Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options

Molderings, Gerhard J.; Brettner, Stefan; Homann, Jürgen; Afrin, Lawrence B.

doi:10.1186/1756-8722-4-10

Cited by 120 publications

(122 citation statements)

References 49 publications

(52 reference statements)

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“…If patient meet all three criteria, MCA is diagnosed [9,13] (see Figures 3 and 4). , [6] published in Biomed Central)…”

Section: Discussionmentioning

confidence: 99%

“…[5] Later on, it was found and accepted that among the MC mediators, tryptase, a serine proteinase enzyme, is a sensitive and specific marker for mast cell activation in mastocytosis. [6] It was thought that mast cell disease exist in two forms, cutaneous and systemic, and are caused by abnormal mast cell proliferation. However, since its discovery there have been reported cases of MC-mediated symptoms with normal or only slightly elevated tryptase levels and without cutaneous evidence of mast cell disease, and not meeting the criteria for systemic mastocytosis on bone marrow evaluation.…”

Section: Introductionmentioning

confidence: 99%

“…However, since its discovery there have been reported cases of MC-mediated symptoms with normal or only slightly elevated tryptase levels and without cutaneous evidence of mast cell disease, and not meeting the criteria for systemic mastocytosis on bone marrow evaluation. [6,7] In 2010, Akin et al proposed new diagnostic criteria for mast cell activation disorders (MCAD) which includes clonal cutaneous and systemic mastocytosis as well as clonal and non-clonal mast cell activation syndromes (MCAS). [8,9] Symptoms of MCAS can occur at any age.…”

Section: Introductionmentioning

confidence: 99%

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Mast cell activation disorder masquerading as a nervous breakdown

Nguyen

Teuber

2017

CRIM

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While mast cells have been implicated in human disease since the early 1900s, mast cell activation syndrome (MCAS) was only recently discovered and recognized as a disorder caused by aberrant release of mast cell mediator enzymes. These mediators trigger a wide variety of symptoms ranging from headache, pruritis and gastrointestinal discomfort to life-threatening anaphylaxis. MCAS is frequently unrecognized and misdiagnosed because symptoms associated with MCAS are often present in other medical conditions and are highly variable among patients. We describe a case of a 62-year-old male who presented to the emergency department (ED) with three days of recurrent flushing, diarrhea, mild edema of his hands bilaterally, and severe emotional lability with recurrent crying. In the ED, he was misdiagnosed with severe anxiety and directly sent to a mental health clinic for assistance. However, he was previously seen in allergy clinic for suspected MCAS. Fortuitously standing labs including plasma histamine, tryptase, and urine studies that were ordered in allergy clinic were drawn concurrently with the initial ED workup. He was found to have elevated histamine level during his ED episode and treatment for MCAS was initiated. In subsequent visits, his symptoms, except for episodes of "brain fog" and emotional lability, improved significantly on a combination of antihistamines, oral cromolyn sodium, and montelukast. Additionally every other day prednisone has substantially cleared the neurocognitive symptoms. This case highlights the difficulty of MCAS diagnosis and the importance of assessing for MCAS, especially in patients who have multisystem complaints.

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“…If patient meet all three criteria, MCA is diagnosed [9,13] (see Figures 3 and 4). , [6] published in Biomed Central)…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Mast cell activation disorder masquerading as a nervous breakdown

Nguyen

Teuber

2017

CRIM

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“…Signs of improvement in symptoms with any specific therapy may be seen within four weeks. 8 Specifically with respect to our case, it is possible that more frequent dosing of histamine type 1 (H1) blockers, combined H1/H2 receptor blockade, or more frequent dosing of montelukast could have led to improved control of her symptoms.…”

