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Journal articles on the topic 'Advance systemic mastocytosis'

Author: Grafiati

Published: 24 May 2025

Last updated: 31 July 2025

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1

Arock, Michel. "A new therapeutic advance for symptomatic systemic mastocytosis?" Lancet 389, no. 10069 (2017): 576–78. http://dx.doi.org/10.1016/s0140-6736(16)31655-5.

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2

Lee, Philina, Tracy I. George, Hongliang Shi, et al. "Systemic Mastocytosis Patient Experience from Mast Cell Connect, the First Patient-Reported Registry for Mastocytosis." Blood 128, no. 22 (2016): 4783. http://dx.doi.org/10.1182/blood.v128.22.4783.4783.

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Abstract Background: Systemic mastocytosis (SM) is a rare mast cell disorder associated with a range of debilitating symptoms and a shortage of effective treatment options. Little is known about the impact of the disease from the patients' perspective. Systematically characterizing the natural history of SM and its impact on patients will facilitate the development of new therapies. Registries that engage patients have proven valuable in other rare diseases by expanding knowledge of current treatment approaches, evaluating disease burden and accelerating clinical trials. Methods: Mast Cell Con

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3

Deininger, Michael W., Daniel J. DeAngelo, Deepti H. Radia, et al. "Effective Control of Advance Systemic Mastocytosis with Avapritinib: Mutational Analysis from the Explorer Clinical Study." Blood 138, Supplement 1 (2021): 318. http://dx.doi.org/10.1182/blood-2021-150872.

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Abstract Introduction: Systemic mastocytosis (SM) is a rare, hematologic neoplasm driven by KIT D816V mutations in ~95% of patients. Outcomes remain poor for patients with advanced SM (AdvSM), which comprises aggressive SM (ASM), SM with an associated hematologic neoplasm (SM-AHN), and mast cell leukemia (MCL). Molecular subtyping reveals a heterogeneous genetic landscape, particularly in patients with an AHN component. The KIT D816V mutation is invariably found in and considered a primary driver of the neoplastic mast cell component, while its role in the AHN component is less clear. Clinical

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4

Pardanani, Animesh, Rhett P. Ketterling, Stephanie R. Brockman, 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 102, no. 9 (2003): 3093–96. http://dx.doi.org/10.1182/blood-2003-05-1627.

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AbstractImatinib mesylate is effective in the treatment of hematologic malignancies that are characterized by either abl- or PDGFRβ- activating mutations. The drug is also active in a subset of patients with eosinophilic disorders and systemic mast cell disease (SMCD). Recently, a novel tyrosine kinase that is generated from fusion of the Fip1-like 1 (FIP1L1) and PDGFRα (PDGFRA) genes has been identified as a therapeutic target for imatinib mesylate in hypereosinophilic syndrome (HES). We used fluorescence in situ hybridization (FISH) to detect deletion of the CHIC2 locus at 4q12 as a surrogat

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5

Shikhbabaeva, D. I., O. Yu Vinogradova, A. L. Neverova, et al. "Targeted therapy for advanced forms of systemic mastocytosis in real clinical practice." Oncohematology 18, no. 4 (2023): 78–89. http://dx.doi.org/10.17650/1818-8346-2023-18-4-78-89.

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Background. Mastocytosis is a heterogeneous group of diseases that are characterized by excessive proliferation and accumulation of clonal (neoplastic) mast cells in one or more organs. Advanced variants of systemic mastocytosis (aggressive systemic mastocytosis, systemic mastocytosis associated with hematological neoplasm, and mast cell leukemia) are characterized by infiltration of organs by mast cells, which leads to organs dysfunction. Such patients require a more active approach and the use of cytoreductive therapy. Available therapeutic options include imatinib, interferon-alpha, cladrib

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6

Veitch, Scott, and Deepti H. Radia. "Recent Advances in the Therapeutic Management of Advanced Systemic Mastocytosis." Diagnostics 14, no. 1 (2023): 80. http://dx.doi.org/10.3390/diagnostics14010080.

