Research & Literature

H1-antihistamines for primary mast cell activation syndromes: a systematic review.

Nurmatov UB, Rhatigan E, Simons FE, Sheikh A

Allergy 2015; (70(9)):1052-61 doi:10.1111/all.12672.

Additional mutations in SRSF2, ASXL1 and/or RUNX1 identify a high-risk group of patients with KIT D816V(+) advanced systemic mastocytosis.

Jawhar M, Schwaab J, Schnittger S, et al.

Leukemia 2016; (30(1)):136-43 doi:10.1038/leu.2015.284.

Bone marrow tryptase as a possible diagnostic criterion for adult systemic mastocytosis.

Bulai Livideanu C, Apoil PA, Lepage B, et al.

Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology 2016; (46(1)):133-41 doi:10.1111/cea.12627.

Impact of centralized evaluation of bone marrow histology in systemic mastocytosis.

Jawhar M, Schwaab J, Horny HP, et al.

European journal of clinical investigation 2016; (46(5)):392-7 doi:10.1111/eci.12607.

Treatment of CD30-positive systemic mastocytosis with brentuximab vedotin.

Borate U, Mehta A, Reddy V, et al.

Leukemia research 2016; (44()):25-31.

Clinical presentation and management practice of systemic mastocytosis. A survey on 460 Italian patients.

Pieri L, Bonadonna P, Elena C, et al.

American journal of hematology 2016; (91(7)):692-9 doi:10.1002/ajh.24382.

Next-generation sequencing in systemic mastocytosis: Derivation of a mutation-augmented clinical prognostic model for survival.

Pardanani A, Lasho T, Elala Y, et al.

American journal of hematology 2016; (91(9)):888-93 doi:10.1002/ajh.24426.

Advanced systemic mastocytosis: from molecular and genetic progress to clinical practice.

Ustun C, Arock M, Kluin-Nelemans HC, et al.

Haematologica 2016; (101(10)):1133-1143 doi:10.3324/haematol.2016.146563.

Advances in the understanding and clinical management of mastocytosis and clonal mast cell activation syndromes.

González-de-Olano D, Matito A, Orfao A, Escribano L

F1000Research 2016; (5()):2666 doi:10.12688/f1000research.9565.1.

Pathogenesis and Pathology of Mastocytosis.

Metcalfe DD, Mekori YA

Annual review of pathology 2017; (12()):487-514 doi:10.1146/annurev-pathol-052016-100312.

Insights in Anaphylaxis and Clonal Mast Cell Disorders.

González-de-Olano D, Álvarez-Twose I

Frontiers in immunology 2017; (8()):792 doi:10.3389/fimmu.2017.00792.

Midostaurin treatment in FLT3-mutated acute myeloid leukemia and systemic mastocytosis.

Kayser S, Levis MJ, Schlenk RF

Expert review of clinical pharmacology 2017; (10(11)):1177-1189 doi:10.1080/17512433.2017.1387051.

Mastocytosis - pathogenesis, clinical manifestation and treatment.

Wagner N, Staubach P

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2018; (16(1)):42-57 doi:10.1111/ddg.13418.

Tyrosine Kinase Inhibition in Mastocytosis: KIT and Beyond KIT.

Bibi S, Arock M

Immunology and allergy clinics of North America 2018; (38(3)):527-543 doi:10.1016/j.iac.2018.04.007.

c-KIT-Positive Fatal Diffuse Cutaneous Mastocytosis With Systemic Manifestations in a Neonate.

Chaudhary N, Shapiro N, Bhutada A, Rastogi S

Journal of pediatric hematology/oncology 2019; (41(5)):e338-e340 doi:10.1097/MPH.0000000000001271.

FDA Approval Summary: Midostaurin for the Treatment of Advanced Systemic Mastocytosis.

Kasamon YL, Ko CW, Subramaniam S, et al.

The oncologist 2018; (23(12)):1511-1519 doi:10.1634/theoncologist.2018-0222.

Cytogenetic abnormalities in systemic mastocytosis: WHO subcategory-specific incidence and prognostic impact among 348 informative cases.

Shah S, Pardanani A, Elala YC, et al.

