Mastocytosis is a rare disease that belongs to myeloproliferative neoplasms. It can occur in both children and adults. In people suffering from mastocytosis, there is an excessive production of mast cells, i.e. mast cells, which belong to the group of white blood cells. Symptoms are not always visible in mastocytosis - it can also be asymptomatic. Therefore, patients often go undiagnosed. How is cutaneous mastocytosis different from systemic mastocytosis? Find out how mastocytosis is treated.

Mastocytosisis basically a group of diseases characterized by excessive proliferation (multiplication) and accumulation of mast cells in one or more organs - most often in the bone marrow, skin, liver, spleen and nodes absorbents.

Systemic vs cutaneous mastocytosis

Mastocytosis occurs with equal frequency in men and women. It can appear at any age - in both children and adults. The disease can take two forms: cutaneous and systemic.

In cases where pathological mast cell accumulation is limited to the skin only, cutaneous mastocytosis is diagnosed when mast cells accumulate in internal organs (most often in the bone marrow, liver, spleen and lymph nodes). systemic mastocytosis. Systemic mastocytosis is also often accompanied by skin involvement.

Mastocytosis in children and adults

According to estimates, typical skin lesions occur in about 80% of all patients with mastocytosis. In 3/4 of patients the disease begins in childhood, mainly in infancy, and is manifested by skin lesions.

The second peak of incidence is the 3-4th decade of life. In 15% of patients, the changes are congenital. In children, the disease is usually milder - usually as hives or solitary / multifocal infiltrates, and may resolve completely during adolescence.

In contrast to pediatric forms, adult mastocytosis is mostly systemic, progressive, and more aggressive. In most patients, not only the internal organs but also the skin are affected.

Mastocytosis - symptoms

Due to the lack of epidemiological studies, it is difficult to precisely determine the frequency of mastocytosis. It is estimated to beit is 10 out of 100,000 people. Experts note, however, that some patients remain undiagnosed.

The non-specific symptoms of the disease are to blame, including the following:

  • fatigue
  • weight loss
  • fever
  • drops in blood pressure
  • peptic ulcer disease
  • diarrhea
  • occurrence of pains and lesions of the type of osteopenia / osteoporosis

In order to make a correct diagnosis - it is therefore often necessary to closely cooperate with the doctors: hematologist, allergist, gastrologist and dermatologist.

In most cases, skin involvement is the first symptom of the disease, and it is often the dermatologist who is the doctor who first visits patients with mastocytosis.

Mastocytosis - diagnosis and treatment

The diagnosis of cutaneous mastocytosis is based on the histological examination of the skin sample.

If the doctor suspects systemic mastocytosis, based on the patient's general symptoms, he or she should order auxiliary tests, including:

  • peripheral blood count
  • bone marrow biopsy
  • examination of internal organs

- abdominal ultrasound
- chest X-ray
- bowel evaluation
- skeletal evaluation

The main goal of treating patients with mastocytosis is to prevent or reduce the severity of symptoms.

In the treatment of cutaneous mastocytosis, drugs that act mainly symptomatically - reduce pruritus, urticarial reactions, the feeling of hot flush and digestive system ailments.

In the case of systemic mastocytosis, intensive treatment is required to reduce the number of mast cells.

Mastocytosis - patient education

Mastocytosis, both cutaneous and systemic, carries the risk of an anaphylactic reaction. It occurs in about 50% of adults and about 5-10% of children with mastocytosis. In adults, it is most often associated with allergy to Hymenoptera venom, and in most children it is provoked by an unknown factor.

The attending physician should inform the patient about the factors that may lead to exacerbation of mastocytosis symptoms, first, and anaphylactic reaction. These include, among others :

  • jady
  • some medications
  • temperature changes
  • solar radiation
  • alcohol
  • some foods
  • food additives or food preservatives
  • spicy spices
  • various allergens
  • physical exertion
  • stress
  • infectionsbacterial and viral

Patients and parents or guardians of sick children should also be trained in first aid in the event of an anaphylactic shock.

Mastocytosis is a disease that cannot be prevented. The key to effective treatment, however, is to see a doctor as soon as possible when the patient experiences disturbing symptoms, so as to make a diagnosis as soon as possible and start appropriate treatment.

Category:

Oncology