Mastocytosis treatment

Systemic mastocytosis is a rare disease of the mast cells. Mast cells are cells of the hematopoietic system and play a decisive role in the defense against infection and in allergic reactions.

Stress as the most common cause of mast cell activation

Systemic mastocytosis is a rare disease of the mast cells. Mast cells are cells of the hematopoietic system and play a decisive role in the defense against infections and allergic reactions. When mast cells are activated, they can release certain substances (e.g. histamine) and thus fight pathogens and also mediate severe allergic reactions, for example to bee or wasp stings and certain medications. The release of these messenger substances is precisely regulated to prevent excessive release and thus damage to the organism. In mastocytosis, this regulatory mechanism is disrupted due to an acquired genetic change (mutation in the c-Kit gene) and the mast cells release an excessive amount of messenger substances after a stimulus or even without a recognizable trigger. Stress appears to be the most common trigger of mast cell activation.

Local mastocytosis

In the case of localized mastocytosis of the skin (“cutaneous mastocytosis”), this usually only leads to local changes in the affected areas of skin (redness and itching). In contrast, systemic mastocytosis, which is characterized by an increase in atypical mast cells outside the skin, is often accompanied by more generalized symptoms. Typical symptoms are: Itching, reddening of the skin and a feeling of heat (“flush”), shortness of breath, fluctuations in blood pressure, palpitations, nausea, vomiting, diarrhea, etc.

Systemic mastocytosis

The treatment of systemic mastocytosis is predominantly symptomatic. The focus is on avoiding foods, medications and situations that experience has shown to lead to symptoms caused by histamine release in individual cases. Older antihistamines are usually used as medication; their sedative effect is deliberately used to reduce the release of histamine. Low doses of benzodiazepines are often used for the same reason. As with other malignant diseases, aggressive forms are often treated “experimentally” with ciclosporin, corticosteroids and interferon α-2b without any evidence of their efficacy. Drugs that can reduce mast cells with high efficiency are now also available. However, these drugs (imatinib, mastinib), which are classified as chemotherapy, are only used for very aggressive forms due to their pronounced side effects and high costs.

PUVA as a therapeutic approach

Another therapeutic approach is PUVA, a treatment with psoralen, a photosensitizing substance, and subsequent exposure of the skin to UVA radiation.

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