Mast cell tumor history and symptoms: Difference between revisions
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==Overview== | ==Overview== | ||
==History and Symptoms== | ==History and Symptoms== | ||
Symptoms of mast cell tumor depends on mast cells burden in different organs and release of clinically relevant mediators such as [[histamine]], [[leukotrienes]], [[tryptase]], and [[heparin]].<ref name="KoenigMorel2008">{{cite journal|last1=Koenig|first1=Martial|last2=Morel|first2=Jérôme|last3=Reynaud|first3=Jacqueline|last4=Varvat|first4=Cécile|last5=Cathébras|first5=Pascal|title=An unusual cause of spontaneous bleeding in the intensive care unit – mastocytosis: a case report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=100|issn=1757-1626|doi=10.1186/1757-1626-1-100}}</ref> There is considerable heterogeneity in the presentation of mastocytosis, and in the rate of disease progression. Clinical features include:<ref name=radiopaedia>Mastocytosis. Dr Alexandra Stanislavsky. Radiopaedia.org 2015. http://radiopaedia.org/articles/mastocytosis Accessed on February 29, 2016 | Symptoms of mast cell tumor depends on mast cells burden in different organs and release of clinically relevant mediators such as [[histamine]], [[leukotrienes]], [[tryptase]], and [[heparin]].<ref name="KoenigMorel2008">{{cite journal|last1=Koenig|first1=Martial|last2=Morel|first2=Jérôme|last3=Reynaud|first3=Jacqueline|last4=Varvat|first4=Cécile|last5=Cathébras|first5=Pascal|title=An unusual cause of spontaneous bleeding in the intensive care unit – mastocytosis: a case report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=100|issn=1757-1626|doi=10.1186/1757-1626-1-100}}</ref> There is considerable heterogeneity in the presentation of mastocytosis, and in the rate of disease progression. Clinical features include:<ref name="radiopaedia">Mastocytosis. Dr Alexandra Stanislavsky. Radiopaedia.org 2015. http://radiopaedia.org/articles/mastocytosis Accessed on February 29, 2016</ref> | ||
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Skin symptoms | Skin symptoms | ||
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May be present in both systemic and cutaneous mastocytosis. Most common in urticaria pigmentosa: | May be present in both systemic and cutaneous mastocytosis. Most common in urticaria pigmentosa: | ||
*Pruritic cutaneous lesions | *Pruritic cutaneous lesions | ||
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Mast cell reslease symptoms | Mast cell reslease symptoms | ||
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May be present in both cutaneous mastocytosis and systemic mastocytosis. Symptoms may occur in episodes: | May be present in both cutaneous mastocytosis and systemic mastocytosis. Symptoms may occur in episodes: | ||
*[[Hypotension]] | *[[Hypotension]] | ||
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Non cutaneous organ infiltration: | Non cutaneous organ infiltration: | ||
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Only present in systemic mastocytosis | Only present in systemic mastocytosis | ||
*[[Anemia]] | *[[Anemia]] | ||
Revision as of 02:07, 25 March 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]
Overview
History and Symptoms
Symptoms of mast cell tumor 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]
| Type | Symptoms |
|---|---|
|
Skin symptoms |
May be present in both systemic and cutaneous mastocytosis. Most common in urticaria pigmentosa:
|
|
Mast cell reslease symptoms |
May be present in both cutaneous mastocytosis and systemic mastocytosis. Symptoms may occur in episodes: |
|
Non cutaneous organ infiltration: |
Only present in systemic mastocytosis
|
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]
- 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]
References
- ↑ 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.