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Advanced systemic mastocytosis (AdvSM) is a rare haematological neoplasm characterised by the accumulation of neoplastic mast cells (MCs) in various organs, resulting in organ dysfunction and reduced life expectancy. The subtypes include aggressive SM (ASM), SM with an associated haematological neoplasm (SM-AHN) and mast cell leukaemia (MCL). The gain of function KIT D816V mutation is present in most cases. The availability of tyrosine kinase inhibitors (TKIs) has revolutionised the treatment landscape for patients with this life-limiting disease. Patients are now able to achieve molecular rem

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7

Chandesris, Marie-Olivia, Gandhi Damaj, Danielle Canioni, et al. "Midostaurin in Advanced Systemic Mastocytosis." New England Journal of Medicine 374, no. 26 (2016): 2605–6. http://dx.doi.org/10.1056/nejmc1515403.

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8

Shomali, William, and Jason Gotlib. "The new tool “KIT” in advanced systemic mastocytosis." Hematology 2018, no. 1 (2018): 127–36. http://dx.doi.org/10.1182/asheducation-2018.1.127.

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AbstractMastocytosis is a rare disease characterized by KIT-driven expansion and accumulation of neoplastic mast cells in various tissues. Although mediator symptoms related to mast cell activation can impose a symptom burden in cutaneous disease and across the spectrum of systemic mastocytosis subtypes, the presence of an associated hematologic neoplasm and/or organ damage denotes advanced disease and the potential for increased morbidity and mortality. In addition to the revised 2016 World Health Organization classification of mastocytosis, a new diagnostic and treatment toolkit, tethered to

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9

Lange, Magdalena, Karin Hartmann, Melody C. Carter, et al. "Molecular Background, Clinical Features and Management of Pediatric Mastocytosis: Status 2021." International Journal of Molecular Sciences 22, no. 5 (2021): 2586. http://dx.doi.org/10.3390/ijms22052586.

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Pediatric mastocytosis is a heterogeneous disease characterized by accumulation of mast cells in the skin and less frequently in other organs. Somatic or germline mutations in the KIT proto-oncogene are detected in most patients. Cutaneous mastocytosis is the most common form of the disease in children. In the majority of cases, skin lesions regress spontaneously around puberty. However, in few patients, mastocytosis is not a self-limiting disease, but persists into adulthood and can show signs of systemic involvement, especially when skin lesions are small-sized and monomorphic. Children with

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10

Schwaab, Juliana, Susanne Schnittger, Karl Sotlar, et al. "Comprehensive mutational profiling in advanced systemic mastocytosis." Blood 122, no. 14 (2013): 2460–66. http://dx.doi.org/10.1182/blood-2013-04-496448.

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Key Points Additional genetic aberrations apart from KIT D816V are found in advanced systemic mastocytosis. Additional genetic aberrations apart from KIT D816V are associated with a significant reduction of overall survival.

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11

Dash, Durga Prasad, David Dinauer, and Michael Janasik. "Possible Early Clinical Management of Systemic Mastocytosis Patients with Recently FDA Approved Drug Avapritinib By Molecular Diagnosis Using a Highly Sensitive KIT D816 Mutation Assay." Blood 138, Supplement 1 (2021): 4622. http://dx.doi.org/10.1182/blood-2021-151962.

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Abstract Recently in June 2021, the Food and Drug Administration approved avapritinib (Ayvakit™) for adult patients with advanced systemic mastocytosis (AdvSM), including patients with aggressive systemic mastocytosis (ASM), systemic mastocytosis with an associated hematological neoplasm (SM-AHN), and mast cell leukemia (MCL). Mastocytosis is a heterogeneous, neoplastic disorder characterized by infiltration of abnormal mast cells in one or more organs. Mastocytosis subtypes are defined by disease distribution and the clinical features include, cutaneous mastocytosis (CM), where mast cell infi

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12

Martina, Rudelius, and Horny Hans-Peter. "Mastocytosis: Principles and Pitfalls in the Diagnosis of a Unique Disease." IgMin Research 2, no. 8 (2024): 694–701. http://dx.doi.org/10.61927/igmin234.