American journal of hematology 2018; (93(12)):1461-1466 doi:10.1002/ajh.25265.

Omalizumab Therapy for Mast Cell-Mediator Symptoms in Patients with ISM, CM, MMAS, and MCAS.

Lemal R, Fouquet G, Terriou L, et al.

The journal of allergy and clinical immunology. In practice 2019; (7(7)):2387-2395.e3 doi:10.1016/j.jaip.2019.03.039.

Getting personal with myelodysplastic syndromes: is now the right time?

Chokr N, Pine AB, Bewersdorf JP, et al.

Expert review of hematology 2019; (12(4)):215-224 doi:10.1080/17474086.2019.1592673.

Relevant updates in systemic mastocytosis.

Coltoff A, Mascarenhas J

Leukemia research 2019; (81()):10-18 doi:10.1016/j.leukres.2019.04.001.

CD30 expression in cutaneous lesions of systemic mastocytosis: clinical, biological and histopathological analysis of 27 patients.

Poirier E, Fraitag S, Tezenas du Montcel S, et al.

Journal of the European Academy of Dermatology and Venereology : JEADV 2019; (33(9)):e344-e347 doi:10.1111/jdv.15633.

Molecular quantification of tissue disease burden is a new biomarker and independent predictor of survival in mastocytosis.

Greiner G, Gurbisz M, Ratzinger F, et al.

Haematologica 2020; (105(2)):366-374 doi:10.3324/haematol.2019.217950.

Molecular genetics in allogeneic blood stem cell transplantation for myelodysplastic syndromes.

Kobbe G, Schroeder T, Rautenberg C, et al.

Expert review of hematology 2019; (12(10)):821-831 doi:10.1080/17474086.2019.1645004.

MARS: Mutation-Adjusted Risk Score for Advanced Systemic Mastocytosis.

Jawhar M, Schwaab J, Álvarez-Twose I, et al.

Journal of clinical oncology : official journal of the American Society of Clinical Oncology 2019; (37(31)):2846-2856 doi:10.1200/JCO.19.00640.

Successful treatment of idiopathic mast cell activation syndrome with low-dose Omalizumab.

Berry R, Hollingsworth P, Lucas M

Clinical & translational immunology 2019; (8(10)):e01075 doi:10.1002/cti2.1075.

Endoscopically diagnosed systemic mastocytosis.

Valdivielso Cortázar E, Yáñez López JÁ, Veiga Barreiro JA

Revista espanola de enfermedades digestivas 2019; (111(11)):892 doi:10.17235/reed.2019.6246/2019.

Evolution of myelodisplasic syndrome with ring sideroblasts (MDS-RS) to systemic mastocytosis and aleukemic mast cell leukemia.

Costa LD, Azambuja AP, Ângelo Lisboa ES, et al.

Hematology, transfusion and cell therapy 2020; (42(4)):387-389 doi:10.1016/j.htct.2019.08.003.

Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification.

Trizuljak J, Sperr WR, Nekvindová L, et al.

Allergy 2020; (75(8)):1927-1938 doi:10.1111/all.14248.

Mast Cell Activation Syndrome: A Primer for the Gastroenterologist.

Weinstock LB, Pace LA, Rezaie A, et al.

Digestive diseases and sciences 2021; (66(4)):965-982 doi:10.1007/s10620-020-06264-9.

Accurate Diagnosis and Prognosis in Systemic Mastocytosis: The Role of Mutational Analysis.

Weiler CR, Akin C

The journal of allergy and clinical immunology. In practice 2020; (8(9)):3128-3129 doi:10.1016/j.jaip.2020.06.030.

Acute aleukemic mast cell leukemia: Report of a case and review of the literature.

Galura GM, Cherukuri SV, Hakim N, et al.

Leukemia research reports 2020; (14()):100230 doi:10.1016/j.lrr.2020.100230.

A rare manifestation of indolent systemic mastocytosis and its management during the coronavirus disease 2019 pandemic; educational lessons from Syria.

Slaibi A, Alshehabi Z, Soltany A, et al.

Annals of medicine and surgery (2012) 2021; (62()):293-297 doi:10.1016/j.amsu.2021.01.062.

Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management.

