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: the cutaneous one (whose symptoms appear only at the skin level) and the systemic one (whose symptoms can appear in any part of the body).
Causes
The causes underlying 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 the parents.
Symptoms
The mast cells that accumulate in the various organs and tissues release large amounts 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 skin redness.
The symptoms caused by systemic mastocytosis, however, are: hepatomegaly, splenomegaly, swollen lymph nodes, peptic ulcers, arthralgia, loss of appetite and body weight, weakness, osteoporosis, increased frequency of urination, palpitations, hot flushes, hypotension, mal headache, nausea, diarrhea, chest pain and shortness of breath.
Furthermore, patients with mastocytosis (both cutaneous and systemic) are at greater risk of experiencing anaphylaxis.
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.
drugs
In truth, there are no drugs that can cure mastocytosis, but therapies can be set up to reduce the symptoms it induces.
The drugs used are of different types and may vary depending on the type of mastocytosis from which one suffers (cutaneous or systemic).
However, the drugs most commonly used in mastocytosis therapy 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 pathologies such as leukemias, lymphomas and multiple myeloma, the doctor may decide to resort to the administration of particular drugs with antitumor action, 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.
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 use topical corticosteroids.
In the case where mastocytosis is the cause of 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 recall:
- Prednisone (Deltacortene ®): prednisone is available for oral administration. The dose of drug usually administered is 5-15 mg a day. The exact dosage of medicine must be established by the doctor 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 either oral administration or topical administration.
When administered orally, the initial dose of medication to be taken should be determined by the doctor, depending on the patient's condition. Subsequently, the dose of drug administered can be changed depending on the patient's response to therapy. However, indicatively, the dose of methylprednisolone used varies from 4 mg to 48 mg a day.
When, on the other hand, methylprednisolone-based preparations are used for skin use, 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 dermal 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 a medical prescription.
- Dexamethasone (Decadron ®, Soldesam ®, Dermadex ®): dexamethasone is available in many pharmaceutical formulations which allow its administration through different routes.
When using dexamethasone based skin cream, it is recommended to apply two or three applications a day, directly on the affected area.
When, on the other hand, dexamethasone is administered orally, the dose to be used must be established by the doctor on an individual basis.
Antihistamines
As can be deduced from their own name, antihistamine drugs are used to counteract the effects of histamine which is released in large quantities in patients suffering from mastocytosis (both cutaneous and systemic).
In this regard, two types of antihistamines are used:
- H1 receptor antagonists for histamine, used to treat the itching and skin redness caused by mastocytosis.
- H2 receptor antagonists for histamine, also known as "anti-ulcer drugs". In fact, these active ingredients block the release of histamine at the gastric level and are used to treat peptic ulcers that typically occur in patients with systemic mastocytosis.
Among the different H1 receptor antagonists that can be used, we mention cetirizine (Cerchio ®, Zirtec ®). It is a drug available for oral administration. The dose of cetirizine usually used in adults is 10 mg a day. In any case, the doctor will determine the exact amount of medicine that each patient will have to take.
Among the H2 receptor antagonists for histamine, however, we recall 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 recall alendronic acid (Alendros ®, Fosamax ®, Adronat ®, Dronal ®). Alendronic acid is available for oral administration. The dose of active ingredient usually administered is 10 mg a day, or 70 mg once a week. The drug should be administered in the morning, at least thirty minutes before taking any food, drink or other medication.
Interferon-alpha
Interferon-alpha is a drug with antitumor properties which 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 (Alfaferone ®) can be given to patients parenterally only by specialized personnel. The dose of medicine to be used will be established by the doctor 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 as tablets or capsules. The dose of drug to be used can range from 100 mg to 800 mg a day. The doctor will determine the exact dosage of imatinib that each patient will have to take.