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Mastocytosis, a hematological neoplasm, manifests with diverse clinical, molecular, and histomorphological features. This review explores the different subtypes of mastocytosis, focusing on the role of molecular pathology and histomorphology in diagnosing systemic mastocytosis (SM). Systemic mastocytosis is characterized by histologically confirmed extracutaneous involvement, presenting a diagnostic challenge due to its rarity and diverse subtypes, ranging from occult SM to mast cell leukemia. The complexity of accurate SM diagnosis underscores the necessity for a comprehensive understanding o

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13

Greiner, Georg, Nadine Witzeneder, Angelika Berger, et al. "CCL2 is a KIT D816V–dependent modulator of the bone marrow microenvironment in systemic mastocytosis." Blood 129, no. 3 (2017): 371–82. http://dx.doi.org/10.1182/blood-2016-09-739003.

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Key Points CCL2 is a KIT D816V–induced cytokine targeting microenvironmental cells in mastocytosis in vitro and in vivo. Serum levels of CCL2 in patients with mastocytosis correlate with advanced disease and poor survival.

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14

Gilreath, JA, L. Tchertanov, and MW Deininger. "Novel approaches to treating advanced systemic mastocytosis." Clinical Pharmacology: Advances and Applications Volume 11 (July 2019): 77–92. http://dx.doi.org/10.2147/cpaa.s206615.

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15

Tremblay, Douglas, Nicole Wagner, and John Mascarenhas. "Management of Advanced Systemic Mastocytosis: Clinical Challenges." Journal of Blood Medicine Volume 15 (September 2024): 421–33. http://dx.doi.org/10.2147/jbm.s366367.

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16

Li, Zhixiong. "New Insights into the Pathogenesis of Systemic Mastocytosis." International Journal of Molecular Sciences 22, no. 9 (2021): 4900. http://dx.doi.org/10.3390/ijms22094900.

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Mastocytosis is a type of myeloid neoplasm characterized by the clonal, neoplastic proliferation of morphologically and immunophenotypically abnormal mast cells that infiltrate one or more organ systems. Systemic mastocytosis (SM) is a more aggressive variant of mastocytosis with extracutaneous involvement, which might be associated with multi-organ dysfunction or failure and shortened survival. Over 80% of patients with SM carry the KIT D816V mutation. However, the KIT D816V mutation serves as a weak oncogene and appears to be a late event in the pathogenesis of mastocytosis. The management o

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17

Zanotti, Roberta, Massimiliano Bonifacio, Ilaria Tanasi, et al. "SYSTEMIC MASTOCYTOSIS: MULTIDISCIPLINARY APPROACH." Mediterranean Journal of Hematology and Infectious Diseases 13, no. 1 (2021): e2021068. http://dx.doi.org/10.4084/mjhid.2021.068.

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Systemic mastocytosis (SM) is a heterogeneous group of diseases that affect almost exclusively adults and are defined by the proliferation and accumulation of clonal mast cells (MC) in various tissues. Disease subtypes range from indolent to rare but aggressive forms. Although SM is classified as a rare disease, it is believed to be likely underdiagnosed. Major signs and symptoms mainly depend on MC activation and less frequently to organ infiltration, typical of more aggressive variants. Diagnosis may be challenging, and symptoms can be aspecific and involve several organs. It is advisable to

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18

Veitch, Scott, and Deepti H. Radia. "Mastocytosis demystified." Hematology 2023, no. 1 (2023): 396–406. http://dx.doi.org/10.1182/hematology.2023000505.