Pardanani A

American journal of hematology 2021; (96(4)):508-525 doi:10.1002/ajh.26118.

Genetic Regulation of Tryptase Production and Clinical Impact: Hereditary Alpha Tryptasemia, Mastocytosis and Beyond.

Sprinzl B, Greiner G, Uyanik G, et al.

International journal of molecular sciences 2021; (22(5)) doi:10.3390/ijms22052458.

Response Criteria in Advanced Systemic Mastocytosis: Evolution in the Era of KIT Inhibitors.

Shomali W, Gotlib J

International journal of molecular sciences 2021; (22(6)) doi:10.3390/ijms22062983.

Bone Marrow Mastocytosis: A Diagnostic Challenge.

Zanotti R, Tanasi I, Bernardelli A, et al.

Journal of clinical medicine 2021; (10(7)) doi:10.3390/jcm10071420.

Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis.

Zanotti R, Bonifacio M, Lucchini G, et al.

Leukemia 2022; (36(2)):516-524 doi:10.1038/s41375-021-01406-y.

Systemic Mastocytosis: Advances in Diagnosis and Current Management.

Pullarkat ST, Wu W, Pullarkat V

Cancer treatment and research 2021; (181()):167-178 doi:10.1007/978-3-030-78311-2_10.

Association of systemic mastocytosis with primary cutaneous marginal zone lymphoma; first case.

Günay MB, Büyükbabani N, Yavuz AS, et al.

Journal of the European Academy of Dermatology and Venereology : JEADV 2022; (36(4)):e275-e276 doi:10.1111/jdv.17797.

Safety and efficacy of avapritinib in advanced systemic mastocytosis: the phase 1 EXPLORER trial.

DeAngelo DJ, Radia DH, George TI, et al.

Nature medicine 2021; (27(12)):2183-2191 doi:10.1038/s41591-021-01538-9.

Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal.

Valent P, Akin C, Hartmann K, et al.

HemaSphere 2021; (5(11)):e646 doi:10.1097/HS9.0000000000000646.

Midostaurin therapy for advanced systemic mastocytosis: Mayo Clinic experience in 33 consecutive cases.

Singh A, Al-Kali A, Begna KH, et al.

American journal of hematology 2022; (97(5)):630-637 doi:10.1002/ajh.26498.

Standards of Genetic Testing in the Diagnosis and Prognostication of Systemic Mastocytosis in 2022: Recommendations of the EU-US Cooperative Group.

Hoermann G, Sotlar K, Jawhar M, et al.

The journal of allergy and clinical immunology. In practice 2022; (10(8)):1953-1963 doi:10.1016/j.jaip.2022.03.001.

Nontryptase Urinary and Hematologic Biomarkers of Mast Cell Expansion and Mast Cell Activation: Status 2022.

Butterfield JH

The journal of allergy and clinical immunology. In practice 2022; (10(8)):1974-1984 doi:10.1016/j.jaip.2022.03.008.

Idiopathic mast cell activation syndrome is more often suspected than diagnosed-A prospective real-life study.

Buttgereit T, Gu S, Carneiro-Leão L, et al.

Allergy 2022; (77(9)):2794-2802 doi:10.1111/all.15304.

Diagnosis and treatment of systemic mastocytosis in Brazil: Recommendations of a multidisciplinary expert panel.

Velloso EDRP, Padulla GA, de Cerqueira AMM, et al.

Hematology, transfusion and cell therapy 2022; (44(4)):582-594 doi:10.1016/j.htct.2022.04.006.

Standards of Pathology in the Diagnosis of Systemic Mastocytosis: Recommendations of the EU-US Cooperative Group.

Sotlar K, George TI, Kluin P, et al.

The journal of allergy and clinical immunology. In practice 2022; (10(8)):1986-1998.e2 doi:10.1016/j.jaip.2022.05.036.

Target Therapies for Systemic Mastocytosis: An Update.

Sciumè M, De Magistris C, Galli N, et al.

Pharmaceuticals (Basel, Switzerland) 2022; (15(6)) doi:10.3390/ph15060738.

Immunohistochemical Staining to Identify Concomitant Systemic Mastocytosis in Acute Myeloid Leukemia with RUNX1::RUNX1T1.

