• Difficulty walking

How is histiocytosis diagnosed?
• A bone x-ray and skeletal survey x-ray are usually done at time of diagnosis and then repeated every 6 months for patients with bone lesions
•  Blood tests include: complete blood count (CBC), liver function test, kidney function test and urine osmolality
• If skin involvement is suspected then physicians will take a skin biopsy. A bone marrow biopsy is taken for patients with anemia to check if abnormal cells are present.
• Chest X-ray - important to check for lung involvement
• Endocrine evaluation: Important to rule out pituitary gland problems that can occur in patients with skull involvement
• CT scan of chest and abdomen: If involvement of internal organs is suspected, especially for patients with abnormal liver function tests

How is histiocytosis treated at KHCC?
Although not a cancer, the disorder is treated in much the same way, using surgery, chemotherapy and radiation therapy in varying degrees. For patients with single organ involvement like skin or bone, local treatment in the form of topical chemotherapy or curettage of involved bone may be all what needs to be done. For patients with multiple bone spots or internal organs involvement, chemotherapy and steroids can be very effective. Some patients with multiple organs and central nervous system (i.e., brain or spine) involvement may need more intensive treatment and in some cases an allogenic bone marrow transplant. All forms of treatment for histiocytosis are available at King Hussein Cancer Center.
 
Physicians
At KHCC, patients with myeloproliferative disorders receive top quality care through a multidisciplinary team of specialists highly experienced in treating blood disorders .
 
Supportive Care
The lymphoma multidisciplinary clinic works in close cooperation with other departments at KHCC so that histiocytosis patients receive the most comprehensive care possible. Supportive care services at KHCC include: