The nonspecific symptoms of the disease are to blame, including fatigue, weight loss, fever, pressure drops, ulcer, diarrhea, bone pain – this is how this cancer manifests itself atypically. Therefore, mastocytosis.
It is a cancer that is difficult to detect. Patients who have been struggling with mastocytosis for years remain undiagnosed. And the disease with equal frequency occurs in women, men, children and can appear at any age
Mastocytosis is a rare myeloproliferative tumor. In patients, there is an excessive production of mast cells, i.e. mast cells, which belong to a group of white blood cells in one or more organs – most commonly in the bone marrow, skin, liver, spleen, and lymph nodes
Mastocytosis is a rare disease that belongs to myeloproliferative neoplasms. 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. The disease may be cutaneous – when mast cells accumulate in the sick or systemic skin – then there is
for the involvement of internal organs [1]. The diagnosis of mastocytosis remains a challenge for clinicians. The disease may be asymptomatic or without specific symptoms. Often, patients remain undiagnosed for years, visiting many doctors.
Mastocytosis is 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 lymph nodes [2].
The disease can attack the body and skin
Mastocytosis occurs with equal frequency in men and women [3]. It can appear at any age – both in children and adults [4]. The disease can take two main forms: cutaneous and systemic. In cases where the pathological accumulation of mast cells is limited to the skin only, skin mastocytosis is diagnosed when mast cells accumulate in internal organs (most often in the bone marrow, liver, spleen and lymph nodes) – systemic mastocytosis is diagnosed [5] , [6]. Systemic mastocytosis is also often associated with skin involvement. According to estimates, typical skin lesions occur in about 80% of all patients with mastocytosis [7]. In 3/4 of patients the disease begins in childhood, mainly in infancy, and is manifested by skin lesions. The second peak in morbidity is
3-4 decade of life. In 15% of patients, the changes are congenital [8]. In children, the disease is usually milder – usually as urticaria or solitary / multifocal infiltrates, and may completely resolve during adolescence. In contrast to pediatric forms, adult mastocytosis is generally systemic, progressive, and more aggressive.
In most patients, not only internal organs but also the skin are affected [9].
Non-specific symptoms make diagnosis difficult
Due to the lack of epidemiological studies, it is difficult to precisely determine the frequency of mastocytosis. It is estimated at 10 per 100 people [000]. Experts note, however, that some patients remain undiagnosed. The nonspecific symptoms of the disease are to blame, including fatigue, weight loss, fever, drops in blood pressure, peptic ulcer disease, diarrhea, aches and lesions such as osteopenia / osteoporosis [10]. In order to make a correct diagnosis, it is often necessary to closely cooperate with hematologists, allergists, gastrologists and dermatologists. In most cases, the involvement of the skin is the first symptom of the disease, and often it is the dermatologist who is the doctor who first visits patients with mastocytosis.
Diagnosis and treatment of mastocytosis
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 should order auxiliary tests, including peripheral blood count, bone marrow biopsy, as well as internal organ examinations (abdominal ultrasound, chest X-ray, intestinal examination, evaluation of the skeletal system). ) [12]. The main goal of treating sick people
for mastocytosis is to prevent or reduce the severity of symptoms.
In the treatment of cutaneous mastocytosis, drugs that act mainly symptomatically – reducing pruritus, urticarial reactions, the feeling of hot flush and gastrointestinal complaints are used.
In the case of systemic mastocytosis, intensive treatment is necessary to reduce the number of mast cells [13].
Patient education is necessary
Both cutaneous and systemic mastocytosis 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, firstly, and anaphylactic reaction. These include, among others: venoms, certain medications, temperature changes, solar radiation, alcohol, certain foods, food additives or food preservatives, spicy spices, various allergens, exercise, stress, and bacterial and viral infections. Sick children and parents or guardians of sick children should also be trained in first aid
in the event of an anaphylactic shock [14].
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.