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Abstract Mastocytosis is a rare, clinically heterogenous clonal hematological neoplasm. Over 95% of patients harbor the driver KIT D816V mutation resulting in mast cell (MC) accumulation and proliferation in various organs, leading to variable symptom manifestations that result from MC mediator release in patients with systemic mastocytosis (SM) and end-organ damage in those with advanced SM. The accurate diagnostic and clinical classification of patients with SM is vital to underpin appropriate treatment options and personalize therapy. This review evaluates the current diagnostic criteria, c

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19

Taylor, F., A. Shields, S. Li, et al. "PRO143 PSYCHOMETRIC EVALUATION OF THE ADVANCED SYSTEMIC MASTOCYTOSIS SYMPTOM ASSESSMENT FORM (ADVSM-SAF) IN PATIENTS WITH ADVANCED SYSTEMIC MASTOCYTOSIS." Value in Health 22 (November 2019): S868. http://dx.doi.org/10.1016/j.jval.2019.09.2472.

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20

Marchaj, Magdalena, Bartosz Basiaga, Klaudia Żądecka-Kobiałka, et al. "Comprehensive Review of Mastocytosis From Pathophysiology to Management Strategies." Journal of Education, Health and Sport 66 (April 22, 2024): 50169. http://dx.doi.org/10.12775/jehs.2024.66.015.

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Introduction: Mastocytosis is a rare hematologic neoplasm characterized by the abnormal proliferation and accumulation of mast cells in various tissues. Its clinical manifestations vary widely, ranging from cutaneous lesions to systemic involvement with potentially life-threatening symptoms. Understanding the pathophysiology, diagnosis, and management of mastocytosis is crucial for improving patient outcomes.   Aim of the Study: The aim of this study was to provide a comprehensive overview of mastocytosis, including its epidemiology, risk factors, pathophysiology, clinical manifest

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21

Jawhar, Mohamad, Juliana Schwaab, Iván Álvarez-Twose, et al. "MARS: Mutation-Adjusted Risk Score for Advanced Systemic Mastocytosis." Journal of Clinical Oncology 37, no. 31 (2019): 2846–56. http://dx.doi.org/10.1200/jco.19.00640.

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PURPOSE To develop a risk score for patients with advanced systemic mastocytosis (AdvSM) that integrates clinical and mutation characteristics. PATIENTS AND METHODS The study included 383 patients with AdvSM from the German Registry on Disorders of Eosinophils and Mast Cells (training set; n = 231) and several centers for mastocytosis in the United States and Europe, all within the European Competence Network on Mastocytosis (validation set; n = 152). A Cox multivariable model was used to select variables that were predictive of overall survival (OS). RESULTS In multivariable analysis, the fol

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22

Chandesris, Marie-Olivia, Gandhi Damaj, Olivier Lortholary, and Olivier Hermine. "Clinical potential of midostaurin in advanced systemic mastocytosis." Blood and Lymphatic Cancer: Targets and Therapy Volume 7 (May 2017): 25–35. http://dx.doi.org/10.2147/blctt.s87186.

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23

Morales-Camacho, Rosario M., Sandra Villanueva-Herraiz, Concepción Prats-Martín, Juan J. Borrero, Ricardo Bernal, and M. Teresa Vargas. "Eosinophil phagocytosis in advanced systemic mastocytosis with eosinophilia." British Journal of Haematology 181, no. 5 (2018): 578. http://dx.doi.org/10.1111/bjh.15168.

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24

Valent, Peter, Wolfgang R. Sperr, and Cem Akin. "How I treat patients with advanced systemic mastocytosis." Blood 116, no. 26 (2010): 5812–17. http://dx.doi.org/10.1182/blood-2010-08-292144.

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Abstract Advanced systemic mastocytosis (SM) is a rare myeloid neoplasm characterized by uncontrolled accumulation of neoplastic mast cells (MCs) in various organs with consecutive impairment of organ function, drug resistance, and a poor prognosis. Advanced SM may present as smoldering or slowly progressing neoplasm but may also present as rapidly progressing aggressive SM or even as MC leukemia. Approximately half of the patients have an associated hematologic non–MC-lineage disease (SM-AHNMD) or develop an AHNMD over time. Drug resistance may not only result from the KIT mutant D816V that i

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25

Fritz, J., E. K. Fishman, J. A. Carrino, and M. S. Horger. "Advanced imaging of skeletal manifestations of systemic mastocytosis." Skeletal Radiology 41, no. 8 (2012): 887–97. http://dx.doi.org/10.1007/s00256-012-1374-9.

