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Refractory Malignant Mastocytosis

MedGen UID:
1734281
•Concept ID:
C5418815
•
Neoplastic Process

Definition

Malignant mastocytosis that is resistant to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Malignant Mastocytosis

Professional guidelines

PubMed

Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management.
Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312
Systemic mastocytosis in adults: 2019 update on diagnosis, risk stratification and management.
Pardanani A
Am J Hematol 2019 Mar;94(3):363-377. Epub 2019 Jan 2 doi: 10.1002/ajh.25371. PMID: 30536695

Recent clinical studies

Etiology

Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management.
Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
Systemic mastocytosis in adults: 2019 update on diagnosis, risk stratification and management.
Pardanani A
Am J Hematol 2019 Mar;94(3):363-377. Epub 2019 Jan 2 doi: 10.1002/ajh.25371. PMID: 30536695
Endocrine manifestations of systemic mastocytosis in bone.
Greene LW, Asadipooya K, Corradi PF, Akin C
Rev Endocr Metab Disord 2016 Sep;17(3):419-431. doi: 10.1007/s11154-016-9362-3. PMID: 27239674
Systemic mastocytosis in adults: 2015 update on diagnosis, risk stratification, and management.
Pardanani A
Am J Hematol 2015 Mar;90(3):250-62. doi: 10.1002/ajh.23931. PMID: 25688753
Indolent systemic mastocytosis: a case and review of the current available treatment options.
Lee Y, Wood P, Soyer HP
Dermatol Online J 2013 Jun 15;19(6):18562. PMID: 24011312

Diagnosis

Safety and efficacy of lirentelimab in patients with refractory indolent systemic mastocytosis: a first-in-human clinical trial.
Siebenhaar F, Altrichter S, Bonnekoh H, Hawro T, Hawro M, Michaelis EG, Kantor AM, Chang AT, Youngblood BA, Singh B, Rasmussen HS, Maurer M
Br J Dermatol 2023 Oct 25;189(5):511-519. doi: 10.1093/bjd/ljad191. PMID: 37290787
Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management.
Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
Mast Cell Activation Syndrome: A Primer for the Gastroenterologist.
Weinstock LB, Pace LA, Rezaie A, Afrin LB, Molderings GJ
Dig Dis Sci 2021 Apr;66(4):965-982. Epub 2020 Apr 23 doi: 10.1007/s10620-020-06264-9. PMID: 32328892
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312
Pharmacotherapy of mast cell disorders.
Gülen T, Akin C
Curr Opin Allergy Clin Immunol 2017 Aug;17(4):295-303. doi: 10.1097/ACI.0000000000000377. PMID: 28570344

Therapy

Safety and efficacy of lirentelimab in patients with refractory indolent systemic mastocytosis: a first-in-human clinical trial.
Siebenhaar F, Altrichter S, Bonnekoh H, Hawro T, Hawro M, Michaelis EG, Kantor AM, Chang AT, Youngblood BA, Singh B, Rasmussen HS, Maurer M
Br J Dermatol 2023 Oct 25;189(5):511-519. doi: 10.1093/bjd/ljad191. PMID: 37290787
Tyrosine kinase inhibitors for the treatment of indolent systemic mastocytosis: Are we there yet?
Akin C, Arock M, Valent P
J Allergy Clin Immunol 2022 Jun;149(6):1912-1918. Epub 2022 Apr 26 doi: 10.1016/j.jaci.2022.04.020. PMID: 35487307
Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management.
Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312
Postural Tachycardia Syndrome (POTS) and the GI Tract: A Primer for the Gastroenterologist.
DiBaise JK, Harris LA, Goodman B
Am J Gastroenterol 2018 Oct;113(10):1458-1467. Epub 2018 Aug 3 doi: 10.1038/s41395-018-0215-4. PMID: 30072778

Prognosis

Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management.
Pardanani A
Am J Hematol 2021 Apr 1;96(4):508-525. Epub 2021 Feb 21 doi: 10.1002/ajh.26118. PMID: 33524167
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312
Systemic mastocytosis in adults: 2019 update on diagnosis, risk stratification and management.
Pardanani A
Am J Hematol 2019 Mar;94(3):363-377. Epub 2019 Jan 2 doi: 10.1002/ajh.25371. PMID: 30536695
Pharmacotherapy of mast cell disorders.
Gülen T, Akin C
Curr Opin Allergy Clin Immunol 2017 Aug;17(4):295-303. doi: 10.1097/ACI.0000000000000377. PMID: 28570344
Endocrine manifestations of systemic mastocytosis in bone.
Greene LW, Asadipooya K, Corradi PF, Akin C
Rev Endocr Metab Disord 2016 Sep;17(3):419-431. doi: 10.1007/s11154-016-9362-3. PMID: 27239674

Clinical prediction guides

Safety and efficacy of lirentelimab in patients with refractory indolent systemic mastocytosis: a first-in-human clinical trial.
Siebenhaar F, Altrichter S, Bonnekoh H, Hawro T, Hawro M, Michaelis EG, Kantor AM, Chang AT, Youngblood BA, Singh B, Rasmussen HS, Maurer M
Br J Dermatol 2023 Oct 25;189(5):511-519. doi: 10.1093/bjd/ljad191. PMID: 37290787
Successful treatment with Omalizumab of a child affected by Systemic Mastocytosis: clinical and biological implications.
Bossi G, Brazzelli V, De Amici M, Pietra D, Raviola C, Naso M, Regalbuto C, Boselli F, Fortina V, Marseglia GL
Ital J Pediatr 2023 Jan 13;49(1):6. doi: 10.1186/s13052-022-01402-7. PMID: 36639823Free PMC Article
New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis.
Reiter A, George TI, Gotlib J
Blood 2020 Apr 16;135(16):1365-1376. doi: 10.1182/blood.2019000932. PMID: 32106312
Pharmacotherapy of mast cell disorders.
Gülen T, Akin C
Curr Opin Allergy Clin Immunol 2017 Aug;17(4):295-303. doi: 10.1097/ACI.0000000000000377. PMID: 28570344
The immunoglobulin E- and calcium-dependent release of histamine and eicosanoids from human dispersed mastocytosis spleen cells.
Robinson C, Benyon RC, Agius RM, Jones DB, Wright DH, Holgate ST
J Invest Dermatol 1988 Mar;90(3):359-65. doi: 10.1111/1523-1747.ep12456379. PMID: 2450144

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