Section: O N O T C O P Ymentioning

confidence: 99%

“…Options include histamine I and II blockers (i.e., diphenhydramine, cetirizine, loratadine, and ranitidine), leukotriene receptor antagonists (i.e., montelukast), and mast cell membrane-stabilizing medications (i.e., cromolyn sodium) and avoiding triggers. 7,8 The general approach involves adding medications sequentially. Signs of improvement in symptoms with any specific therapy may be seen within four weeks.…”

Section: O N O T C O P Ymentioning

confidence: 99%

A 60-year-old woman with recurrent episodes of flushing, urticaria, and angioedema

Patel

Baldwin

2015

allergy asthma proc

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Recurrent episodes of flushing, urticaria, and angioedema raise suspicion for many conditions with a wide differential diagnosis. The diagnostic approach involves consideration of allergic, cardiovascular, gastrointestinal, endocrine, infectious, neurologic, dermatologic, and drug-related A 60 year-old woman with recurrent episodes of flushing, urticaria, and angioedema presented to the allergy clinic for follow-up after an emergency department (ED) visit for "total body flushing" about an hour after eating wheat crackers, cheddar cheese, and decaffeinated tea. She had previously presented to a local ED with "flushing" involving her arms, legs, torso, neck, and face. She denied gastrointestinal symptoms at that time, but she reported previous similar episodes that were associated with abdominal discomfort and diarrhea. In the ED, she was treated with epinephrine, diphenhydramine, and steroids with resolution of her symptoms.Her medical history included asthma, fibromyalgia, osteoarthritis, migraine headaches, hypothyroidism, and hyperlipidemia. Her medications included albuterol as needed, sumatriptan, fexofenadine, levothyroxine, and fluoxetine. Her allergy history included hives when exposed to tetracycline, gastrointestinal upset, and a syncopal episode when exposed to smells of eggs and popcorn and local swelling to stinging insects. PHYSICAL EXAMINATIONShe was a comfortable-appearing woman. Her vital signs were temperature 36.7°C, heart rate 68 beats per minute, blood pressure 120/72 mm Hg, and respiratory rate 16 breaths per minute. Head, eyes, ears, nose, and throat exam was unremarkable. Lungs were clear to auscultation bilaterally. Cardiac exam revealed a regular rate with no murmurs. Skin exam revealed no flushing, urticaria, angioedema, eczema rashes, dermatographism, or urticaria pigmentosa lesions. The remainder of the physical examination was unremarkable.

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Mast cell disorders in Ehlers–Danlos syndrome

Seneviratne¹,

Maitland²,

Afrin³

2017

American J of Med Genetics Pt C

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Well known for their role in allergic disorders, mast cells (MCs) play a key role in homeostatic mechanisms and surveillance, recognizing and responding to different pathogens, and tissue injury, with an array of chemical mediators. After being recruited to connective tissues, resident MCs progenitors undergo further differentiation, under the influence of signals from surrounding microenvironment. It is the differential tissue homing and local maturation factors which result in a diverse population of resident MC phenotypes. An abundance of MC reside in connective tissue that borders with the external world (the skin as well as gastrointestinal, respiratory, and urogenital tracts). Situated near nerve fibers, lymphatics, and blood vessels, as well as coupled with their ability to secrete potent mediators, MCs can modulate the function of local and distant structures (e.g., other immune cell populations, fibroblasts, angiogenesis), and MC dysregulation has been implicated in immediate and delayed hypersensitivity syndromes, neuropathies, and connective tissue disorders (CTDs). This report reviews basic biology of mast cells and mast cell activation as well as recent research efforts, which implicate a role of MC dysregulation beyond atopic disorders and in a cluster of Ehlers-Danlos Syndromes, non-IGE mediated hypersensitivity disorders, and dysautonomia.

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Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options

Cited by 120 publications

References 49 publications

Mast cell activation disorder masquerading as a nervous breakdown

Mast cell activation disorder masquerading as a nervous breakdown

A 60-year-old woman with recurrent episodes of flushing, urticaria, and angioedema

Mast cell disorders in Ehlers–Danlos syndrome

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