Review and updates on systemic mastocytosis and related entities

Li, Julie Y.; Ryder, Christopher B.; Zhang, Hailing; Cockey, Samuel G.; Hyjek, Elizabeth; Moscinski, Lynn C.; Sagatys, Elizabeth; Song, Jinming

doi:10.3390/cancers15235626

Cited by 31Mast cells and histamineGastrointestinal Tumor Research and TreatmentHistiocytic Disorders and Treatments

Review and updates on systemic mastocytosis and related entities

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Li, Julie Y., Ryder, Christopher B., Zhang, Hailing, Cockey, Samuel G., Hyjek, Elizabeth, Moscinski, Lynn C., Sagatys, Elizabeth, Song, Jinming·2023·Cancers

DOI 10.3390/cancers15235626PMC PMC10705510

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Abstract

Mast cell disorders range from benign proliferations to systemic diseases that cause anaphylaxis and other diverse symptoms to mast cell neoplasms with varied clinical outcomes. Mastocytosis is the pathologic process of the accumulation of abnormal mast cells in different organs, mostly driven by KIT mutations, and can present as cutaneous mastocytosis, systemic mastocytosis (SM), and mast cell sarcoma. The WHO 5th edition classification divides systemic mastocytosis into bone marrow mastocytosis, indolent systemic mastocytosis, smoldering systemic mastocytosis, aggressive systemic mastocytosis, systemic mastocytosis with an associated hematologic neoplasm, and mast cell leukemia. The new ICC classifies SM slightly differently. The diagnosis of SM requires the integration of bone marrow morphologic, immunophenotypic, and molecular findings, as well as clinical signs and symptoms. Moreover, understanding the wide range of clinical presentations for patients with mast cell disorders is necessary for accurate and timely diagnosis. This review provides an updated overview of mast cell disorders, with a special emphasis on SM, including the latest approaches to diagnosis, prognostic stratification, and management of this rare disease.

