Systemic Mastocytosis: Practice Essentials, Background, Pathophysiology (2024)

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  12. Ustun C, Corless CL, Savage N, et al. Chemotherapy and dasatinib induce long-term hematologic and molecular remission in systemic mastocytosis with acute myeloid leukemia with KIT D816V. Leuk Res. 2009 May. 33(5):735-41. [QxMD MEDLINE Link].

  13. Gotlib J, Kluin-Nelemans HC, George TI, Akin C, Sotlar K, Hermine O, et al. Efficacy and Safety of Midostaurin in Advanced Systemic Mastocytosis. N Engl J Med. 2016 Jun 30. 374 (26):2530-41. [QxMD MEDLINE Link]. [Full Text].

  14. FDA Approves Avapritinib for Advanced Systemic Mastocytosis. The ASCO Post. Available at https://ascopost.com/news/june-2021/fda-approves-avapritinib-for-advanced-systemic-mastocytosis/?utm_source=TAP%2DEN%2D061721%2DTrending%5FHeme&utm_medium=email&utm_term=046fca8ed7062b1f9d17ba521501b8c9?bc_md5=046fca8ed7062b1f9d17ba521501b8c9. June 17, 2021; Accessed: June 21, 2021.

  15. FDA Approves Avapritinib for Indolent Systemic Mastocytosis. The ASCO Post. Available at https://ascopost.com/news/may-2023/fda-approves-avapritinib-for-indolent-systemic-mastocytosis/?utm_source=TAP%2DEN%2D052323&utm_medium=email&utm_term=046fca8ed7062b1f9d17ba521501b8c9. May 23, 2023; Accessed: May 23, 2023.

  16. Pardanani A, Ketterling RP, Brockman SR, et al. CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy. Blood. 2003 Nov 1. 102(9):3093-6. [QxMD MEDLINE Link]. [Full Text].

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  17. Pieri L, Bonadonna P, Elena C, Papayannidis C, Grifoni FI, et al. Clinical presentation and management practice of systemic mastocytosis. A survey on 460 Italian patients. Am J Hematol. 2016 Apr 7. [QxMD MEDLINE Link].

  18. Ehrlich P. Beitrage zur Kenntnis der Anilinfarbungen und ihrer Verwendung in der mikroskopischen Technik. Arch mikr Anat. 1877. 13:263-7.

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  20. Kirshenbaum AS, Goff JP, Semere T, Foster B, Scott LM, Metcalfe DD. Demonstration that human mast cells arise from a progenitor cell population that is CD34(+), c-kit(+), and expresses aminopeptidase N (CD13). Blood. 1999 Oct 1. 94(7):2333-42. [QxMD MEDLINE Link].

  21. Mueller N, Wicklein D, Eisenwort G, Jawhar M, Berger D, Stefanzl G, et al. CD44 is a RAS/STAT5-regulated invasion receptor that triggers disease expansion in advanced mastocytosis. Blood. 2018 Jul 17. [QxMD MEDLINE Link].

  22. Ho CL, Kito H, Squillace DL, Lasho TL, Tefferi A. Clinical correlates of serum pro-major basic protein in a spectrum of eosinophilic disorders and myelofibrosis. Acta Haematol. 2008. 120(3):158-64. [QxMD MEDLINE Link].

  23. Jensen RT. Gastrointestinal abnormalities and involvement in systemic mastocytosis. Hematol Oncol Clin North Am. 2000 Jun. 14(3):579-623. [QxMD MEDLINE Link].

  24. Kirsch R, Geboes K, Shepherd NA, et al. Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases. Mod Pathol. 2008 Dec. 21(12):1508-16. [QxMD MEDLINE Link].

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  26. Schwaab J, Schnittger S, Sotlar K, Walz C, Fabarius A, Pfirrmann M, et al. Comprehensive mutational profiling in advanced systemic mastocytosis. Blood. 2013 Oct 3. 122 (14):2460-6. [QxMD MEDLINE Link].

  27. Tefferi A, Levine RL, Lim KH, Abdel-Wahab O, Lasho TL, Patel J, et al. Frequent TET2 mutations in systemic mastocytosis: clinical, KITD816V and FIP1L1-PDGFRA correlates. Leukemia. 2009 May. 23(5):900-4. [QxMD MEDLINE Link].