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26

Ustun, Celalettin, Andreas Reiter, Bart L. Scott, et al. "Hematopoietic Stem-Cell Transplantation for Advanced Systemic Mastocytosis." Journal of Clinical Oncology 32, no. 29 (2014): 3264–74. http://dx.doi.org/10.1200/jco.2014.55.2018.

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Purpose Advanced systemic mastocytosis (SM), a fatal hematopoietic malignancy characterized by drug resistance, has no standard therapy. The effectiveness of allogeneic hematopoietic stem-cell transplantation (alloHCT) in SM remains unknown. Patients and Methods In a global effort to define the value of HCT in SM, 57 patients with the following subtypes of SM were evaluated: SM associated with clonal hematologic non–mast cell disorders (SM-AHNMD; n = 38), mast cell leukemia (MCL; n = 12), and aggressive SM (ASM; n = 7). Median age of patients was 46 years (range, 11 to 67 years). Donors were H

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27

Jawhar, Mohamad, Juliana Schwaab, Nicole Naumann, et al. "Genomic landscape in KIT D816V+ advanced systemic mastocytosis." Clinical Lymphoma Myeloma and Leukemia 15 (September 2015): S55—S56. http://dx.doi.org/10.1016/j.clml.2015.07.113.

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28

Nicolosi, Maura, Andrea Patriarca, Annalisa Andorno, et al. "Precision Medicine in Systemic Mastocytosis." Medicina 57, no. 11 (2021): 1135. http://dx.doi.org/10.3390/medicina57111135.

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Mastocytosis is a rare hematological neoplasm characterized by the proliferation of abnormal clonal mast cells (MCs) in different cutaneous and extracutaneous organs. Its diagnosis is based on well-defined major and minor criteria, including the pathognomonic dense infiltrate of MCs detected in bone marrow (BM), elevated serum tryptase level, abnormal MCs CD25 expression, and the identification of KIT D816V mutation. The World Health Organization (WHO) classification subdivides mastocytosis into a cutaneous form (CM) and five systemic variants (SM), namely indolent/smoldering (ISM/SSM) and adv

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29

Sciumè, Mariarita, Claudio De Magistris, Nicole Galli, et al. "Target Therapies for Systemic Mastocytosis: An Update." Pharmaceuticals 15, no. 6 (2022): 738. http://dx.doi.org/10.3390/ph15060738.

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Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MCs) in extra-cutaneous organs. It could be divided into indolent SM, smoldering SM, SM with an associated hematologic (non-MC lineage) neoplasm, aggressive SM, and mast cell leukemia. SM is generally associated with the presence of a gain-of-function somatic mutation in KIT at codon 816. Clinical features could be related to MC mediator release or to uncontrolled infiltration of MCs in different organs. Whereas indolent forms have a near-normal life expectancy, advanced diseases have a poor prognosis with s

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30

Chan, Ching, Yun Bai, Irina Maric, Amy Klion, Dean Metcalfe, and Todd Wilson. "Expression of KIT isoforms in systemic mastocytosis: correlation with disease severity and KITD816V (153.32)." Journal of Immunology 186, no. 1_Supplement (2011): 153.32. http://dx.doi.org/10.4049/jimmunol.186.supp.153.32.

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Abstract Human KIT has several variants as the result of alternative mRNA splicing. Two major isoforms differ by the presence or absence of four amino acids (GNNK) at the juxta-membrane region of the extracellular domain. Somatic gain of function mutations in the tyrosine kinase domain of c-kit, most notably KITD816V, are detected in the majority of adult patients with systemic mastocytosis. We hypothesized that the expression pattern of GNNK variants may differ with disease severity, and transcripts containing the KITD816V mutation may segregate preferentially to one GNNK variant. A quantitat

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31

Castells, Mariana, and Cem Akin. "Finding the right KIT inhibitor for advanced systemic mastocytosis." Nature Medicine 27, no. 12 (2021): 2081–82. http://dx.doi.org/10.1038/s41591-021-01588-z.