Hwang SM, Kim BJ, Lee JS, et al.

Annals of laboratory medicine 2022; (42(6)):678-682 doi:10.3343/alm.2022.42.6.678.

Systemic Mastocytosis and Other Entities Involving Mast Cells: A Practical Review and Update.

El Hussein S, Chifotides HT, Khoury JD, et al.

Cancers 2022; (14(14)) doi:10.3390/cancers14143474.

Perceptions of patient disease burden and management approaches in systemic mastocytosis: Results of the TouchStone Healthcare Provider Survey.

Mesa RA, Sullivan EM, Dubinski D, et al.

Cancer 2022; (128(20)):3700-3708 doi:10.1002/cncr.34421.

Patient-reported outcomes among patients with systemic mastocytosis in routine clinical practice: Results of the TouchStone SM Patient Survey.

Mesa RA, Sullivan EM, Dubinski D, et al.

Cancer 2022; (128(20)):3691-3699 doi:10.1002/cncr.34420.

The international consensus classification of mastocytosis and related entities.

Leguit RJ, Wang SA, George TI, et al.

Virchows Archiv : an international journal of pathology 2023; (482(1)):99-112 doi:10.1007/s00428-022-03423-3.

Mastocytosis and related entities: a practical roadmap.

Beyens M, Elst J, van der Poorten ML, et al.

Acta clinica Belgica 2023; (78(4)):325-335 doi:10.1080/17843286.2022.2137631.

Successful treatment with Omalizumab of a child affected by Systemic Mastocytosis: clinical and biological implications.

Bossi G, Brazzelli V, De Amici M, et al.

Italian journal of pediatrics 2023; (49(1)):6 doi:10.1186/s13052-022-01402-7.

Comprehensive mastocytosis data analysis from a single center.

Tiryaki TO, Özkan SG, Erdem S, et al.

BMC cancer 2023; (23(1)):82 doi:10.1186/s12885-022-10498-3.

Hereditary alpha-tryptasemia and complete deletion of exon 8 of the c-kit gene in patients with mast cell activation syndrome.

Jiang M, Vadas P

Leukemia & lymphoma 2023; (64(7)):1348-1351 doi:10.1080/10428194.2023.2203286.

Indolent systemic mastocytosis and aleukemic mast cell leukemia: Subtle diagnostic differences with distinct management approaches.

Paiva ML, Yumeen S, Saliba E, DiMarco C

JAAD case reports 2023; (36()):63-66 doi:10.1016/j.jdcr.2023.04.009.

The international consensus classification of eosinophilic disorders and systemic mastocytosis.

Wang SA, Orazi A, Gotlib J, et al.

American journal of hematology 2023; (98(8)):1286-1306 doi:10.1002/ajh.26966.

Avapritinib treatment of KIT D816V-mutant atypical chronic myeloid leukemia.

Sandow L, Heinrich M

Leukemia research reports 2023; (19()):100371 doi:10.1016/j.lrr.2023.100371.

KIT Mutations and Other Genetic Defects in Mastocytosis: Implications for Disease Pathology and Targeted Therapies.

Chantran Y, Valent P, Arock M

Immunology and allergy clinics of North America 2023; (43(4)):651-664 doi:10.1016/j.iac.2023.04.008.

Management of Advanced Systemic Mastocytosis and Associated Myeloid Neoplasms.

Tashi T, Deininger MW

Immunology and allergy clinics of North America 2023; (43(4)):723-741 doi:10.1016/j.iac.2023.04.009.

Aggressive systemic mastocytosis with multiple organ involvement: a case report.

Iranijam E, Salimi M, Negaresh M, Javanshir N

Oxford medical case reports 2023; (2023(11)):omad095 doi:10.1093/omcr/omad095.

Review and Updates on Systemic Mastocytosis and Related Entities.

Li JY, Ryder CB, Zhang H, et al.

Cancers 2023; (15(23)) doi:10.3390/cancers15235626.

Urticaria pigmentosa and systemic mastocytosis.

Keow J, Chin-Yee B, Hsia CC, Robertson K

Clinical case reports 2023; (11(12)):e8302 doi:10.1002/ccr3.8302.

