Mast cell tumor history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
History and Symptoms
Clinical pattern depends on mast cells burden in different organs and release of clinically relevant mediators such as histamine, leukotrienes, tryptase, and heparin.[1] There is considerable heterogeneity in the presentation of mastocytosis, and in the rate of disease progression. Clinical features include:[2]
- Pruritic cutaneous lesions
- Flushing
- Itching
- Rhinorrhea
- Abdominal cramping
- Wheezing
- Headache
- Dyspnea
- Nausea
- Vomiting
- Diarrhea
- Hypotension and syncope
- Abdominal pain
- Malabsorption
- Bone pain and pathological fractures
- Weight loss
- Fever and night sweats
| Type | Symptoms |
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Skin symptoms |
Most common in urticaria pigmentosa
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Mast cell reslease symptoms |
Observed in both cutaneous mastocytosis and systemic mastocytosis
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Non cutaneous organ infiltration |
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Urticaria Pigmentosa
- Urticaria pigmentosa is the most common clinical variant in which fixed, reddish brown lesions occurring as maculo-papules, plaques, nodules, or blisters are found.[3]
- These lesions urticate in response to physical irritation (Darier’s sign).
- Urticaria Pigmentosa (UP) lesions tend to be larger, better delineated, and more hyperpigmented in children, as compared to adults, who tend to have numerous small lesions that coalesce to form mottled areas.
- The trunk and thigh are more commonly involved with sparing of face, palms and soles.
Diffuse Cutaneous Mastocytosis
- Diffuse cutaneous mastocytosis is a rare variant of childhood mastocytosis that appears as diffuse infiltrative yellow-orange xanthogranuloma-like subcutaneous nodules, or as a widespread urticarial eruption with bullae and redness.
- The clinical course is more severe and can even be life-threatening, due to hypovolemic shock, mast cell leukemia, gastrointestinal hemorrhage, and cachexia[3]
Kinetics of blood clotting may be altered due to fibrinogenolytic and anticoagulant activities of tryptase and heparin respectively. Severe bleeding leading to the death of a patient with systemic mastocytosis due to heparin-like anticoagulant has been recently reported, and may represent a difficult diagnosis and a therapeutic challenge in the emergency room.[1]
References
- ↑ 1.0 1.1 Koenig, Martial; Morel, Jérôme; Reynaud, Jacqueline; Varvat, Cécile; Cathébras, Pascal (2008). "An unusual cause of spontaneous bleeding in the intensive care unit – mastocytosis: a case report". Cases Journal. 1 (1): 100. doi:10.1186/1757-1626-1-100. ISSN 1757-1626.
- ↑ Mastocytosis. Dr Alexandra Stanislavsky. Radiopaedia.org 2015. http://radiopaedia.org/articles/mastocytosis Accessed on February 29, 2016
- ↑ 3.0 3.1 Ferrante, Giuliana; Scavone, Valeria; Muscia, Maria; Adrignola, Emilia; Corsello, Giovanni; Passalacqua, Giovanni; La Grutta, Stefania (2015). "The care pathway for children with urticaria, angioedema, mastocytosis". World Allergy Organization Journal. 8 (1): 5. doi:10.1186/s40413-014-0052-x. ISSN 1939-4551.