Mastocytosis. Review of the literature and description of clinical cases

Меликян, А Л; Subortseva, Irina N.; Горячева, С. Р.; Колошейнова, Т И; Вахрушева, М В; Kovrigina, А М; Sudarikov, Andrey; Двирнык, В Н; Obukhova, T N

doi:10.17116/terarkh20148612127-134

Cited by 4 publications

(3 citation statements)

References 57 publications

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“…Пациентам следует избегать перегревания (бани, сауны), инсоляции, соблюдать строгую диету, исключающую морепродукты (особенно креветки, лобстеры, крабы, икру), алкоголь, шоколад, тирамин-содержащие продукты (сыры, орехи, злаковые, бобовые), консервы. Подобные ограничения эффективны и в отношении профилактики частоты приступов мигрени [15].…”

Section: заключениеunclassified

Cephalalgia against the background of systemic mastocytosis: a clinical case

Кузнецова¹,

Танашян²,

Меликян³

et al. 2021

Bûll. sib. med.

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The article presents a clinical case of comorbid pathology -development of migraine against the background of systemic mastocytosis. The classification and clinical manifestations of systemic mastocytosis, a rare blood disease, are given. This clinical case illustrates an example of excessive mast cell degranulation (with the release of proinflammatory and vasodilating agents as a result of mast cell pathology). In this regard, in addition to the pathogen-specific therapy for systemic mastocytosis (including prevention of mast cell degranulation), it seems important to describe the effectiveness and the possibility of prescribing preventive and emergency therapy for migraine against the background of the underlying hematological disease -systemic mastocytosis.Treatment of cephalalgia in patients with mastocytosis is a complex clinical task, in the solution of which it is necessary to take into account serious limitations in prescription of acetylsalicylic acid and other non-steroidal anti-inflammatory drugs.

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Section: заключениеunclassified

Cephalalgia against the background of systemic mastocytosis: a clinical case

Кузнецова¹,

Танашян²,

Меликян³

et al. 2021

Bûll. sib. med.

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“…Occasionally, 3-4 nodes can be present predominantly on the neck and forearms. This subtype of mastocytosis is found only in newborns and is characterized by spontaneous regression (14).…”

Section: Clinical Manifestations and Prognosis Of MCmentioning

confidence: 99%

“…In patients with mastocytosis symptoms, associated with MC mediator excretion, are present, including periodic flushing (erythema, appearing spontaneously or as a reaction to a number of triggers), itching, urticarial dermographism, positive Darier's symptom (spots get reddish and swollen when rubbing) (17). Other symptoms, associated with MC mediators include: anaphylactic reactions, recurrent syncopes, occasional hypotension, gastrointestinal distress such as diarrhea, abdominal pain, gastric ulcers and neurological and psychiatric disorders (5,14).…”

Section: Clinical Manifestations and Prognosis Of MCmentioning

confidence: 99%

Laboratory Tests in Diagnosis of Mastocytosis: Literature Review and Case Report

Орлова

Шкурлатовская

Silina

et al. 2020

Serbian Journal of Experimental and Clinical Research

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Mastocytosis is a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MCs) in the skin and/or other organ systems. Mastocytosis is a rare disease. The annual incidence is 5–10 cases per 1 million people. However, the majority of cases stay undiagnosed due to the lack of specific tests and a wide variety of clinical features of the disease. In mastocytosis, somatic mutations of KIT gene lead to autocrine dysregulation and constitutive c-KIT activation in the absence of its ligand SCF. Clinical symptoms of the disease are determined by MC mediator release and/or infiltration of tissues by MCs. According to the World Health Organisation classification updated in 2016 mastocytosis is divided to cutaneous mastocytosis (CM), indolent systemic mastocytosis (ISM), smoldering systemic mastocytosis (SSM), SM with an associated hematologic (non-MC-lineage) neoplasm (SMAHN), aggressive SM (ASM), MC leukemia (MCL) and MC sarcoma (MCS). The CM and ISM prognosis is excellent with (almost) normal life expectancy, unlike aggressive forms (ASM and MCL) with poor prognosis. In this paper the key aspects of clinical features and diagnostic criteria of mastocytosis are discussed. We present a case report of a patient with mastocytosis in the skin following psoralen plus ultraviolet A (PUVA) therapy with good response.

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Pathomorphology of cutaneous mastocytosis

et al. 2023

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Introduction. Mastocytosis as a result of mast cell infiltration of the skin and other organs develops at any age. The relevance of this work is determined by the lack of systematic data on the pathomorphological features of the clinical forms and types of cutaneous mastocytosis, defined by the Consensus European-American Mastocytosis Group and the WHO classification in 2016. The variability of dermatological clinical manifestations in children and adults, the difficulties in interpreting laboratory tests create a problem for verifying the diagnosis and further treatment. The aim. To determine the main pathohistological features of clinical forms and types of cutaneous mastocytosis. Material and methods. The article includes data from patients who were treated and monitored at the Moscow Scientific and Practical Center of Dermatovenereology and Cosmetology in 2019–2022. Results. The literature and own data on the characteristic pathomorphological patterns that are important for the diagnosis of mastocytosis with skin lesions in children and adults are systematized. The pathological substrate of lesions in diffuse cutaneous mastocytosis and mastocytoma was a massive infiltrate of mast cells in the papillary and reticular layers of the dermis. The histological picture in the monomorphic type of maculopapular cutaneous mastocytosis (MPCM) in children was characterized by a relatively lower density of mast cell infiltration of the papillary dermis compared to other clinical forms of mastocytosis. Also, in children, the density of mast cells in the affected skin was significantly higher compared to adults. In addition, in adults with PPCM, the papillary dermis was less infiltrated with mast cells. This confirms the opinion that the monomorphic type of MPCM in children is phenotypically similar to MPCM in adults. Conclusions. Further prospective follow-up of patients may allow conclusions to be drawn about the prognostic value of pathological examination in mastocytosis in adults and children.

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Mastocytosis. Review of the literature and description of clinical cases

Cited by 4 publications

References 57 publications

Cephalalgia against the background of systemic mastocytosis: a clinical case

Cephalalgia against the background of systemic mastocytosis: a clinical case

Laboratory Tests in Diagnosis of Mastocytosis: Literature Review and Case Report

Pathomorphology of cutaneous mastocytosis

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