Mastocytic Enterocolitis: A Newly Described Mast Cell Entity

Ogilvie-McDaniel, Chrishana; Blaiss, Michael S.; Osborn, F. David; Carpenter, Julie

doi:10.1016/s1081-1206(10)60231-1

Cited by 13 publications

(12 citation statements)

References 2 publications

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“…Appendicitis typically presents when an obstructed fecalith leads to an acute inflammatory response. Some studies suggest that mucosal inflammation may lead to mast cell infiltrates as part of an acute inflammatory response [13]. Other studies indicate that mast cell degranulation in the lamina propria may directly interact with motor neurons and silent nociceptors leading to smooth muscle contraction, diarrhea, and abdominal pain [14].…”

Section: Discussionmentioning

confidence: 99%

Systemic Mastocytosis Presenting as Acute Appendicitis: A Case Report and Review of the Literature

Akbar¹,

Raza²,

Denney

et al. 2013

Case Rep Oncol

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Systemic mastocytosis is characterized by abnormal growth and accumulation of mast cells in various organs. Gastrointestinal (GI) symptoms are common disease manifestations in this disease and can significantly impair the quality of life. Signs of GI systemic mastocytosis include steatorrhea, malabsorption, hepatomegaly, splenomegaly, portal hypertension, and ascites. Acute appendicitis as a presenting feature in systemic mastocytosis has not been reported in the literature previously. In this report, we discuss the case of a female patient with systemic mastocytosis (c-KIT D816V (+)) who was admitted for right-sided acute abdominal pain. Laboratory study revealed an normal white blood cell count with eosinophilia and an elevated serum tryptase level of 23 μg/l. CT of the abdomen and pelvis showed an enlarged appendix of 12 mm in diameter, with minimal wall enhancement. Laparoscopic appendectomy was performed. The appendix was found to be hyperemic and firm, and it was densely adherent to the posterior cecum, the surrounding peritoneal wall, and the overlying mesenteric fat. Pathology revealed acute appendicitis with greater than 30 mast cells per high-power field by immunoperoxidase studies with mast cell tryptase and CD117. The patient subsequently improved and was discharged home. This case is the first reported case with a histological diagnosis of acute appendicitis resulting from mast cell infiltration. Physicians should be aware of acute appendicitis as a manifestation of systemic mastocytosis. Prompt diagnosis and management may prevent potentially fatal complications of appendiceal perforation and peritonitis.

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Section: Discussionmentioning

confidence: 99%

Systemic Mastocytosis Presenting as Acute Appendicitis: A Case Report and Review of the Literature

Akbar¹,

Raza²,

Denney

et al. 2013

Case Rep Oncol

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“…Of these 33 patients, 67% had a cessation or reduction of diarrhoea after receiving H1 and H2 receptor antagonists, with or without an MC mediator release inhibitor [19]. Further cases have since been reported [58][59][60][61][62][63][64] (Table 1).…”

Section: Mastocytic Enterocolitismentioning

confidence: 99%

Mastocytic Enterocolits and the Role of Mast Cells in Functional and Inflammatory Intestinal Disorders: A Systematic Review

Capannolo¹,

Ciccone²,

Latella³

2018

Dig Dis

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Background: Chronic diarrhoea affects 5–10% of the adult population. Histologic lesions of possible diagnostic significance are found under normal colonoscopy in approximately 30% of patients affected by chronic diarrhoea. Mastocytic enterocolitis is characterized by an increase in the number of mucosal mast cells (MC) in the gut of patients with chronic intractable diarrhoea, detected by immunohistochemical staining, responding to mast-cell targeted drugs. The question arises whether to search for MC infiltration in specific subsets of patients as a matter of routine clinical practice. Summary: A systematic electronic search of the English literature up to December 2017 was performed, using Medline, EMBASE, Web of Science, Scopus, and the Cochrane Library. This revealed 9 studies reporting an increased number of MC in the gut mucosa of patients with chronic diarrhoea. No consensus was found, however, on the actual cutoff point, the overlap in range between patients and controls being too great to be of clinical significance. The available evidence does not therefore justify the routine evaluation of MC count. Key Messages: More studies are needed to better define MC count and the significance of MC degranulation in normal and pathological settings. Until these become available, the search for MC infiltration in specific subsets of patients should be restricted to research settings.

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“…At present, it is unclear if this represents a true entity or simply a morphologic reaction pattern [115]. No predisposing factor has been identified and it is not associated with systemic mastocytosis or cutaneous mastocytosis and serum tryptase is normal [114].…”

Section: Mastocytic Enterocolitismentioning

confidence: 99%

Biopsy interpretation of colonic biopsies when inflammatory bowel disease is excluded

et al. 2012

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The interpretation of colonic biopsies related to inflammatory conditions can be challenging because the colorectal mucosa has a limited repertoire of morphologic responses to various injurious agents. Only few processes have specific diagnostic features, and many of the various histological patterns reflect severity and duration of the disease. Importantly the correlation with endoscopic and clinical information is often cardinal to arrive at a specific diagnosis in many cases.

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Mastocytic Enterocolitis: A Newly Described Mast Cell Entity

Cited by 13 publications

References 2 publications

Systemic Mastocytosis Presenting as Acute Appendicitis: A Case Report and Review of the Literature

Systemic Mastocytosis Presenting as Acute Appendicitis: A Case Report and Review of the Literature

Mastocytic Enterocolits and the Role of Mast Cells in Functional and Inflammatory Intestinal Disorders: A Systematic Review

Biopsy interpretation of colonic biopsies when inflammatory bowel disease is excluded

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