  28. Kondo R, Gleixner KV, Mayerhofer M, Vales A, Gruze A, Samorapoompichit P, et al. Identification of heat shock protein 32 (Hsp32) as a novel survival factor and therapeutic target in neoplastic mast cells. Blood. 2007 Jul 15. 110 (2):661-9. [QxMD MEDLINE Link].

  29. Ma Y, Zeng S, Metcalfe DD, et al. The c-KIT mutation causing human mastocytosis is resistant to STI571 and other KIT kinase inhibitors; kinases with enzymatic site mutations show different inhibitor sensitivity profiles than wild-type kinases and those with regulatory-type mutations. Blood. 2002 Mar 1. 99(5):1741-4. [QxMD MEDLINE Link]. [Full Text].

  30. Pardanani A, Elliott M, Reeder T, et al. Imatinib for systemic mast-cell disease. Lancet. 2003 Aug 16. 362(9383):535-6. [QxMD MEDLINE Link].

  31. Garcia-Montero AC, Jara-Acevedo M, Teodosio C, Sanchez ML, Nunez R, Prados A, et al. KIT mutation in mast cells and other bone marrow hematopoietic cell lineages in systemic mast cell disorders: a prospective study of the Spanish Network on Mastocytosis (REMA) in a series of 113 patients. Blood. 2006 Oct 1. 108(7):2366-72. [QxMD MEDLINE Link].

  32. Lim KH, Tefferi A, Lasho TL, et al. Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors. Blood. 2009 Jun 4. 113(23):5727-36. [QxMD MEDLINE Link].

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  33. Pardanani A, Lim KH, Lasho TL, et al. Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignancies. Blood. 2009 Oct 29. 114(18):3769-72. [QxMD MEDLINE Link].

  34. Jawhar M, Schwaab J, Álvarez-Twose I, et al. MARS: Mutation-Adjusted Risk Score for Advanced Systemic Mastocytosis. J Clin Oncol. 2019 Nov 1. 37 (31):2846-2856. [QxMD MEDLINE Link]. [Full Text].

  35. Butterfield JH. Survey of aspirin administration in systemic mastocytosis. Prostaglandins Other Lipid Mediat. 2009 Apr. 88(3-4):122-4. [QxMD MEDLINE Link].

  36. Mital A, Prejzner W, Hellmann A. Acquired von Willebrand syndrome during the course of systemic mastocytosis - analysis of 21 cases. Pol Arch Intern Med. 2018 Jul 11. [QxMD MEDLINE Link]. [Full Text].

  37. 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. Eur J Clin Invest. 2016 Feb 23. [QxMD MEDLINE Link].

  38. Donker ML, van Doormaal JJ, van Doormaal FF, Kluin PM, van der Veer E, de Monchy JG, et al. Biochemical markers predictive for bone marrow involvement in systemic mastocytosis. Haematologica. 2008 Jan. 93 (1):120-3. [QxMD MEDLINE Link]. [Full Text].

  39. Lueke AJ, Meeusen JW, Donato LJ, Gray AV, Butterfield JH, Saenger AK. Analytical and clinical validation of an LC-MS/MS method for urine leukotriene E4: A marker of systemic mastocytosis. Clin Biochem. 2016 Feb 18. 264 (1):217-22. [QxMD MEDLINE Link].

  40. Gilreath JA, Tchertanov L, Deininger MW. Novel approaches to treating advanced systemic mastocytosis. Clin Pharmacol. 2019. 11:77-92. [QxMD MEDLINE Link]. [Full Text].

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  42. 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. Eur J Clin Invest. 2016 Feb 23. [QxMD MEDLINE Link].

  43. Hahn HP, Hornick JL. Immunoreactivity for CD25 in gastrointestinal mucosal mast cells is specific for systemic mastocytosis. Am J Surg Pathol. 2007 Nov. 31 (11):1669-76. [QxMD MEDLINE Link].

  44. Arber DA, Orazi A, Hasserjian RP, et al. International Consensus Classification of Myeloid Neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data. Blood. 2022 Sep 15. 140 (11):1200-1228. [QxMD MEDLINE Link]. [Full Text].

