Definition
Mastocytosis is a disease characterized by the accumulation of mast cells in various tissues and organs of the body.
Basically, two different forms of mastocytosis can be distinguished: cutaneous (whose symptoms occur only at the skin level) and systemic (whose symptoms can occur in any part of the body).
Causes
The underlying causes of the onset of mastocytosis have not yet been fully identified. However, it seems that the development of the disease is favored by a mutation in the c-KIT gene. This mutation can occur spontaneously, or it can be inherited. from parents.
Symptoms
The mast cells that accumulate in the various organs and tissues release large quantities of histamine and it is the excessive release of this neurotransmitter that is responsible for most of the symptoms induced by mastocytosis.
The characteristic symptoms of cutaneous mastocytosis consist in the formation of macules, papules, plaques, nodules or blisters on the skin. These manifestations are usually accompanied by itching, swelling and redness of the skin.
The symptoms caused by systemic mastocytosis, on the other hand, are: hepatomegaly, splenomegaly, enlarged lymph nodes, peptic ulcers, arthralgia, loss of appetite and body weight, weakness, osteoporosis, increased frequency of urination, palpitations, hot flashes, hypotension, pain headache, nausea, diarrhea, chest pain and shortness of breath.
Furthermore, patients with mastocytosis (both cutaneous and systemic) are exposed to an increased risk of experiencing anaphylaxis.
The information on Mastocytosis - Drugs for the Treatment of Mastocytosis is not intended to replace the direct relationship between health professional and patient. Always consult your doctor and / or specialist before taking Mastocytosis - Drugs for the Treatment of Mastocytosis.
Medicines
In truth, there are no drugs capable of treating mastocytosis, but therapies can be instituted to reduce the symptoms it induces.
The drugs used are of different types and may vary according to the type of mastocytosis you suffer from (cutaneous or systemic).
However, the most commonly used drugs in the treatment of mastocytosis are corticosteroids, antihistamines and - in the case of patients with osteoporosis - bisphosphonates.
In cases of aggressive systemic mastocytosis and in cases of systemic mastocytosis associated with diseases such as leukemias, lymphomas and multiple myeloma, the doctor may decide to resort to the administration of particular anticancer drugs, such as interferon-alpha and imatinib.
Finally, for the treatment of skin lesions caused by mastocytosis, it may be useful to subject patients to P-UVA therapy.
The following are the classes of drugs most used in the therapy against mastocytosis and some examples of pharmacological specialties; it is up to the doctor to choose the active ingredient and dosage most suitable for the patient, based on the severity of the disease, the state of health of the patient and his response to treatment.
Corticosteroids
Corticosteroids are powerful anti-inflammatory drugs that work by interfering with the activity of the immune system.
In the treatment of moderate intensity cutaneous mastocytosis, it is usually preferred to resort to the use of topical corticosteroids.
In the event that mastocytosis causes intense itching or particularly severe arthralgia, the doctor may decide to intervene by administering corticosteroids systemically.
Among the different steroidal anti-inflammatories that can be used, we remember:
- Prednisone (Deltacortene ®): Prednisone is available for oral administration. The dose of drug usually administered is 5-15 mg per day. The exact dosage of the medicinal product must be determined by the physician on an individual basis, depending on the severity of the disease.
- Methylprednisolone (Urbason ®, Medrol ®, Solu-Medrol ®, Advantan ®): Methylprednisolone is available in pharmaceutical formulations suitable for both oral and topical administration.
When administered orally, the initial dose of drug to be taken should be determined by the physician, depending on the patient's condition. Thereafter, the drug dose administered can be changed according to the patient's response to therapy. In any case, indicatively, the dose of methylprednisolone used varies from 4 mg up to 48 mg per day.
When, however, preparations for cutaneous use based on methylprednisolone are used, it is recommended to apply the product once a day, directly on the skin area affected by the lesions. - Hydrocortisone (Locoidon ®, Dermirit ®): Hydrocortisone is available for ocular, rectal and skin administration.
To treat skin lesions caused by cutaneous mastocytosis, it is recommended to apply the hydrocortisone-based product directly on the affected area, once or twice a day, according to medical prescription. - Dexamethasone (Decadron ®, Soldesam ®, Dermadex ®): dexamethasone is available in many pharmaceutical formulations that allow it to be administered through different routes.
When using dexamethasone skin cream, it is recommended to perform two or three applications per day, directly on the affected area.
However, when dexamethasone is administered orally, the dose to be used should be determined by the physician on an individual basis.
Antihistamines
As can be deduced from their name, antihistamine drugs are used to counteract the effects of histamine which is released in large quantities in patients with mastocytosis (both cutaneous and systemic).
In this regard, the antihistamines that are used are of two types:
- Histamine H1 receptor antagonists, used to treat skin itching and redness caused by mastocytosis.
- H2 receptor antagonists for histamine, also known as "anti-ulcer drugs". These active ingredients, in fact, block the release of histamine in the stomach and are used to treat the peptic ulcer that typically occurs in patients with systemic mastocytosis.
Among the various H1 receptor antagonists that can be used, we mention cetirizine (Cerchio ®, Zirtec ®). It is a drug available for oral administration. The usual dose of cetirizine used in adults is 10 mg per day. In any case, the doctor will determine the exact amount of medicine that each patient should take.
Among the H2 receptor antagonists for histamine, however, we mention ranitidine (Ranidil ®, Zantac ®, Livin ®). This drug is administered orally at the usual dose of 300 mg per day, to be taken in two divided doses.
Bisphosphonates
Bisphosphonates can be used to treat osteoporosis that systemic mastocytosis can cause.
Among the various bisphosphonates that can be used, we mention alendronic acid (Alendros ®, Fosamax ®, Adronat ®, Dronal ®). Alendronic acid is available for oral administration. The dose of active ingredient usually administered is 10 mg per day, or 70 mg once a week. The drug should be administered in the morning, at least thirty minutes before the intake of any food, drink or other drug.
Interferon-alpha
Interferon-alpha is a drug with anticancer properties that is used in the treatment of aggressive systemic mastocytosis and, above all, in the treatment of systemic mastocytosis associated with leukemia, lymphoma or multiple myeloma.
Interferon-alpha (Alfaferon ®) can only be administered parenterally to patients by specialized personnel. The dose of medicine to be used will be determined by the physician on an individual basis for each patient.
Imatinib
Imatinib is also an anticancer drug that can be used for the treatment of aggressive systemic mastocytosis and for the treatment of systemic mastocytosis associated with leukemia, lymphoma or multiple myeloma.
Imatinib (Glivec ®, Imatinib Medac ®, Imatinib Accord ®) is available for oral administration in the form of tablets or capsules. The dose of drug to be used can vary from 100 mg up to 800 mg per day. determine the exact dosage of imatinib to be taken by each patient.
