Efficacy of Omalizumab in Indolent Systemic Mastocytosis
Open accessAbstract
Background. Systemic mastocytosis (SM) comprises a heterogeneous group of disorders characterized by the proliferation of clonal mast cells in skin and various internal organs. Omalizumab is an established, labelled therapy for allergic asthma and chronic urticaria, but its experience in the efficacy of SM is limited. Methods. A retrospective analysis of 6 patients diagnosed with indolent SM treated with omalizumab at St. Michael’s Hospital between 2009 and 2018 is described. Reported frequency of anaphylaxis, baseline and follow-up tryptase levels, and SM-related symptoms were captured to measure the control of the disease. Results. Of the 5 patients who had experienced unprovoked anaphylaxis prior to treatment with omalizumab, 3 had no further episodes of anaphylaxis following initiation of omalizumab, while the remaining 2 patients had milder multisystem reactions. Significant improvement in cutaneous symptoms was also observed. Conclusion. These data suggest that omalizumab provides benefit to patients with SM who remain highly symptomatic in spite of treatment with conventional therapies.
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Response to additional drugs, including multitargeted tyrosine kinase inhibitors (in patients with SM, such as midostaurin) and anti-IgE therapy (omalizumab), may be considered an indirect sign of MCA or MCAS.
“Response to additional drugs, including multitargeted tyrosine kinase inhibitors (in patients with SM, such as midostaurin) and anti-IgE therapy (ie, omalizumab) may be considered an indirect sign of MCA or MCAS.”
From Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review (2021) · cites this paper as [84]
The Discussion (page not provided) reports that omalizumab suppressed anaphylaxis and other mast-cell-related symptoms in patients with indolent systemic mastocytosis, supporting the treatment-response portion of the claim. It does not say that response is an indirect sign of mast-cell activation or MCAS. It also states that midostaurin was unavailable to the patients, so the paper provides no evidence about their response to it. (LLM, conf=0.84)
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
Valent, P., Akin, C., Escribano, L., Födinger, M., Hartmann, K., Brockow, K., Castells, M., Sperr, W. R., Kluin‐Nelemans, H. C., Hamdy, N. A. T., et al. (2007) · European Journal of Clinical Investigation · doi:10.1111/j.1365-2362.2007.01807.x · Cited by 750 · see citing works
Omalizumab for the treatment of unprovoked anaphylaxis in patients with systemic mastocytosis
Carter, M. C., Robyn, J. A., Bressler, P. B., Walker, J. C., Shapiro, G. G., & Metcalfe, D. D. (2007) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2007.03.032 · Cited by 172 · see citing works
The serum tryptase test: an emerging robust biomarker in clinical hematology
Valent, P., Sperr, W. R., Sotlar, K., Reiter, A., Akin, C., Gotlib, J., Horny, H.-P., & Arock, M. (2014) · Expert Review of Hematology · doi:10.1586/17474086.2014.955008 · Cited by 83 · see citing works
Risk Factor Analysis of Anaphylactic Reactions in Patients With Systemic Mastocytosis
Gülen, T., Ljung, C., Nilsson, G., & Akin, C. (2017) · The Journal of Allergy and Clinical Immunology: In Practice · doi:10.1016/j.jaip.2017.02.008 · Cited by 83 · see citing works
Omalizumab therapy for mast cell-mediator symptoms in patients with ISM, CM, MMAS, and MCAS
Lemal, R., Fouquet, G., Terriou, L., Vaes, M., Livideanu, C. B., Frenzel, L., Barete, S., Canioni, D., Lhermitte, L., Rossignol, J., et al. (2019) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2019.03.039 · Cited by 70 · see citing works