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32

Gotlib, Jason, Hanneke C. Kluin-Nelemans, Tracy I. George, et al. "Efficacy and Safety of Midostaurin in Advanced Systemic Mastocytosis." New England Journal of Medicine 374, no. 26 (2016): 2530–41. http://dx.doi.org/10.1056/nejmoa1513098.

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33

Hoermann, Gregor, Katharina Blatt, Georg Greiner, et al. "CD52 is a molecular target in advanced systemic mastocytosis." FASEB Journal 28, no. 8 (2014): 3540–51. http://dx.doi.org/10.1096/fj.14-250894.

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34

Dahlin, J. S., J. S. Ungerstedt, J. Grootens, et al. "Detection of circulating mast cells in advanced systemic mastocytosis." Leukemia 30, no. 9 (2016): 1953–56. http://dx.doi.org/10.1038/leu.2016.105.

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35

Baird, John H., and Jason Gotlib. "Clinical Validation of KIT Inhibition in Advanced Systemic Mastocytosis." Current Hematologic Malignancy Reports 13, no. 5 (2018): 407–16. http://dx.doi.org/10.1007/s11899-018-0469-3.

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36

Lee, Hyun Jung. "Recent advances in diagnosis and therapy in systemic mastocytosis." Blood Research 58, S1 (2023): S96—S108. http://dx.doi.org/10.5045/br.2023.2023024.

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37

Sthel, VM, and VLP Figueiredo. "CASE REPORT: ADVANCED SYSTEMIC MASTOCYTOSIS ASSOCIATED WITH HEMATOLOGICAL NEOPLASIA." Hematology, Transfusion and Cell Therapy 45 (October 2023): S430. http://dx.doi.org/10.1016/j.htct.2023.09.806.

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38

Christen, Deborah, Johannes Lübke, Anne Kaiser, et al. "Allogeneic Hematopoietic Stem Cell Transplantation in Advanced Systemic Mastocytosis." Blood 142, Supplement 1 (2023): 3605. http://dx.doi.org/10.1182/blood-2023-178304.

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Purpose Advanced systemic mastocytosis (AdvSM) is driven by the KIT D816V mutation in &gt;90% of patients (pts.). Depending on subtype, median survival is between 1.5 and 4 years. Despite recent advances on responses and survival due to the availability of effective KIT inhibitors, allogeneic hematopoietic stem cell transplantation (alloHSCT) remains the only curative treatment option. Data on optimal timing, conditioning regimens, and pre- as well as post-alloHSCT treatments for maintenance or relapse are however scarce. Current recommendations mainly refer to a retrospective analysis pub

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39

El Hussein, Siba, Helen T. Chifotides, Joseph D. Khoury, Srdan Verstovsek, and Beenu Thakral. "Systemic Mastocytosis and Other Entities Involving Mast Cells: A Practical Review and Update." Cancers 14, no. 14 (2022): 3474. http://dx.doi.org/10.3390/cancers14143474.

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Evidence in the recent literature suggests that the presentation spectrum of mast cell neoplasms is broad. In this article, we elaborate on recent data pertaining to minor diagnostic criteria of systemic mastocytosis (SM), including sensitive testing methods for detection of activating mutations in the KIT gene or its variants, and adjusted serum tryptase levels in cases with hereditary α-tryptasemia. We also summarize entities that require differential diagnosis, such as the recently reclassified SM subtype named bone marrow mastocytosis, mast cell leukemia (an SM subtype that can be acute or

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Gotlib, Jason, Animesh Pardanani, Cem Akin, et al. "International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) & European Competence Network on Mastocytosis (ECNM) consensus response criteria in advanced systemic mastocytosis." Blood 121, no. 13 (2013): 2393–401. http://dx.doi.org/10.1182/blood-2012-09-458521.

