From the Guidelines
Mastocytosis management should focus on symptom control, preventing mast cell activation, and avoiding known triggers, with a multidisciplinary approach involving allergists, dermatologists, and hematologists. The primary goal is to prevent mast cell activation during and in the immediate aftermath of surgical procedures or other triggering events 1.
Key Management Strategies
- First-line treatment includes H1 antihistamines (such as cetirizine 10mg daily or loratadine 10mg daily) to manage pruritus, flushing, and urticaria, along with H2 blockers (like ranitidine 150mg twice daily or famotidine 20mg twice daily) for gastrointestinal symptoms 1.
- For severe symptoms, oral cromolyn sodium (200mg four times daily) can stabilize mast cells.
- Patients should carry injectable epinephrine (EpiPen 0.3mg or 0.15mg for children) for anaphylactic reactions.
- Systemic corticosteroids may be used for short-term management of severe symptoms.
- For advanced systemic mastocytosis, tyrosine kinase inhibitors like midostaurin or avapritinib target the KIT D816V mutation.
Preventing Mast Cell Activation
- Avoid known triggers such as alcohol, extreme temperatures, certain medications (NSAIDs, opioids), and physical stress 1.
- Careful review of prior anesthetic records and identification/avoidance of known triggers of mast cell activation are critical.
- Temperature extremes (hypothermia or hyperthermia) and unnecessary trauma (eg, with patient positioning) that could lead to mast cell activation symptoms, skin blistering, or osteolytic fractures should be avoided in the operating room.
Monitoring and Multidisciplinary Care
- Regular monitoring of disease progression through serum tryptase levels, bone density scans, and complete blood counts is essential.
- Multidisciplinary care involving allergists, dermatologists, and hematologists provides comprehensive management for this complex condition where excessive mast cell accumulation leads to chronic inflammatory symptoms and potential organ damage 1.
Perioperative Management
- Pre-anesthetic treatment is probably helpful in reducing the frequency and/or severity of mast cell activation events, including the use of anxiolytic agents (eg, benzodiazepines), antihistamines (H1 and H2 blockers), and possibly corticosteroids 1.
- Certain perioperative drugs are considered safer, although the supporting data are anecdotal and not evidence-based, such as propofol, sevoflurane, or isoflurane for anesthesia, and fentanyl or remifentanil for analgesia.
- Agents to be avoided include the muscle relaxants atracurium and mivacurium, and succinylcholine, with caution exercised with opiates like codeine or morphine, as they can trigger mast cell activation 1.
From the FDA Drug Label
Cromolyn Sodium Oral Solution (Concentrate) is indicated in the management of patients with mastocytosis. Use of this product has been associated with improvement in diarrhea, flushing, headaches, vomiting, urticaria, abdominal pain, nausea, and itching in some patients. The management recommendations for patients with mastocytosis include the use of cromolyn sodium. Key benefits of this treatment may include:
- Improvement in diarrhea
- Improvement in flushing
- Improvement in headaches
- Improvement in vomiting
- Improvement in urticaria
- Improvement in abdominal pain
- Improvement in nausea
- Improvement in itching 2
From the Research
Management Recommendations for Mastocytosis
The management of mastocytosis involves a multi-faceted approach, including:
- Avoidance of factors triggering acute mediator release 3
- Treatment of acute mast cell mediator release 3, 4
- Treatment of chronic mast cell mediator release 3, 4
- Attempt to treat organ infiltration by mast cells 3
- Cytoreductive therapies for advanced variants, such as aggressive mastocytosis (ASM), MC leukemia (MCL), and MC sarcoma (MCS) 5, 6
Pharmacotherapy
Pharmacotherapy for mastocytosis includes:
- H1 and H2 antihistamines in combination or alone 4, 7, 6
- Cromolyn sodium 7
- Proton pump inhibitors 6
- Anti-leukotriene agents 6
- Injectable epinephrine for possible anaphylaxis 4, 6
- Cytoreductive agents, such as interferon alpha, glucocorticoids, and cladribine, for severe cases 6
- Polychemotherapeutic agents, such as daunomycin, etoposide, and 6-mercaptopurine, for severe cases 6
- Tyrosine kinase inhibitors, such as imatinib and midostaurine, for very specific and severe cases 6
Individualized Treatment
Treatment of mastocytosis should be individualized for each patient, considering the category of disease, reduction of risk of anaphylaxis, constitutional symptoms, and comorbidities, including osteoporosis 5