Treatment of Mastocytosis

Currently, there is no cure for mastocytosis. However, there are options for treating the symptoms of this disease. Symptoms vary from person to person and can include itching, flushing, hiving, stomach pains, bone pains, and headache. The treatment options for the symptoms of mastocytosis include:

1   Identifying and avoiding triggers

2   Taking H1 antihistamines

3   Taking H2 antihistamines

4   Taking mast cell stabilizers

5   Taking epinephrine

6   Taking corticosteroids

7   Other treatments

Not all patients will require all of these types of medication. Please note that these treatments are considered options. They are compiled from journal articles and the experiences of people who have shared their child’s treatment plans. They do not represent the medical recommendations of Mastokids.Org. This organization recommends that you discuss treatment options with your physician.

Identifying and Avoiding Triggers

Because this disease is so individualized (that is, what may trigger a reaction of the mast cells in one person, may be harmless to someone else), it is very important to track a person’s reactions and identify potential triggers. To assist with this tracking, many people find it useful to keep a daily journal, citing possible triggers for the day’s mastocytosis-related reactions. Triggers can include food and specific ingredients, environmental factors, friction, and emotional distress. Once a person is aware of the specific triggers that cause his or her mast cells to degranulate, avoiding those triggers becomes a part of the treatment plan. To see a list of common triggers, check out the Degranulator List.

H1 Antihistamines

H1 antihistamines are drugs that are commonly used to treat the symptoms of itching, hiving and flushing. It can also bring some relief to stomach-related symptoms. Examples of H1 antihistamines include:

    Atarax (hydroxyzine HCl)*

    Zyrtec® (cetirizine HCl)

    Claritin® (loratadine)

    Benadryl (diphenhydramine HCl)*

    Allegra® (fexofenadine HCL)

    Chlor-Trimeton® (chloropheniramine maleate)

Note that Atarax and Zyrtec® are essentially the same drug. Atarax may cause drowsiness, whereas Zyrtec® usually does not. It is fine to use both, especially if the non-drowsy Zyrtec® is used in the morning and Atarax in the evening, but you and the doctor need to keep track of total dosage.

H2 Antihistamines

H2 antihistamines are drugs that relieve stomach-related symptoms associated with mastocytosis. These are ulcer or reflux medications. Examples of H2 antihistamines include:

    Zantac® (ranitidine HCl)*

    Pepcid® (famotidine)

* Syrup preparations of these medications may contain alcohol, a potential trigger for mastocytosis patients.

Mast Cell Stabilizers

While H1 and H2 antihistamines are drugs that block the action of histamine, mast cell stabilizers actually stabilize the mast cell itself, or prevent it from degranulating. Taken orally, mast cell stabilizers have been used to treat stomach and bone pains, headaches and cognitive functioning, as well as flushing. Taken topically, they have been used to treat itchiness and hiving. Inhaled, they have been used to treat lung-related symptoms such as excessive coughing.

    Cromolyn sodium

    Ketotifen (not available in the US, Ketotifen is also a potent H1 antihistamine)

Cromolyn sodium is a mast cell stabilizer that comes in many forms:

    Gastrocrom® (ampule): Can be mixed into a lotion and applied topically to relieve itching. This “Magic Masto Lotion” has been found to be very soothing by a number of mastocytosis patients.

    Nalcrom (powdered): Available in many countries.

    Intal (inhaler): Helps with lung symptoms such as excessive coughing. It can be used in a nebulizer as well.

    Nasalcrom (nasal spray): Helps with nasal congestion.

    Alocril (eye drop): Helps to relieve eye pain, itchiness, and irritation.

Epinephrine

According to the Hematology/Onocolgy Clinics of North America book on Mast Cell Disorders (authors: Dean Metcalfe, MD, NIH, and Nicholas Soter, MD), all mastocytosis patient should carry emergency medicines with them, including an EpiPen®. An EpiPen® is used in case of emergency to treat a patient who is in anaphylactic shock. .

Corticosteroids

Corticosteroids are used to manage malabsorption, ascites, and severe skin symptoms. An example of a corticosteroid is Prednisone.

Other Treatments

Other treatments include surgery to remove individual mastocytomas, and PUVA (psoralen plus ultraviolet A). PUVA is used to fade the appearance of pigmented lesions.

References

Alto W.A. and L. Clarcq. “Cutaneous and Systemic Manifestations of Mastocytosis.” American Family Physician, 1999.