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  49. Slapnicar C, Trinkaus M, Hicks L, Vadas P. Efficacy of Omalizumab in Indolent Systemic Mastocytosis. Case Rep Hematol. 2019. 2019:3787586. [QxMD MEDLINE Link]. [Full Text].

  50. McComish JS, Slade CA, Buizen L, et al. Randomized controlled trial of omalizumab in treatment-resistant systemic and cutaneous mastocytosis (ROAM). J Allergy Clin Immunol Pract. 2023 Apr 23. [QxMD MEDLINE Link]. [Full Text].

  51. [Guideline] Sacta MA, Alsaati N, Spergel J. Anaphylaxis: A 2023 practice parameter update: Major changes in management of anaphylaxis. Ann Allergy Asthma Immunol. 2024 Feb. 132 (2):109-110. [QxMD MEDLINE Link]. [Full Text].

  52. van der Veer E, van der Goot W, de Monchy JG, Kluin-Nelemans HC, van Doormaal JJ. High prevalence of fractures and osteoporosis in patients with indolent systemic mastocytosis. Allergy. 2012 Mar. 67(3):431-8. [QxMD MEDLINE Link].

  53. Radia D, Deininger M, Gotlib J, et al. Avapritinib, a potent and selective inhibitor of KIT D816V, induces complete and durable responses in patients (pts) with advanced systemic mastocytosis (AdvSM). European Hematology Association. Available at https://library.ehaweb.org/eha/2019/24th/267413/deepti.radia.avapritinib.a.potent.and.selective.inhibitor.of.kit.d816v.induces.html?f=listing%3D3%2Abrowseby%3D8%2Asortby%3D1%2Amedia%3D1. June 15, 2019; Accessed: June 22, 2020.

  54. Gotlib J, Castells M, Elberink HO, Siebenhaar F, Hartmann K, Broesby-Olsen S, et al. Avapritinib versus placebo in indolent systemic mastocytosis. NEJM Evid. 2023 May 23. 2(6):[Full Text].

  55. Lee HJ. Recent advances in diagnosis and therapy in systemic mastocytosis. Blood Res. 2023 Apr 30. 58 (S1):96-108. [QxMD MEDLINE Link]. [Full Text].

  56. Valent P, Sperr WR, Akin C. How I treat patients with advanced systemic mastocytosis. Blood. 2010 Dec 23. 116 (26):5812-7. [QxMD MEDLINE Link]. [Full Text].

  57. Pardanani A. Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management. Am J Hematol. 2021 Apr 1. 96 (4):508-525. [QxMD MEDLINE Link]. [Full Text].

  58. [Guideline] National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Systemic Mastocytosis Version 1.2023. Available at https://www.nccn.org/professionals/physician_gls/pdf/mastocytosis.pdf. May 24, 2023; Accessed: May 28, 2023.

  59. Gruson B, Lortholary O, Canioni D, Chandesris O, Lanternier F, Bruneau J, et al. Thalidomide in systemic mastocytosis: results from an open-label, multicentre, phase II study. Br J Haematol. 2013 May. 161 (3):434-42. [QxMD MEDLINE Link].

  60. Yacoub A, Prochaska L. Ruxolitinib improves symptoms and quality of life in a patient with systemic mastocytosis. Biomark Res. 2016. 4:2. [QxMD MEDLINE Link]. [Full Text].

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  62. Ustun C, Reiter A, Scott BL, et al. Hematopoietic stem-cell transplantation for advanced systemic mastocytosis. J Clin Oncol. 2014 Oct 10. 32 (29):3264-74. [QxMD MEDLINE Link]. [Full Text].

  63. Chaar CI, Bell RL, Duffy TP, Duffy AJ. Guidelines for safe surgery in patients with systemic mastocytosis. Am Surg. 2009 Jan. 75(1):74-80. [QxMD MEDLINE Link].

  64. Alvarez-Twose I, Matito A, Morgado JM, Sánchez-Muñoz L, Jara-Acevedo M, García-Montero A, et al. Imatinib in systemic mastocytosis: a phase IV clinical trial in patients lacking exon 17 KIT mutations and review of the literature. Oncotarget. 2017 Sep 15. 8 (40):68950-68963. [QxMD MEDLINE Link].

Systemic Mastocytosis: Practice Essentials, Background, Pathophysiology (2024)

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