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Abstract Systemic mastocytosis (SM) is characterized by accumulation of neoplastic mast cells and is classified into indolent and aggressive forms. The latter include aggressive SM (ASM), mast cell leukemia (MCL), and SM associated with a myeloid neoplasm wherein 1 or both disease compartments exhibit advanced features. These variants, henceforth collectively referred to as advanced SM for the purposes of this report, are typically characterized by organ damage and shortened survival duration. In contrast to indolent SM, in which symptoms are usually managed by noncytotoxic antimediator therap

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Valent, Peter, Cem Akin, Karoline V. Gleixner, et al. "Multidisciplinary Challenges in Mastocytosis and How to Address with Personalized Medicine Approaches." International Journal of Molecular Sciences 20, no. 12 (2019): 2976. http://dx.doi.org/10.3390/ijms20122976.

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Mastocytosis is a hematopoietic neoplasm defined by abnormal expansion and focal accumulation of clonal tissue mast cells in various organ-systems. The disease exhibits a complex pathology and an equally complex clinical behavior. The classification of the World Health Organization (WHO) divides mastocytosis into cutaneous forms, systemic variants, and localized mast cell tumors. In >80% of patients with systemic mastocytosis (SM), a somatic point mutation in KIT at codon 816 is found. Whereas patients with indolent forms of the disease have a normal or near-normal life expectancy, patients

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Letizia Mauro, Giulia, Jessica Accomando, Sofia Tomasello, et al. "Osteoporosis in Systemic Mastocytosis: A Scoping Review." Medicina 60, no. 11 (2024): 1752. http://dx.doi.org/10.3390/medicina60111752.

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Background: Mastocytosis (MS) is a rare disease that can involve various organs, including the bone. Given the incidence of the disease in the global population, MS poses a challenge for physicians, and early therapeutic intervention in the initial stages could significantly impact the quality of life of affected patients. Objective: The aim of this scoping review was to provide an overview of secondary osteoporosis in systemic mastocytosis (SM), focusing on the heterogeneity of its manifestations, the benefits of early diagnosis, and appropriate pharmacological treatment. Design: A technical

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Frank, Kuo, Jon C. Aster, and Coleman Lindsley. "Two-Year Experience of Performing a Next-Generation-Sequencing Based Panel Test in an Academic Medical Center and Its Clinical Impact." Blood 128, no. 22 (2016): 1707. http://dx.doi.org/10.1182/blood.v128.22.1707.1707.

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Abstract Our ability to interrogate a broad array of genetic alterations in myeloid neoplasm has increased significantly with the advance in next-generation sequencing (NGS). In addition to morphologic examination, flow cytometry and cytogenetics, NGS-based testing can add additional useful information to the diagnostic workup. With improved turnaround time, decreasing costs and an expanding knowledge of the therapeutic and prognostic significance of the detected variants, NGS-based panel testing has increasingly played a major role in the management of patients with myeloid neoplasm. Rapid He

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Arock, Michel, Ghaith Wedeh, Gregor Hoermann, et al. "Preclinical human models and emerging therapeutics for advanced systemic mastocytosis." Haematologica 103, no. 11 (2018): 1760–71. http://dx.doi.org/10.3324/haematol.2018.195867.

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Jawhar, M., J. Schwaab, I. Álvarez-Twose, et al. "S1611 MARS: MUTATION-ADJUSTED RISK SCORE FOR ADVANCED SYSTEMIC MASTOCYTOSIS." HemaSphere 3, S1 (2019): 742–43. http://dx.doi.org/10.1097/01.hs9.0000564692.21696.81.

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Atieh, Tahani, Janet Woodroof, and Abdulraheem Yacoub. "Venetoclax and Decitabine Show Robust Activity in Advanced Systemic Mastocytosis." Blood 136, Supplement 1 (2020): 25–26. http://dx.doi.org/10.1182/blood-2020-138826.