Jagdis, A., & Vadas, P. (2014) · Annals of Allergy, Asthma & Immunology · doi:10.1016/j.anai.2014.05.001 · Cited by 28 · see citing works
Flagged first, then by citations
Research works printed but not linked: 23
- [1]Valent P. Akin C. Metcalfe D. D. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts Blood 2017 129 11 1420 1427 10.1182/blood-2016-09-731893 2-s2.0-85015822266 28031180 PMC5356454 · DOI
- [2]Pardanani A. Systemic mastocytosis in adults: 2019 update on diagnosis, risk stratification and management American Journal of Hematology 2019 94 3 363 377 10.1002/ajh.25371 2-s2.0-85059450949 30536695 · DOI
- [3]Castells M. Butterfield J. Mast cell activation syndrome and mastocytosis: initial treatment options and long-term management The Journal of Allergy and Clinical Immunology: In Practice 2019 7 4 1097 1106 10.1016/j.jaip.2019.02.002 2-s2.0-85063522698 30961835 · DOI · Paper page
- [4]Akin C. Molecular diagnosis of mast cell disorders The Journal of Molecular Diagnostics 2006 8 4 412 419 10.2353/jmoldx.2006.060022 2-s2.0-33748615147 16931579 PMC1867614 · DOI
- [5]Nagata H. Worobec A. S. Oh C. K. Identification of a point mutation in the catalytic domain of the protooncogene c-kit in peripheral blood mononuclear cells of patients who have mastocytosis with an associated hematologic disorder Proceedings of the National Academy of Sciences 1995 92 23 10560 10564 10.1073/pnas.92.23.10560 2-s2.0-0028856070 PMC40651 7479840 · DOI
- [6]Schwartz L. B. Irani A.-M. A. Serum tryptase and the laboratory diagnosis of systemic mastocytosis Hematology/Oncology Clinics of North America 2000 14 3 641 657 10.1016/s0889-8588(05)70300-2 2-s2.0-0033932870 10909044 · DOI
- [8]Matito A. Morgado J. M. Sánchez-López P. Management of anesthesia in adult and pediatric mastocytosis: a study of the Spanish network on mastocytosis (REMA) based on 726 anesthetic procedures International Archives of Allergy and Immunology 2015 167 1 47 56 10.1159/000436969 2-s2.0-84936976580 26160029 · DOI
- [10]Horny H.-P. Sotlar K. Valent P. Mastocytosis: state of the art Pathobiology 2007 74 2 121 132 10.1159/000101711 2-s2.0-34250796815 17587883 · DOI
- [12]Cohen S. S. Skovbo S. Vestergaard H. Epidemiology of systemic mastocytosis in Denmark British Journal of Haematology 2014 166 4 521 528 10.1111/bjh.12916 2-s2.0-84905042721 24761987 · DOI
- [13]van Doormal J. J. Arends S. Brunekreeft K. L. Prevalence of indolent systemic mastocytosis in a Dutch region The Journal of Allergy and Clinical Immunology 2013 131 1429 1431 23219169
- [14]Valent P. Sperr W. R. Akin C. How I treat patients with advanced systemic mastocytosis Blood 2010 116 26 5812 5817 10.1182/blood-2010-08-292144 2-s2.0-78650673033 20855864 · DOI
- [15]Siebenhaar F. Akin C. Bindslev-Jensen C. Maurer M. Broesby-Olsen S. Treatment strategies in mastocytosis Immunology and Allergy Clinics of North America 2014 34 2 433 447 10.1016/j.iac.2014.01.012 2-s2.0-84898783850 24745685 · DOI
- [16]Broesby-Olsen S. Vestergaard H. Mortz C. G. Omalizumab prevents anaphylaxis and improves symptoms in systemic mastocytosis: efficacy and safety observations Allergy 2018 73 1 230 238 10.1111/all.13237 2-s2.0-85026321881 28662309 · DOI · Paper page
- [18]Brown S. G. A. Clinical features and severity grading of anaphylaxis Journal of Allergy and Clinical Immunology 2004 114 2 371 376 10.1016/j.jaci.2004.04.029 2-s2.0-4344610304 15316518 · DOI
- [19]Slapnicar C. A. Smeenk C. S. Hicks L. Trinkaus M. Vadas P. Omalizumab and systemic mastocytosis: a single centre case series Journal of Allergy and Clinical Immunology 2019 143 2 AB182 10.1016/j.jaci.2018.12.557 · DOI
