نتایج جستجو برای: systemic mastocytosis
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INTRODUCTION The mast cell lives a hidden life, but it is implicated in several physiological reactions. Its ability to react to different stimuli impacts a variety of conditions such as asthma, atopic dermatitis, urticaria and anaphylaxis. It is not until recent decades that the evolution of the cell has been described and its fascinating biology has only recently been depicted. We here give a...
Mast cell disorders are defined by an abnormal accumulation of tissue mast cells in one or more organ systems. In systemic mastocytosis, at least one extracutaneous organ is involved by definition. Although, systemic mastocytosis usually represents with skin lesion called urticaria pigmentosa, in a small proportion, there is extracutaneous involvement without skin infiltration. Other manifestat...
Adult's mastocytosis is usually associated with persistent systemic involvement and c-kit 816 mutation, while pediatrics disease is mostly limited to the skin and often resolves spontaneously. We prospectively included 142 adult patients with histologically proven mastocytosis. We compared phenotypic and genotypic features of adults patients whose disease started during childhood (Group 1, n = ...
BACKGROUND Systemic mastocytosis is a rare myeloproliferative disease characterized by the uncontrolled proliferation of aberrant mast cells. It has varying clinical manifestations. For unknown reasons, pulmonary localization of mastocytosis is extremely rare. CASE PRESENTATION In this report, we describe a case of a young Caucasian female with systemic mastocytosis who had an associated hema...
Mastocytosis is a rare heterogeneous group of diseases with multi-organ involvement. Gastrointestinal (GI) tract infiltration by neoplastic mast cell is uncommon in systemic mastocytosis. In our literature review, there are only 11 published mastocytosis reports including 42 cases of biopsy confirmed direct colon involvement. We report here a rare case of focal mastocytosis in the rectum withou...
We present the case of a 40-year-old male with a past medical history of urticaria pigmentosa (UP) who presented for consultation to the Allergy clinic for an opinion of systemic mastocytosis. Previous workups included elevated serum tryptase level, UP on skin biopsy and an increased number of CD117-positive/CD25-positive mast cells on bone marrow biopsy. This case emphasizes the importance of ...
Diffuse cutaneous mastocytosis, the most rare form of cutaneous mastocytosis, often manifests as bullous lesions. Although cutaneous mastocytosis should be included in a differential diagnosis for pruritic skin lesions in children, early diagnosis of the disease is not easy due to its rare occurrence. A 17-month-old boy presented with recurrent itchy bullous skin lesions. He had been treated as...
OBJECTIVE Mastocytosis comprises a group of diseases characterized by accumulation of mast cells on the skin, with the possibility of affecting other systems. Symptoms can be exclusively cutaneous, associated with symptoms of the organs involved or even systemic, due to the release of chemical mediators. Three cases of systemic mastocytosis are described, each case presenting different manifest...
BACKGROUND 2D7 and BB1 are thought to be basophil-specific markers. In this study, we tested both antibodies in different skin and mast cell disorders with the aim of determining whether it was possible to differentiate between benign and aggressive presentations of mastocytosis. METHODS Using the antibodies 2D7, BB1, and c-Kit, we performed an immunohistochemical study of skin biopsy specime...
Cooperating genetic events are likely to contribute to the phenotypic diversity of KIT-D816V systemic mastocytosis. In this study, 44 patients with KIT-D816V systemic mastocytosis were evaluated for coexisting NRAS, KRAS, HRAS or MRAS mutations. Activating NRAS mutations were identified in 2 of 8 patients with advanced disease. NRAS mutations were not found in patients with indolent systemic ma...
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