Prevalence of indolent systemic mastocytosis in a Dutch region

Doormaal, Jasper J. van; Arends, Suzanne; Brunekreeft, Kim L; Wal, V. Bauco van der; Sietsma, Johanna; Vader, Pieter; Elberink, Joanne N.G. Oude; Kluin-Nelemans, Hanneke C.; Veer, Eveline van der; Monchy, J. G. R. De

doi:10.1016/j.jaci.2012.10.015

Cited by 85 publications

(58 citation statements)

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“…However, according to recent molecular genetic findings 55,60,61 , the subclasses and clinical subtypes of MCAD do not represent distinct disease entities but should be more accurately regarded as varying compositions of a common mast cell dysfunction. 50,62-64 While the prevalence for SM in Europeans ranges between 0.3 and 13:100,000 [65][66][67] , the prevalence for MCAS may be as high as 17% (in Germany). 68…”

Section: Physiological and Pathophysiological Roles Of Mast Cells In mentioning

confidence: 99%

Cardiovascular symptoms in patients with systemic mast cell activation disease

Kolck

Haenisch

Molderings

2016

Translational Research

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Section: Physiological and Pathophysiological Roles Of Mast Cells In mentioning

confidence: 99%

Cardiovascular symptoms in patients with systemic mast cell activation disease

Kolck

Haenisch

Molderings

2016

Translational Research

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“…21 This value is comparable to prevalence estimates of ISM in the Netherlands. 36 SM with subtype unknown was the second most common (11%), followed by SM-AHNMD (4%), ASM (2%), and MCL (1%). 21 This breakdown is in contrast to a report by the Mayo Clinic, which examined 342 consecutive patients with SM seen at their institution.…”

Section: Diagnosis and Classificationmentioning

confidence: 99%

Systemic Mastocytosis: Clinical Update and Future Directions

Tremblay

Carreau

Kremyanskaya

et al. 2015

Clinical Lymphoma Myeloma and Leukemia

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“…In the Danish nationwide cohort study, the incidence and prevalence rates were 0.89 per 100,000 inhabitants per year and 9.59 per 100,000 inhabitants, respectively, for the period between 1997 and 2010 [2]. Similarly, in the Groningen region, The Netherlands, prevalence of indolent SM (ISM) was estimated at 13 per 100,000 inhabitants, albeit with a roughly five-to sixfold increase in cases from 1998 to 2010, the latter likely relating to a recent increased awareness of SM combined with advances in diagnostic testing and expertise in specialized centers for this disease [3]. The increasing number of cases diagnosed with SM at least partly reflects the development of standardized diagnostic, classification and treatment-response criteria by international collaborative organizations such as the World Health Organization (WHO) and European Competence Network on Mastocytosis (ECNM) [4][5][6].…”

mentioning

confidence: 99%

Systemic mastocytosis: evolving lessons from large patient registry datasets

Pardanani

2016

American J Hematol

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Systemic mastocytosis (SM) is a clinically heterogeneous and relatively infrequent chronic myeloproliferative neoplasm [1]. In the Danish nationwide cohort study, the incidence and prevalence rates were 0.89 per 100,000 inhabitants per year and 9.59 per 100,000 inhabitants, respectively, for the period between 1997 and 2010 [2]. Similarly, in the Groningen region, The Netherlands, prevalence of indolent SM (ISM) was estimated at 13 per 100,000 inhabitants, albeit with a roughly five-to sixfold increase in cases from 1998 to 2010, the latter likely relating to a recent increased awareness of SM combined with advances in diagnostic testing and expertise in specialized centers for this disease [3]. The increasing number of cases diagnosed with SM at least partly reflects the development of standardized diagnostic, classification and treatment-response criteria by international collaborative organizations such as the World Health Organization (WHO) and European Competence Network on Mastocytosis (ECNM) [4][5][6]. Prior to these larger-scale projects, most SM related data emerged from single center or single country registry studies, which despite their inherent limitations helped establish the early foundational principles of SM evaluation and treatment [7][8][9][10][11][12][13]. The article by Pieri et al. in the current issue of the American Journal of Hematology represents a large study of SM patients from 10 Italian centers with dedicated mastocytosis programs participating in the Italian Registry of Mastocytosis [14].There are two aspects of special note related to the study by Pieri et al. First, it represents the largest study of SM patients diagnosed by WHO 2008 criteria ever published [5]. Second, patients in this study were diagnosed relatively recently, with 75% being diagnosed between the years 2009 and 2014. The latter aspect ensured the use of relatively state-of-the-art diagnostic testing in the current study, which is not the case in older studies wherein a significant subset of patients was diagnosed prior to the development of such tests. A downside, however, of the recency bias in the article by Pieri et al. is the short patient follow up which limits the conclusions one might draw regarding clinical outcomes in SM patients.There are several additional observations that flow from the above two points: first, while the study by Pieri et al. includes 460 patients, as compared with the erstwhile largest study of 342 patients from the Mayo Clinic group [8], it is unclear whether either study is generalizable to the SM patient at large. While both were multidisciplinary studies, the former is predominantly a study of ISM patients (89% of all cases), likely reflecting a bias toward patient enrollment via dermatology and/or allergy clinics (73% had allergy/anaphylaxis symptoms). In contrast, the study by Lim et al. exhibited a hematology bias with 46% and 40% presenting with ISM and SM with associated clonal non-mast cell lineage disease (SM-AHNMD), respectively. This has to be taken into account when ex...

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Prevalence of indolent systemic mastocytosis in a Dutch region

Cited by 85 publications

References 3 publications

Cardiovascular symptoms in patients with systemic mast cell activation disease

Cardiovascular symptoms in patients with systemic mast cell activation disease

Systemic Mastocytosis: Clinical Update and Future Directions

Systemic mastocytosis: evolving lessons from large patient registry datasets

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