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Chronic IBS and mastocystic enterocolitis

Question:

1/24/2018
I am caring for woman who has a 20-year history of chronic IBS and recently found to have mastocystic enterocolitis, > 150 MC/HPF, after coping with chronic hives for 2 years, with an unremarkable blood and urine chemistry work up for CIU (IGE 6.3 ku/ml, tryptase 5.2 ng/ml, plasma histamine 0.68, neg CU index. As a professional singer, she has now suffered recurrent vocal cord hemorrhage, after overuse of her voice and mold exposure in playhouse, and did not see any improvement with the use of histamine blockers (which her otolaryngologist prefers to d/c due to the dryness), saw no improvement of cromolyn and feels that she reports hair loss on oral cromolyn.

Three questions/thoughts: there seems to be two etiologies proposed for mastocytic enterocolitis- infectious or clonal mast cell disorder. Are there any new insights, data supporting etiology of increased MC burden in GI tract?

Are there any new thoughts on treatment options, given these two theories to help with management of this patient with mastocystic enterocolitis (still undergoing evaluation for clonal mastocytosis)?

When reviewing previous ask the expert questions about vocal cord dysfunction and mast cells, there was an implication of a somatic disorder, since the tryptase is normal. To my understanding, MC activation syndrome can be diagnosed with two or more tryptase levels, if the tryptase goes above baseline by 2 ng/ml + 20. As seen with hereditary angioedema, which was originally termed Hereditary angioneurotic edema, mast cell activation syndrome, with its myriad of signs and symptoms, including neuropsychiatric symptoms, can this ask the expert response be revisited? encouraging further evaluation of a possible mast cell disorder before labelling a psychiatric disorder and the consequential treatments that patients like this will then be relegated to?

Answer:

There are no criteria for pathologic GI mastocytosis based upon the number of mast cells. Other characteristics should be analyzed including clustering of mast cells, morphology of mast cells such as spindle shape, cKIT mutations and CD2 and CD25 markers. If the concern is that the patient has systemic mastocytosis with GI involvement, I would recommend a bone marrow biopsy with analysis of mast cells using the above, as specified in the reference below. Otherwise, I would question the diagnosis of 'mast cell enterocolitis'. There are reports of irritable bowel complaints with increased mast cell number but this is not systemic mastocytosis or a specific mast cell diseae (1,2,3). To my knowledge there is no specific therapy for this condition and likely would require symptomatic therapy as for irritable bowel syndrome. I would doubt that vocal cord hemorrhage is related to a mast cell disorder. The role of mold in this problem is also difficult to judge but seems unlikely to me.

I am not aware of no new thoughts on treatment options for mast cells in GI tract. There is question if mast cell number defines mast cell dependent GI pathology if other features of a systemic mast cell disease are not identified. I would consider therapies for irritable bowel syndrome.

Multiple somatic complaints associated with mast cells in the GI tract is not a mast cell activation disorder. The criteria of a tryptase change of 1.2 X baseline + 2 ng/ml is a statistical cut off that identifies mast cell activation but this could be from allergy rather than a mast cell disorder. Consultation at a mast cell clinic or with a mast cell expert may be of help but the bone marrow will be necessary. Urinary studies and mast cell activation, as defined by tryptase, are supportive findings but cannot suffice on their own (4,5).

In summary, I do not think your patient's problems are related to mast cell disease. I am not aware of any new therapies for mast cells in the GI tract and emphasize that mast cell number is not sufficient alone for the diagnosis of a GI mast cell disease. GI mast cell number may be a marker of irritable bowel syndrome. I would suggest treatment of GI complaints with therapies for irritable bowel syndrome. I would recommend bone marrow biopsy and utilization of specific criteria for diagnosing mast cell disease. Finally, I would continue to follow and offer as much symptomatic care and reassurance as possible. If new findings develop, these should be investigated.

1. Jakate S et al. Mastocytic enterocolitis: increased mucosal mast cells in chronic intractable diarrhea. ArchPathol Lab Med 2006;130:362-67.
2. Akhavein AM et al. Allergic mastocytic gastroenteritis and colitis: an unexplained etiology in chronic abdominal pain and gastrointestinal dysmotility. Gastroenterology Research and Practice 2012.
3. Martinez C et al. Diarrhea-predominant irritable bowel syndrome: an organic disorder with structural abnormalities in the jejunal epithelial barrier. Gut 2013;62:1160-68.
4. Akin C et al. mast cell activation syndrome: proposed diagnostic criteria. J Allergy Clin Immunol 2010;126:1099-1104.
5. Valent P et al. Definitions, criteria and global classification of mast cell disorders with special reference to mast activation sydnormes: a consensus proposal. Int Arch Allergy Immunol 2012;157:215-225.

I hope this information is of help to you and your patient.

All my best.

Dennis K. Ledford, MD, FAAAAI