- [20]Yılmaz İ. Bahçecioğlu S. N. Türk M. Combination of omalizumab and bee venom immunotherapy: does it work? Asia Pacific Allergy 2018 8 1 10.5415/apallergy.2018.8.e2 PMC5796962 29423369 · DOI
- [21]Galera C. Soohun N. Zankar N. Caimmi S. Gallen C. Demoly P. Severe anaphylaxis to bee venom immunotherapy: efficacy of pretreatment and concurrent treatment with omalizumab Journal of Investigational Allergology & Clinical Immunology 2009 19 3 225 229 19610266
- [22]Molderings G. J. Raithel M. Kratz F. Omalizumab treatment of systemic mast cell activation disease: experiences from four cases Internal Medicine 2011 50 6 611 615 10.2169/internalmedicine.50.4640 2-s2.0-84857086336 21422688 · DOI
- [23]Pitt T. J. Cisneros N. Kalicinsky C. Becker A. B. Successful treatment of idiopathic anaphylaxis in an adolescent Journal of Allergy and Clinical Immunology 2010 126 2 415 416 10.1016/j.jaci.2010.05.043 2-s2.0-77955921599 20624653 · DOI
- [25]Kibsgaard L. Skjold T. Deleuran M. Vestergaard C. Omalizumab induced remission of idiopathic anaphylaxis in a patient suffering from indolent systemic mastocytosis Acta Dermato Venereologica 2014 94 3 363 364 10.2340/00015555-1687 2-s2.0-84899945914 24162824 · DOI
- [26]Jandus P. Hausmann O. Haeberli G. Gentinetta T. Mueller U. Helbling A. Unpredicted adverse reaction to omalizumab Journal of Investigational Allergology & Clinical Immunology 2011 21 7 563 566 22312942
- [27]Chang T. W. Chen C. Lin C.-J. Metz M. Church M. K. Maurer M. The potential pharmacologic mechanisms of omalizumab in patients with chronic spontaneous urticaria Journal of Allergy and Clinical Immunology 2015 135 2 337 342 10.1016/j.jaci.2014.04.036 2-s2.0-84922343034 24948369 · DOI
- [28]van Anrooij B. Kluin-Nelemans J. C. Safy M. Flokstra-de Blok B. M. J. Oude Elberink J. N. G. Patient-reported disease-specific quality-of-life and symptom severity in systemic mastocytosis Allergy 2016 71 11 1585 1593 10.1111/all.12920 2-s2.0-84977134477 27089859 · DOI
Cited by
Sorted by citations
Gülen, T., Akin, C., Bonadonna, P., Siebenhaar, F., Broesby-Olsen, S., Brockow, K., Niedoszytko, M., Nedoszytko, B., Oude Elberink, H. N. G., Butterfield, J. H., et al. (2021) · The Journal of Allergy and Clinical Immunology: In Practice · doi:10.1016/j.jaip.2021.06.011 · Cited by 83 · see citing works
“Response to additional drugs, including multitargeted tyrosine kinase inhibitors (in patients with SM, such as midostaurin) and anti-IgE therapy (ie, omalizumab) may be considered an indirect sign of MCA or MCAS.(80-88) Agents targeting growth and activation of neoplastic MCs, such as KIT-targeting kinase blockers, ar…”
Background
- Authors
- Slapnicar, C., Trinkaus, M., Hicks, L., & Vadas, P.
- Affiliations
- St. Michael's Hospital (CA)
- Corresponding author
- Vadas, Peter
- Venue
- Case Reports in Hematology
- Publisher
- Wiley
- Published
- 2019-09-16
- Volume
- 2019
- Pages
- 1-5
- ISSN
- 2090-6560 (ISSN-L)
- 2090-6579
- PMC
- PMC6766258
- OpenAlex
- W2974907868
- Cited by
- 16
- References
- 6
- Citations by year
- 2025: 4 · 2024: 1 · 2023: 2 · 2022: 3 · 2021: 4 · 2020: 2
- FWCI
- 0.90
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- Case Reports in Hematology (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- TSpace (University of Toronto) (submittedVersion, cc-by)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
- Health Sciences › Medicine › Rheumatology › Eosinophilic Disorders and Syndromes
- Health Sciences › Medicine › Gastroenterology › Gastrointestinal Tumor Research and Treatment
- Keywords
- omalizumab, indolent systemic mastocytosis, mast cell activation, anaphylaxis, tryptase, cutaneous manifestations, anti-IgE therapy
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review