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Systemic mastocytosis (SM) is a heterogeneous group of diseases characterized by the proliferation of abnormal mast cells in the bone marrow or other organs. 1 Activating mutations in KIT are found in the majority of patients, with the KIT D816V mutation being the most common .2 While patients with indolent systemic mastocytosis (ISM) have a life-expectancy similar to the general population, approximately 40-53% of patients with SM have an associated hematologic neoplasm (SM-AHN) with a median overall survival of 2 years. 1-3 Treatment of SM-AHN is primarily directed at the AHN as this determi

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Helbig, Grzegorz, Anna Koclęga, Władysław B. Gaweł, et al. "The Efficacy of Cladribine (2-CdA) in Advanced Systemic Mastocytosis." Indian Journal of Hematology and Blood Transfusion 36, no. 4 (2020): 661–66. http://dx.doi.org/10.1007/s12288-020-01279-8.

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Abstract:

Abstract Systemic mastocytosis (SM) is a rare clonal disorder with multi-organ involvements and shortened life expectancy. To date, no curative treatment for SM exists. Cladribine (2-CdA) is a purine analogue showing activity against neoplastic mast cells and its use was found to be effective in some patients with SM. Nine patients (six males and three females) with advanced SM at median age of 63 years (range 33–67) who received at least one course of 2-CdA were included in a retrospective analysis. Study patients were classified as having aggressive SM (ASM; n = 7) and SM with an associated

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Shikhbabaeva, D. I., O. Yu Vinogradova, Yu N. Kobzev, et al. "Genetic features of indolent and advanced forms of systemic mastocytosis." Oncohematology 20, no. 2 (2025): 37–52. https://doi.org/10.17650/1818-8346-2025-20-2-37-52.

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Abstract:

Background. Mastocytosis is a group of diseases characterized by tumor proliferation of mast cells and their accumulation in organs and tissues, including skin, hematopoietic organs (bone marrow, spleen, lymph nodes), and the gastrointestinal tract, which is clinically manifested by symptoms of mast cell activation and organ infiltration in the form of hepatosplenomegaly, portal hypertension, ascites, and cytopenia. Of the greatest scientific and clinical interest is systemic mastocytosis (SM), where indolent (indolent SM, smoldering SM, SM with isolated bone marrow involvement) and advanced f

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Dasilva-Freire, Noelia, Andrea Mayado, Cristina Teodosio, et al. "Bone Marrow Mast Cell Antibody-Targetable Cell Surface Protein Expression Profiles in Systemic Mastocytosis." International Journal of Molecular Sciences 20, no. 3 (2019): 552. http://dx.doi.org/10.3390/ijms20030552.

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Abstract:

Despite recent therapeutic advances, systemic mastocytosis (SM) remains an incurable disease due to limited complete remission (CR) rates even after novel therapies. To date, no study has evaluated the expression on SM bone marrow mast cells (BMMC) of large panel of cell surface suitable for antibody-targeted therapy. In this study, we analyzed the expression profile of six cell-surface proteins for which antibody-based therapies are available, on BMMC from 166 SM patients vs. 40 controls. Overall, variable patterns of expression for the markers evaluated were observed among SM BMMC. Thus, CD2

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Reiter, Andreas, Tracy I. George, and Jason Gotlib. "New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis." Blood 135, no. 16 (2020): 1365–76. http://dx.doi.org/10.1182/blood.2019000932.

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Abstract:

Abstract Systemic mastocytosis (SM) has greatly benefited from the broad application of precision medicine techniques to hematolymphoid neoplasms. Sensitive detection of the recurrent KIT D816V mutation and use of next-generation sequencing (NGS) panels to profile the genetic landscape of SM variants have been critical adjuncts to the diagnosis and subclassification of SM, and development of clinical-molecular prognostic scoring systems. Multilineage KIT involvement and multimutated clones are characteristic of advanced SM (advSM), especially SM with an associated hematologic neoplasm (AHN). A

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