Cancer Treatment Today

Current classification and diagnosis of systemic mastocytosis, and its distinction from other myeloid malignancies associated with bone marrow mastocytosis, remain challenging for both clinicians and hematopathologists. World Health Organization (WHO) classification system for myeloid malignancies considers mast cell disease as a myeloproliferative neoplasm and systemic mastocytosis as a subcategory of mast cell disease with bone marrow involvement
Drug treatment of Mast Cell Activation Disease (MCAD) patients is highly individualized. Curative therapies are not avail-able, and each MCAD patient should be treated in accordance with his symptoms and complications. Moldering et al say: “Polychemotherapy including intensive induction regimens of the kind used in treating acute myeloid leukemia, as well as high-dose therapy with stem cell rescue, represent investigational approaches restricted to rare, selected patients.” There is a clinical trial: Stem Cell Transplantation to Treat Systemic Mastocytosis”.

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

Peter Valent, Wolfgang R. Sperr, and Cem Akin, How I treat patients with advanced systemic mastocytosis (Blood. 2010;116(26): 5812-5817)
Ustun C, Reiter A, Scott BL, Nakamura R, Damaj G, Kreil S, Shanley R, Hogan WJ, Perales MA, Shore T, Baurmann H, Stuart R, Gruhn B, Doubek M, Hsu JW, Tholouli E, Gromke T, Godley LA, Pagano L, Gilman A, Wagner EM, Shwayder T, Bornhäuser M, Papadopoulos EB, Böhm A, Vercellotti G, Van Lint MT, Schmid C, Rabitsch W, Pullarkat V, Legrand F, Yakoub-Agha I, Saber W, Barrett J, Hermine O, Hagglund H, Sperr WR, Popat U, Alyea EP, Devine S, Deeg HJ, Weisdorf D, Akin C, Valent P. Hematopoietic stem-cell transplantation for advanced systemic mastocytosis. J Clin Oncol. 2014 Oct 10;32(29):3264-74.

https://clinicaltrials.gov/ct2/show/NCT00006413