Efficacy of <scp>PUVA</scp> in Russian patients with mastocytosis: a case series and review of literature

Pyatilova, Polina; Teplyuk, N. P.; Olisova, Olga Yu.; Kovrigina, А М; Gadaev, I. Yu.; Kolkhir, Pavel

doi:10.1111/ijd.14103

Cited by 4 publications

(2 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The first clinical observations suggest that skin lesions may indeed decrease and sometimes even fade away under such TKI therapy [56]. Ultraviolet A (UVA) therapy can also suppress skin lesions in SM [93,94]. However, after treatment discontinuation, the skin lesions always re-appear.…”

Section: Management Of Mediator-related Symptoms Anaphylaxis and mentioning

confidence: 99%

Multidisciplinary Challenges in Mastocytosis and How to Address with Personalized Medicine Approaches

Valent

Akin

Gleixner

et al. 2019

IJMS

100

View full text Add to dashboard Cite

Mastocytosis is a hematopoietic neoplasm defined by abnormal expansion and focal accumulation of clonal tissue mast cells in various organ-systems. The disease exhibits a complex pathology and an equally complex clinical behavior. The classification of the World Health Organization (WHO) divides mastocytosis into cutaneous forms, systemic variants, and localized mast cell tumors. In >80% of patients with systemic mastocytosis (SM), a somatic point mutation in KIT at codon 816 is found. Whereas patients with indolent forms of the disease have a normal or near-normal life expectancy, patients with advanced mast cell neoplasms, including aggressive SM and mast cell leukemia, have a poor prognosis with short survival times. In a majority of these patients, multiple somatic mutations and/or an associated hematologic neoplasm, such as a myeloid leukemia, may be detected. Independent of the category of mastocytosis and the serum tryptase level, patients may suffer from mediator-related symptoms and/or osteopathy. Depending on the presence of co-morbidities, the symptomatology in such patients may be mild, severe or even life-threatening. Most relevant co-morbidities in such patients are IgE-dependent allergies, psychiatric, psychological or mental problems, and vitamin D deficiency. The diagnosis and management of mastocytosis is an emerging challenge in clinical practice and requires vast knowledge, a multidisciplinary approach, and personalized medicine procedures. In this article, the current knowledge about mastocytosis is reviewed with special emphasis on the multidisciplinary aspects of the disease and related challenges in daily practice.

show abstract

Section: Management Of Mediator-related Symptoms Anaphylaxis and mentioning

confidence: 99%

Multidisciplinary Challenges in Mastocytosis and How to Address with Personalized Medicine Approaches

Valent

Akin

Gleixner

et al. 2019

IJMS

100

View full text Add to dashboard Cite

show abstract

“…Napromienianie ultrafioletowe (UVA, ultraviolet) może stłumić zmiany skórne w SM. Jednak zwykle efekty są krótkotrwałe i po zakończeniu leczenia zmiany pojawiają się ponownie [36,37]. Pierwsze obserwacje kliniczne z zastosowaniem nowych inhibitorów kinazy tyrozynowej KIT, takich jak midostauryna lub awaprytinib, sugerują, że typowe dla mastocytozy skórne przebarwienia mogą się zmniejszać, a czasem nawet zupełnie zanikać w przebiegu leczenia [32] (tab.…”

Section: Zmiany Skórneunclassified

Diagnostyka i leczenie mastocytozy układowej — stanowisko ekspertów

et al. 2020

View full text Add to dashboard Cite

Systemic mastocytosis (SM) is a rare hematological malignancy characterized by an abnormal expansion and accumulation of pathological mast cells in bone marrow and other organs includ-ing skin, liver, spleen and lymph nodes. The clinical manifestation can be extremely heterogeneous, from limited skin changes to multi-organ involvement or mast cell tumors. The median survival of patients diagnosed with indolent form is comparable to healthy population, while prognosis for patients with advanced disease is poor, with an estimated survival ranging from several months to several years. In most patients (> 90%), a somatic mutation in codon 816 of the c-KIT gene encoding tyrosine kinase receptor is detected. Additional molecular abnormalities and even coexistence of other hematological cancers, e.g. acute leukemia, are also observed. Regardless of the form of disease or serum tryptase concentration, patients are exposed to symptoms resulting from the release of mast cell mediators -most often itching, paroxysmal redness and blisters, and general mediator-induced symptoms -such as nausea, vomiting, diarrhea, abdominal pain, hypotensive episodes, fatigue, headache, fever, shortness of breath, osteopenia, osteoporosis and severe anaphylactic reactions. This paper presents current outlook on the diagnostic and treatment process of SM, taking into account the interdisciplinary aspects of the disease.

show abstract

Urticaria Pigmentosa – A Case Report with a Review of the Literature

Yungareva,

Broshtilova,

Smilov

et al. 2024

IJCD

View full text Add to dashboard Cite

Mastocytosis encompasses a rare group of diseases characterized by the accumulation of mast cells, primarily in the skin or internal organs. In the 2016 revised WHO classification, mastocytosis is divided into cutaneous mastocytosis (CM), systemic mastocytosis (CM), and localized mast cell tumor. Cutaneous mastocytosis (CM) includes maculo-papular CM/urticaria pigmentosa (UP), diffuse CM and cutaneous mastocytoma. Urticaria pigmentosa is the most common skin variant. It presents with erythematous brownish macules or papules, often accompanied by pruritus. A case of a 48-year-old woman with disseminated, mildly pruritic, sharply demarcated, livid-brownish macules on the skin of the trunk and extremities is presented. A positive Darier symptom was established. Laboratory tests revealed granulocytosis (73%) and lymphocytosis (19.8%). Serum tryptase and 24-hour urine 5-hydroxyindoleacetic acid were not elevated. The diagnosis of urticaria pigmentosa was confirmed by the histopathological examination revealing perivascular infiltrates of mast cells, mainly in the deep dermis. Screening performed did not detect systemic involvement. Symptomatic treatment with H1 and H2 blockers and topical corticosteroid was carried out. Avoidance of triggers is recommended. The patient's condition has improved. Both a historical review of mastocytosis and a revised classification, as well as the epidemiology, etiology with triggering factors, clinical presentation, laboratory investigations, and management of urticaria pigmentosa are presented.

show abstract

Efficacy of PUVA in Russian patients with mastocytosis: a case series and review of literature

Cited by 4 publications

References 25 publications

Multidisciplinary Challenges in Mastocytosis and How to Address with Personalized Medicine Approaches

Multidisciplinary Challenges in Mastocytosis and How to Address with Personalized Medicine Approaches

Diagnostyka i leczenie mastocytozy układowej — stanowisko ekspertów

Urticaria Pigmentosa – A Case Report with a Review of the Literature

Contact Info

Product

Resources

About