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Severe episodes were twice more frequent in individuals with both SM and HαT than in those with SM but without HαT.
“severe episodes were twice more frequent in individuals with both SM and HαT than in those with SM but without HαT (17, 18).”
From Defining “Normal” basal serum tryptase levels: a context-dependent approach to improve diagnostics in systemic mastocytosis (2025) · cites this paper as [18]
Section 2.2 (page not available) says concurrent HαT predisposes patients with mastocytosis to severe mediator-related symptoms. It does not report that severe episodes are twice as frequent in patients with both SM and HαT as in patients with SM alone. (LLM, conf=0.84)
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Avapritinib for Systemic Mastocytosis
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Mastocytosis: Imaging Spectrum and Diagnostic Insights
Khot, R., Diab, M., Jing, J. M., Gao, M., Elbanna, A., Rupe, E. S., Mahmoud, H. S., Klekers, A. R., Gaballah, A. H., & Elsayes, K. M. (2025) · Radiographics · doi:10.1148/rg.250075 · Cited by 1 · see citing works
Multiphasic anaphylaxis in the emergency and intensive care setting
Schneider, M. A., de Groot, H., & Geerts, J. W. H. J. (2026) · BMJ Case Reports · doi:10.1136/bcr-2025-270996 · Cited by 1 · see citing works
Characterization of CD123 expression by mast cells in systemic mastocytosis with multicolor flow cytometry
Shean, R. C., George, T. I., & Ng, D. P. (2026) · Cytometry Part B Clinical Cytometry · doi:10.1002/cyto.b.70023 · Cited by 1 · see citing works
Histamine and Pruritus: From Molecular Mechanisms to Clinical Therapy
Lan, Y., Guo, J., Yao, M., Liao, Z.-R., & Jing, Y. (2026) · Cell Biochemistry and Function · doi:10.1002/cbf.70199 · Cited by 1 · see citing works
Treatment-refractory chronic urticaria with neutrophilia and elevated inflammatory markers
Sivaloganathan, D. M., Smith, R. M., & Saini, S. S. (2026) · Allergy and Asthma Proceedings · doi:10.2500/aap.2026.47.260065 · Cited by 1 · see citing works
Lesiones óseas blásticas, una forma de presentación infrecuente de mastocitosis sistémica
Manchado-Reinoso, M. D. M., Rodríguez, B. C., & de la Puebla-Giménez, R. Á. F. (2025) · Revista Española de Casos Clínicos en Medicina Interna · doi:10.32818/reccmi.a10n1a11
Selecting optimal therapy in systemic mastocytosis: current state and future directions
Ike, I., Samperio, V. M., & Dasanu, C. A. (2025) · Expert Opinion on Pharmacotherapy · doi:10.1080/14656566.2025.2503847
Mastocitosis sistémica indolente con respuesta a fototerapia en una paciente con otras neoplasias no hematológicas: un reto terapéutico
Santamaría-Domínguez, D. G., Pérez-Rubio, J., Osorio-Aragón, I. Y., Vega‐Memije, M. E., & Lacy‐Niebla, R. M. (2025) · Revista Medicina Cutánea Ibero-Latino-Americana · doi:10.24875/mcut.24000087
KIT‐Negative Systemic Mastocytosis Associated With Acute Myeloid Leukemia
Ali, K., Rashid, T., Miatech, J., Karan, A., Chandler, Z. D., Garcia, G. D., & Quan, W. J. (2025) · Case Reports in Oncological Medicine · doi:10.1155/crom/3563591
AXL mediates mast cell survival and resistance to tyrosine kinase inhibitors in mastocytosisPreprint
Kangboonruang, K., Drabent, P., Maksut, F., Heintzé, M., Lepelletier, Y., Lhermitte, L., Féroul, M., Letard, S., Kabore, C., Brenet, F. A., et al. (2025) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2025.11.03.686205
Aggiornamenti sull'utilizzo della triptasi alla luce delle nuove conoscenze dell'alfa-triptasemia ereditaria
Platzgummer, S., SIPMeL, A. N. D. G.-A., Onida, R., Bruno, L., Caponi, L., Anzivino, P., Conte, M., ZANONI, G., Ghisellini, S., Deleonardi, G., et al. (2026) · La Rivista Italiana della Medicina di Laboratorio - Italian Journal of Laboratory Medicine · doi:10.23736/s1825-859x.25.00312-3
Aggressive systemic mastocytosis presenting as diffuse osteoblastic bone disease: a diagnostic pitfall mimicking metastatic cancer
Melício, A., Gonçalves, H. D., Dias, M., Vasconcelos-Teixeira, S., & Lopes, J. M. (2026) · European Journal of Case Reports in Internal Medicine · doi:10.12890/2026_006143
If It Is Not Immunoglobulin E-Mediated Allergy, What Is It?
Johnston, S. L., & Madrigal‐Burgaleta, R. (2026) · British Journal of Hospital Medicine · doi:10.31083/bjhm52244
Clinical Spectrum of Drug Hypersensitivity Reactions in Systemic Mastocytosis: Drug-Induced Anaphylaxis as a Unique Clinical Presentation
Aslan, E., Okan, K., Kural, R. F., İnan, S., Özeke, Y., Ateş, Ü., Yıldırım, O., Galata, Z., Kırdok, K., Ay, E., et al. (2026) · Medicina · doi:10.3390/medicina62040711
Modern approaches to the diagnosis of alpha-tryptasemia
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肥満細胞性白血病に対する化学療法開始直後に難治性アナフィラキシーショックを来した1例(A case of refractory anaphylactic shock immediately following initiation of chemotherapy for mast cell leukemia)
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Unmasking Indolent Systemic Mastocytosis in Patients with Unexplained or Treatment-Refractory Osteoporosis: A Case Series with Diagnostic and Therapeutic Implications
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Bone Marrow
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The Clinical and Molecular Characteristics of Systemic Mastocytosis with Associated Non-Mast Cell Myeloid Neoplasm
Seth, N., & Deb, P. Q. (2026) · Journal of Clinical Medicine · doi:10.3390/jcm15187232
More Than Just a Gut Feeling: Unmasking Systemic Mastocytosis Behind Chronic Gastrointestinal Symptoms in a 2-Patient Case Series
Ramírez, G., Khoudari, A., Gonzalez, Y., & Davis-Yadley, A. H. (2026) · ACG Case Reports Journal · doi:10.14309/crj.0000000000002310
From Non-Advanced Disease to Mast Cell Leukemia: Diagnostic, Morphologic, and Molecular Heterogeneity of Systemic Mastocytosis in a Single-Center Real-World Series
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Recent advances in diagnosis and therapy in systemic mastocytosis
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Mastocytosis: Pathophysiology, Clinical Manifestations, and Current Therapeutic Approaches – A Narrative Review
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Systemic mastocytosis: a (slightly) simplified diagnostic approach
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Place of cytology in the diagnosis of systemic mastocytosis: A case report
Quiddi, W., Boumaazi, H., Eddyb, S., Yahyaoui, H., Aitameur, M., & Chakour, M. (2021) · GSC Advanced Research and Reviews · doi:10.30574/gscarr.2021.7.2.0096
Systemic mastocytosis in adults — current classification, pathogenesis, comprehensive diagnostics, and therapeutic strategies
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Authors: Li, J. Y., Ryder, C. B., Zhang, H., Cockey, S. G., Hyjek, E., Moscinski, L. C., Sagatys, E., & Song, J.
Affiliations: Moffitt Cancer Center (US)
University of South Florida (US)
Corresponding author: Li, Julie Y.
Song, Jinming
ORCID: Li, Julie Y.
Ryder, Christopher B.
Zhang, Hailing
Cockey, Samuel G.
Hyjek, Elizabeth
Moscinski, Lynn C.
Sagatys, Elizabeth
Song, Jinming
Venue: Cancers
Publisher: MDPI AG
Published: 2023-11-28
Volume: 15
Issue: 23
Pages: 5626
ISSN: 2072-6694 (ISSN-L)
DOI: 10.3390/cancers15235626
PMC: PMC10705510
OpenAlex: W4389144191
Cited by: 31
References: 138
Citations by year: 2026: 15 · 2025: 14 · 2024: 2
FWCI: 0.86
Type: journal article
Language: en
Open access: gold
doi.org
Licence: cc-by
creativecommons.org/licenses/by/4.0/ (vor)
Locations: Cancers (publishedVersion, cc-by)
PubMed Central (publishedVersion, cc-by)
PubMed (publishedVersion)
Topics: Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
Health Sciences › Medicine › Gastroenterology › Gastrointestinal Tumor Research and Treatment
Health Sciences › Medicine › Physiology › Histiocytic Disorders and Treatments
Keywords: systemic mastocytosis, mast cell disease, KIT mutations, mast cell neoplasms, mast cell sarcoma, mast cell leukemia, WHO classification, bone marrow mastocytosis, indolent systemic mastocytosis, aggressive systemic mastocytosis, anaphylaxis, diagnosis, prognostic stratification

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