Histiocytosis

Histiocytosis General Information

Histiocytosis is a medical condition described as the presence of an excessive number of histiocytes, cells that are part of the immune system of humans and represents a general name given to a certain group of syndromes. Histiocyte is also considered a tissue macrophage cell. Some scientists believe that these cells are derivate from the Langerhans cells. This term typically refers to a group of very rare medical conditions that have this as a common characteristic. Histiocyte cells are considered to be dendritic cells, monocytes and macrophages. Histiocytosis is also known in the medical domain under other several names, for example: eosinophilic granuloma, Hand-Schuler-Christian disease, nonlipid reticuloendotheliosis and pulmonary Langerhans cell granulomatosis. This medical condition can be divided in three major classes: non-Langerhans cell histiocytoses, Langerhans cell histiocytoses and T-cell lymphoma, also known as malignant histiocytoses syndrome. This rare blood disorder appears when the white cells of the blood (called histiocytes) cluster together and affect different parts of the body, for example: ears, gums, eyes, lung, spleen, skin, liver, bones and the central nervous system or CNS. This medical condition can have a limited involvement and then regress spontaneously or can have a progressive involvement of much more organs, reaching to a debilitating and chronic stage. This is why, in some serious cases, Histiocytosis can represent a real threat to the patient’s life. It is considered to have many similarities with cancer, being treated by many oncologists with radiation therapies and with chemotherapy. It can also go into remission without a certain therapy.

Histiocytosis Symptoms

The signs and symptoms specific for the medical condition known as Histiocytosis vary depending on the age of the patient. Other symptoms, besides the ones we are going to present you in this chapter have also been reported. In most cases that involve children, the disorder appears from birth to the age of fourteen and involves problems with the bones. Depending on the number of affected organs, the health care providers will apply the adequate treatment option. In children, the signs and symptoms are more numerous and include: pain felt in the abdominal area, weight loss, nausea, vomiting, dizziness, jaundice, failure to thrive, irritability, limping, fever, headaches, seizures, seborrheic dermatitis of the scalp, chronically draining ears, delayed puberty, frequent urination, increased eyeball protrusion, mental deterioration, thirst, short stature, swollen lymph glands, possible pain of the bones, or generalized rash(such as purpura or petechiae). In adults, the main signs and symptoms of Histiocytosis are fewer and include: shortness of breath, increased amount of urine output, pain felt in the chest area, cough, fever, bone pain, increased drinking of fluids, weight loss, rash, thirst, uneasiness, general discomfort or ill feeling (for example, malaise). If not treated in time, there is the risk that the persons suffering from this blood disorder develop some more complicated health problems, for example: diffuse interstitial pulmonary fibrosis, diabetes insipidus , problems with the lungs that will lead to lung failure, spontaneous pneumothorax, dysfunctions of the pituitary gland leading to growth failure, invasion of the bone marrow by the tumors that is associated with subsequent severe anemia.

Histiocytosis Treatment

If you start experiencing pain in the chest area, shortness of breath along with other signs and symptoms specific for this medical condition known as Histiocytosis, then we strongly recommend you to contact medical professional help. Depending on the severity of the clinical manifestations and on the extent of the disease, you should call your health care provider and establish a date for the consultation. However, if your health status is seriously damaged, you must go to the emergency room. The examination of the patient must be carefully done so that the health care provider can proceed as soon as possible with the appropriate medical therapy. The methods of examination may differ if the patient is a child or an adult. The most common tests that an adult must perform are: X-rays of the chest, tests of the pulmonary functioning and bronchoscopy along with a biopsy that will usually reveal abnormalities in histiocytes. On the other hand, children must do X-rays of the bones that may show punched-out appearance of the bone lesions, CBC (complete blood count), biopsy of a small sample of the skin and biopsy of the bone marrow to see if Langerhans cells are present, X-rays of the entire skeleton, called skeletal survey X-ray that will help the doctor to see how much of the skeleton is damaged. In other particular cases, if needed, some additional tests can be performed.

After establishing the right diagnosis (of Histiocytosis), the health care provider should proceed with the treatment option that best fits with the patient’s demands and needs. Usually, the first treatment option is the one based on corticosteroids. Corticosteroids are actually anti-inflammatory drugs that have the role of suppressing the immune functions, especially against the dangerous cells. If the patient diagnosed with this medical condition smokes, he or she should quit, because it is well known that smoking has the effect of worsening the response to this type of treatment. Some examples of the most common corticosteroids that we can offer as guidance are: Etoposide, Vinblastine, Prednisone, cyclophosphamide, Methotrexate and Mercaptopurine. When Histiocytosis affects children, prednisone and mercaptopurine are not used because are more powerful and can cause several adverse effects. There were cases when Histiocytosis regressed on its own without a specific treatment. But there were also cases that needed therapy with radiation or chemotherapy. These two treatment options have proved to be very effective against this medical condition. A number of health care providers can choose limited surgery or therapy based on radiation in order to treat bone lesions, if they have occurred. Because the immune system of a person that suffers from Histiocytosis is affected, being severely damaged, he or she may get infected with other conditions, more easily and more seriously. This is why many patients receive supportive care that will reduce the signs and symptoms and will treat the infections. In these cases, the doctor may prescribe to the patient several antibiotics, physical therapy, hormonal replacement therapy, and so on.