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Management of Mast Cell Activation Disorder (MCAD)

Unfortunately for healthcare providers and patients, there is no one single treatment that can be used to address MCAD. The main strategy is avoidance of identified triggers and allergens, such as insect venoms, temperature extremes, irritation, alcohol, or medications (i.e. aspirin, radiocontrast agents, certain anesthetic agents).1 It is possible that some patients may have no identifiable or reliable triggers, while other patients have variable tolerance patterns and avoidance lists.

Drug treatment is also highly individualized and specific to a patient’s disease, symptoms, and complications. As no tools yet exist to identify exactly which therapeutic regimen will be effective in treating your patient’s MCAD, a combination of tailored therapies may be utilized to optimize outcomes.

The form of MCAD your patient has will influence your long-term management of the disease. Mastocytosis, for example, has two forms, systemic and cutaneous, that vary in severity and may require different treatment strategies. It is important to set expectations and develop a treatment plan with the patient’s specific disease in mind.

Learn about identifying your patient's specific form of MCAD >

To assist you in selecting the most appropriate treatments, consider consulting with these emerging research concepts as resources:

 

 

“Regardless of the specific clinical presentation of MCAD, evidence-based therapy consists of trigger avoidance, antihistamines, and mast cell membrane-stabilizing compounds (basic therapy, supplemented as needed by medications targeting individual mast cell mediator-induced symptoms or complications.”1

Patient Education: Best When Combined with Specificity

Mast cell activation disorder may be capable of causing life-threatening anaphylactic reactions, making patient education a critical aspect of ongoing management. It is ideal to identify a patient’s triggers, or if they are reliable and repeated, so that patients can be educated and inspired to take a leading role in their care. For example, patients with a known sensitivity to Hymenoptera venom and a history of allergic reaction to bees and ants should then be informed of proper risk-reducing protocols to decrease the likelihood of a further reactions, such as the inclusion of venom immunotherapy (VIT). In most patients with a history of a reaction, VIT may induce protective measures against further, more severe reactions for 3-5 years.2

In other cases, the individual sensitizations and triggers may be unknown or unpredictable. For those patients, it is most helpful to identify the triggers. Then you can educate patients about the best ways to prevent a severe reaction or how to adhere to the most appropriate treatment plan, thereby enhancing outcomes.

Could your MACD patient have specific venom sensitizations?

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Follow Up, Reevaluation, and Further Testing

Even with a specific management plan in place, symptoms may recur. Regular follow-up visits can help detect this and give you the information needed to modify the treatment plan. You may find that a combination of therapies is needed to adequately reduce symptoms.

Patients with a history of anaphylaxis require the most vigilant maintenance. If anaphylaxis is initiated by a known allergen, especially that of Hymenoptera venom, you will likely want to consider immunotherapy and discuss the possibility with your patients during subsequent follow-up visits.2 If symptoms persist, or if you suspect additional allergies have developed, it may be helpful to conduct further testing.

Regular follow-up visits may also be necessary for patients with Cutaneous Mastocytosis (CM). This form of mastocytosis may develop into the more severe form of the disease, Systemic Mastocytosis (SM).3 The possibility of more severe MCAD should be examined further in patients with CM who present the following symptoms associated with SM:

  • Flushing
  • Headache
  • Trouble breathing (dyspnea)

 

  • Wheezing
  • Runny nose (rhinorrhea)
  • Nausea

 

  • Vomiting
  • Diarrhea
  • Fainting (syncope)

 

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References
  1. Molderings GJ, Brettner S, Homann J, et al. Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options. J Hematol Oncol. 2011;4:10.

  2. González de Olano D, Alvarez-Twose I, Esteban-López MI, et al. Safety and effectiveness of immunotherapy in patients with indolent systemic mastocytosis presenting with Hymenoptera venom anaphylaxis. J Allergy Clin Immunol. 2008;121(2):519-526.

  3. Castells, M, Metcalfe DD, and Escribano L. Guidelines for the Diagnosis and Treatment of Cutaneous Mastocytosis in Children. Am J Clin Dermatol. 2011;12(4):259-270.