Gastrointestinal Dysfunction in Systemic Mastocytosis

Cherner, Jay A.; Jensen, Robert T.; Dubois, A.; O'Dorisio, Thomas M.; Gardner, Jerry D.; Metcalfe, Dean D.

doi:10.1016/s0016-5085(88)80012-x

Cited by 134 publications

(87 citation statements)

References 38 publications

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“…These findings are similar to those reported in other small series and case reports. 2,[5][6][7][8][9][10][11]13,14,[16][17][18][21][22][23]26,30,31 Malabsorption, which occurred in two patients in our series is well described in systemic mastocytosis, 2,5,7,9,10,16,23,30,31 occurring in 5-30% of patients. 30,32 While all patients in our series were women, the literature indicates an equal sex distribution for systemic mastocytosis in general 33 and for those cases with direct gastrointestinal involvement.…”

Section: Discussionmentioning

confidence: 97%

“…2,[5][6][7][8][9][10][11]13,14,[16][17][18][21][22][23]26,30,31 Malabsorption, which occurred in two patients in our series is well described in systemic mastocytosis, 2,5,7,9,10,16,23,30,31 occurring in 5-30% of patients. 30,32 While all patients in our series were women, the literature indicates an equal sex distribution for systemic mastocytosis in general 33 and for those cases with direct gastrointestinal involvement. [5][6][7][8][9][10][11]13,[16][17][18][19][21][22][23]31 Endoscopically and grossly, the most frequent and distinctive finding in our series was that of mucosal nodularity (present in four of five patients).…”

Section: Discussionmentioning

confidence: 97%

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Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

et al. 2008

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 97%

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

et al. 2008

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“…Interestingly, no gastroduodenal ulcers were observed in patients with secondary MCL whereas they were found in 38% of de novo MCL cases. As gastroduodenal ulcers are linked to the release of histamine by pathologic MC, 21,22 we could hypothesize that in de novo MCL, pathologic MC are highly aggressive, proliferate rapidly, infiltrate all organs (including the digestive tract), and degranulate easily, releasing large amounts of histamine, leading to gastroduodenal ulcer formation.…”

Section: De Novo or Secondary MCLmentioning

confidence: 99%

Mast cell leukemia

Georgin‐Lavialle

Lhermitte

Dubreuil

et al. 2013

Blood

158

215

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“…The most frequent symptoms are bloating and diarrhea followed by abdominal pain and nausea. 24,[72][73][74] Symptoms may be caused by peptic ulcer disease, esophagitis gastrointestinal edema or dysmotility.…”

Section: Clinical Presentation and Gastrointestinal Involvementmentioning

confidence: 99%

Flushing Disorders Associated with Gastrointestinal Symptoms: Part 1, Neuroendocrine Tumors, Mast Cell Disorders and Hyperbasophila

et al. 2018

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Flushing is the subjective sensation of warmth accompanied by visible cutaneous erythema occurring throughout the body with a predilection for the face, neck, pinnae, and upper trunk where the skin is thinnest and cutaneous vessels are superficially located and in greatest numbers. Flushing can be present in either a wet or dry form depending upon whether neural-mediated mechanisms are involved. Activation of the sympathetic nervous system results in wet flushing, accompanied by diaphoresis, due to concomitant stimulation of eccrine sweat glands. Wet flushing is caused by certain medications, panic disorder and paroxysmal extreme pain disorder (PEPD). Vasodilator mediated flushing due to the formation and release of a variety of biogenic amines, neuropeptides and phospholipid mediators such as histamine, serotonin and prostaglandins, respectively, typically presents as dry flushing where sweating is characteristically absent. Flushing occurring with neuroendocrine tumors accompanied by gastrointestinal symptoms is generally of the dry flushing variant, which may be an important clinical clue to the differential diagnosis. A number of primary diseases of the gastrointestinal tract cause flushing, and conversely extra-intestinal conditions are associated with flushing and gastrointestinal symptoms. Gastrointestinal findings vary and include one or more of the following non-specific symptoms such as abdominal pain, nausea, vomiting, diarrhea or constipation. The purpose of this review is to provide a focused comprehensive discussion on the presentation, pathophysiology, diagnostic evaluation and management of those diseases that arise from the gastrointestinal tract or other site that may cause gastrointestinal symptoms secondarily accompanied by flushing. This review is divided into two parts given the scope of conditions that cause flushing and affect the gastrointestinal tract: Part 1 covers neuroendocrine tumors (carcinoid, pheochromocytomas, vasoactive intestinal polypeptide, medullary carcinoma of the thyroid), polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS), and conditions involving mast cells and basophils; while Part 2 covers dumping syndrome, mesenteric traction syndrome, rosacea, hyperthyroidism and thyroid storm, anaphylaxis, panic disorders, paroxysmal extreme pain disorder, and food, alcohol and medications.

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Gastrointestinal Dysfunction in Systemic Mastocytosis

Cited by 134 publications

References 38 publications

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

Mast cell leukemia

Flushing Disorders Associated with Gastrointestinal Symptoms: Part 1, Neuroendocrine Tumors, Mast Cell Disorders and Hyperbasophila

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