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Systemic mastocytosis — Clinical Guidelines

Overview

Systemic mastocytosis is a rare disorder characterized by clonal expansion and accumulation of mast cells in various organs, leading to diverse clinical manifestations due to mediator release and tissue infiltration 1.

Diagnosis

Bone marrow biopsy: Essential for diagnosis, evaluating mast cell infiltration and KIT D816V mutation status 4.

Serum tryptase levels: Elevated levels support the diagnosis 4.

Histological evaluation: Centralized review by specialized pathologists improves diagnostic accuracy compared to local evaluations 4.

Endoscopic findings: Characteristic "urticaria-like" lesions in the gastric mucosa can aid diagnosis 11.

Management

First-line treatments:

- Corticosteroids: Used for symptom control and managing acute crises (e.g., prednisolone) 5. - H1/H2 receptor antagonists: For managing allergic reactions and mediator release 5.

Adjunctive treatments:

- Interferon-alpha: Not effective in the case described; efficacy varies 5. - Radiotherapy: Considered for refractory bone pain 6 7. - Anesthetic precautions: Special care required during procedures due to potential mediator release 2.

Special Populations

Pregnancy: Anesthetic management requires heightened vigilance due to potential mast cell activation from labor stress and drug sensitivities 2.

Elderly: Management focuses on symptom control and minimizing triggers, with radiotherapy considered for refractory symptoms 6 7.

Key Recommendations

Refer patients to specialized centers with expertise in mastocytosis for comprehensive multidisciplinary care (Evidence: Expert opinion 1).

Utilize centralized bone marrow evaluation by specialized pathologists to enhance diagnostic accuracy (Evidence: Moderate 4).

Employ corticosteroids and H1/H2 receptor antagonists for acute symptoms and anaphylaxis management (Evidence: Moderate 5).

Consider radiotherapy for refractory bone pain in systemic mastocytosis (Evidence: Weak 6 7).

Exercise caution and tailor anesthetic approaches carefully in pregnant patients with systemic mastocytosis (Evidence: Expert opinion 2).

References

1 Gotlib J, Gerds AT, Abdelmessieh P, Ali H, Castells M, Dunbar A et al.. NCCN Guidelines® Insights: Systemic Mastocytosis, Version 3.2024. Journal of the National Comprehensive Cancer Network : JNCCN 2024. link 2 Fernandes AL, Mendonça M, Costa D, Freitas SC, Pereira SM. Anaesthesia in a parturient with systemic mastocytosis. Revista espanola de anestesiologia y reanimacion 2022. link 3 Valdivielso Cortázar E, Yáñez López JÁ, Veiga Barreiro JA. Endoscopically diagnosed systemic mastocytosis. Revista espanola de enfermedades digestivas 2019. link 4 Jawhar M, Schwaab J, Horny HP, Sotlar K, Naumann N, Fabarius A et al.. Impact of centralized evaluation of bone marrow histology in systemic mastocytosis. European journal of clinical investigation 2016. link 5 Chen G, Chen L, Qin X, Xie X, Li G, Xu B. Systemic mastocytosis with recurrent anaphylactic shock and multiple organ dysfunction failure. Clinical laboratory 2015. link 6 Johnstone PA, Mican JM, Metcalfe DD, DeLaney TF. Radiotherapy of refractory bone pain due to systemic mast cell disease. American journal of clinical oncology 1994. link 7 Janjan NA, Conway P, Lundberg J, Derfus G. Radiation therapy in a case of systemic mastocytosis: evaluation of histamine levels and mucosal effects. American journal of clinical oncology 1992. link 8 Sumpio BE, O'Leary G, Gusberg RJ. Variceal bleeding, hypersplenism, and systemic mastocytosis. Pathophysiology and management. Archives of surgery (Chicago, Ill. : 1960) 1988. link 9 Schwartz LB, Foley JV, Austen KF, Soter NA, Shepard R, Murphy GF. Localization of tryptase to human cutaneous mast cells and keratinocytes by immunofluorescence and immunoperoxidase cytochemistry with monoclonal antitryptase antibody. The Journal of allergy and clinical immunology 1985. link90699-2) 10 Johansson O, Nordlind K. Immunohistochemical localization of somatostatin-like immunoreactivity in skin lesions from patients with urticaria pigmentosa. Virchows Archiv. B, Cell pathology including molecular pathology 1984. link 11 Borda F, Uribarrena R, Rivero-Puente A. Gastroscopic findings in systemic mastocytosis. Endoscopy 1983. link 12 Forni M, Klatt EC, Shaw ST, Taylor CR, Lukes RJ, Meyer PR. Immunohistochemical characterization of reactive and neoplastic mast cells. American journal of clinical pathology 1983. link 13 Meneghini CL, Angelini G. Systemic mastocytosis with diffuse crocodile-like pachydermic skin, pedunculated pseudofibromas and comedones. The British journal of dermatology 1980. link

Systemic mastocytosis