Histiocytosis is a term used to identify a rare blood disease. The disease is due to an excess of histiocytes (white blood cells) in the blood stream. Histiocytosis is also used to refer to other diseases, but mainly for this rare syndrome. Excess histiocytes form a cluster that attack bones, lungs, liver, spleen, gums, ears, eyes, the central nervous system and skin. The disease can have a small affect on a patient and then disappear or it can have a serious and chronic multi-organ affect. This can be a life threatening disease depending on the organs that are attacked.
Histiocytosis is very similar to cancer and has been treated with chemotherapy and radiation. It is not like cancer in that it can disappear from the body without any intervention or treatment. There are at least eight forms of histiocytosis, some of which are Langerhans cell Histiocytosis, Hemophagocytic lymphohistiocytosis, Juvenile Xanthogranuloma, Erdheim-Chester disease, and Rosai-Dorfman disease
Patients are mostly children under the age of 10, though sometimes it can be diagnosed in adults. This extremely rare disease affects 1 in 200,000 children.
Langerhans cell histiocytosis (LCH) is a rare disorder that primarily affects children. The disease was first described in medical literature around the turn of the 20th century.
A histiocyte is a form of white blood cell. Its job is to help destroy certain foreign materials and fight infection. For unknown reasons, patients with this disease have too many histiocytes (Langerhans cells). These cells accumulate in different organs and can result in a variety of symptoms.
Symptoms depend on which part of the body is affected or involved with histiocytosis. Not every patient will experience the same symptoms.
Bone involvement presents symptoms such as one or many lesions, pain, headache (from skull lesions) or a limp (from leg lesions). Gastrointestinal involvement can produce pain, jaundice, naseau, esophagus bleeding, diarrhea, vomiting and weight loss. Skin involvement may produce a waxy rash that looks like scales and does not respond to typical intervention. Pulmonary involvement can present as feeding disorders, vomiting, diarrhea, difficulty breathing, cough with or without blood, chest pain and weight loss. When histiocytosis occurs in the pituitary gland it is known as Diabetes Insipidus and causes dehydration, excessive urination and thirst, delayed puberty and short stature or stunted growth. Brain involvement has the symptoms of mental deterioration, diabetes insipidus, seizures, vertigo, protruding eyeballs, swallowing problems, vomiting and headaches. Organ involvement refers to liver, lung and spleen and the symptoms are dysfunction of these organs. Eye involvement presents as bulging eyes and vision difficulties. Ear involvement will have these symptoms: inflamed ear canal, rash on scalp or behind the ear, cysts in the ear and a bad smelling oozing. Mouth involvement will cause pain and swelling, loose or lost teeth, swollen, bleeding gums and swollen lymph nodes in the area.
Universal symptoms are fever, fatigue, failure to thrive (in infants) or to gain weight (in older children).
A biopsy of the infected tissue is needed to make a diagnoses of histiocytosis. Other tests are used to determine the amount or involvement of the disease within the body, such as blood test, MRI, and a biopsy of the liver or bone marrow.
Treatment is designed to be as minimal as possible in order to give the patient’s body an opportunity to heal itself. Histiocytosis of the skin is usually seen in males under the age of one and the lesion resolves itself. It is important to determine where histiocytosis is in the body to assess treatment and prognosis. An individual lesion on the bone will heal itself. A biopsy of the lesion on the bone has been known to initiate healing of the lesion. Chemotherapy and radiation are sometimes used to treat this disease, but in very small doses.
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