Źródła:
[1] Magdalena Lange, Iwona Flisiak, Monika L. Kapińska-Mrowiecka, Andrzej Kaszuba, Joanna Maj, Lidia Rudnicka, Małgorzata Sokołowska-Wojdyło, “Mastocytosis. Diagnostic and therapeutic recommendations of the Polish Dermatological Society ”. The material is available at: https://www.termedia.pl/Mastocytoza-Rekomendacje-diagnostyczno-terapeutyczne-Polskiego-T Towarzystwa-Dermatologiczne,56,33305,0,0.html
[2] Izabela Dereń-Wagemann, Małgorzata Kuliszkiewicz-Janus, Kazimierz Kuliczkowski, “Mastocytosis – diagnosis and treatment”, Postępy High Med. Dosw. (online), 2009, pp. 564-576. The material is available at: https://phmd.pl/api/files/view/25939.pdf
[3] Izabela Dereń-Wagemann, Małgorzata Kuliszkiewicz-Janus …
[4] Krzysztof Lewandowski “Mastocytosis” [w.]: “Clinical Oncology” edited by Maciej Krzakowski, Piotr Potemski, Krzysztof Warzocha, Piotr Wysocki, Via Medica, vol. 3, p. 1040.
[5] Ibid.
[6] Magdalena Lange, Iwona Flisiak, Monika L. Kapińska-Mrowiecka …
[7] Ibid.
[8] Izabela Dereń-Wagemann, Małgorzata Kuliszkiewicz-Janus …
[9] Krzysztof Lewandowski “Mastocytosis” …, p. 1041.
[10] Magdalena Lange, Iwona Flisiak, Monika L. Kapińska-Mrowiecka …
[11] Krzysztof Lewandowski “Mastocytosis” …, p. 1041.
[12] Lek. Aleksandra Badzian, Mastocytosis. The material is available at: https://dermatologia.mp.pl/choroby/chorobyskory/73421,mastocytozy
[13] Ibidem…
[14] Magdalena Lange, Iwona Flisiak, Monika L. Kapińska-Mrowiecka Magdalena Lange, Iwona Flisiak, Monika L. Kapińska-Mrowiecka, Andrzej Kaszuba, Joanna Maj, Lidia Rudnicka, Małgorzata Sokołowska-Wojdyło, “Mastocytosis. Diagnostic and therapeutic recommendations of the Polish Dermatological Society ”. The material is available at: https://www.termedia.pl/Mastocytoza-Rekomendacje-diagnostyczno-terapeutyczne-Polskiego-T Towarzystwa-Dermatologiczne,56,33305,0,0.html, Izabela Dereń-Wagemann, Małgorzata Kuliszkiewicz-Janus, Kazimierz Kuliczkowski, “Mastocytosis – diagnosis and treatment”, Postępy High Med. Dosw. (online), 2009, pp. 564-576. The material is available at: https://phmd.pl/api/files/view/25939.pdf
[1] Izabela Dereń-Wagemann, Małgorzata Kuliszkiewicz-Janus …
[1] Krzysztof Lewandowski “Mastocytosis” [w.]: “Clinical Oncology” edited by Maciej Krzakowski, Piotr Potemski, Krzysztof Warzocha, Piotr Wysocki, Via Medica, vol. 3, p. 1040.
[1] Ibid.
[1] Magdalena Lange, Iwona Flisiak, Monika L. Kapińska-Mrowiecka …
[1] Ibid.
[1] Izabela Dereń-Wagemann, Małgorzata Kuliszkiewicz-Janus …
[1] Krzysztof Lewandowski “Mastocytosis” …, p. 1041.
[1] Magdalena Lange, Iwona Flisiak, Monika L. Kapińska-Mrowiecka …
[1] Krzysztof Lewandowski “Mastocytosis” …, p. 1041.
[1] Lek. Aleksandra Badzian, Mastocytosis. The material is available at: https://dermatologia.mp.pl/choroby/chorobyskory/73421,mastocytozy
[1] Ibidem…
[1] Magdalena Lange, Iwona Flisiak, Monika L. Kapińska-Mrowiecka …