Avapritinib versus Placebo in Indolent Systemic Mastocytosis.

Gotlib J, Castells M, Elberink HO, et al.

NEJM evidence 2023; (2(6)):EVIDoa2200339 doi:10.1056/EVIDoa2200339.

Comparison between indolent systemic mastocytosis and clonal mast cell disease not meeting WHO diagnostic criteria: A nationwide multicenter retrospective analysis.

Sciumè M, Serpenti F, Zanotti R, et al.

Hematological oncology 2024; (42(3)):e3277 doi:10.1002/hon.3277.

How I diagnose systemic mastocytosis.

Rets AV, George TI

American journal of clinical pathology 2024; (162(4)):332-348 doi:10.1093/ajcp/aqae047.

NCCN Guidelines® Insights: Systemic Mastocytosis, Version 3.2024.

Gotlib J, Gerds AT, Abdelmessieh P, et al.

Journal of the National Comprehensive Cancer Network : JNCCN 2024; (22(2 D)).

Systemic Mastocytosis: State of the Art.

Farmer I, Radia DH

Current hematologic malignancy reports 2024; (19(5)):197-207 doi:10.1007/s11899-024-00737-8.

Management of Advanced Systemic Mastocytosis: Clinical Challenges.

Tremblay D, Wagner NE, Mascarenhas J

Journal of blood medicine 2024; (15()):421-433 doi:10.2147/JBM.S366367.

Clinical-genomic profiling of MDS to inform allo-HCT: recommendations from an international panel on behalf of the EBMT.

Gurnari C, Robin M, Adès L, et al.

Blood 2025; (145(18)):1987-2001 doi:10.1182/blood.2024025131.

High-sensitivity KIT D816V variation analysis by droplet digital polymerase chain reaction: The reference laboratory perspective.

Shean RC, Hellwig S, Saadalla A, et al.

American journal of clinical pathology 2025; (164(2)):145-149 doi:10.1093/ajcp/aqaf008.

Mastocytosis.

Akin C, Arock M, Carter MC, et al.

Nature reviews. Disease primers 2025; (11(1)):30 doi:10.1038/s41572-025-00611-8.

New treatments for systemic mastocytosis in 2025.

Costanzo G, Marzio V, Cavaglià E, et al.

Current opinion in allergy and clinical immunology 2025; (25(4)):277-292 doi:10.1097/ACI.0000000000001079.

Patient-Reported Outcomes and Provider Perceptions of Systemic Mastocytosis: Results From the PRISM Study.

Triggiani M, Hobart J, Alvarez-Twose I, et al.

Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology 2025; (55(9)):784-794 doi:10.1111/cea.70101.

Management of indolent mastocytosis and mast cell activation syndrome: A clinical yardstick.

Akin C, Butterfield JH, Castells M, Lyons JJ

Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology 2025; (135(4)):466-477 doi:10.1016/j.anai.2025.06.032.

Real-world characteristics of systemic mastocytosis in Romania: insights from a reference-center-based descriptive study.

Soare D, Soare D, Petruțescu B, et al.

Journal of medicine and life 2025; (18(7)):640-647 doi:10.25122/jml-2025-0103.

Diagnosing Systemic Mastocytosis: State of the Art.

Rets A, George TI

International journal of laboratory hematology 2026; (48(3)):551-560 doi:10.1111/ijlh.70011.

Management of skeletal-related events and fracture prevention in systemic mastocytosis.

Lunn EM, Lacount S, Greene LW, Asadipooya K

Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA 2026; (37(3)):605-619 doi:10.1007/s00198-025-07726-4.

Management of Mastocytosis and Mast Cell Activation in Children.

Carter MC, Lange M, Alvarez-Twose I, et al.

The journal of allergy and clinical immunology. In practice 2026; (14(1)):30-42 doi:10.1016/j.jaip.2025.11.016.

Mastocytosis: Imaging Spectrum and Diagnostic Insights.

Khot R, Diab M, Jing JM, et al.

Radiographics : a review publication of the Radiological Society of North America, Inc 2026; (46(1)):e250075 doi:10.1148/rg.250075.