Lower prevalence of vitamin D insufficiency in German patients with systemic mast cell activation syndrome compared with the general population

2017 ◽  
Vol 55 (12) ◽  
pp. 1297-1306
Author(s):  
Stefan Wirz ◽  
Hans-Jörg Hertfelder ◽  
Holger Seidel ◽  
Jürgen Homann ◽  
Gerhard Molderings

Abstract Introduction The common mastocytosis variant systemic mast cell activation syndrome (MCAS) may underlie at least a subset of patients with irritable bowel syndrome (IBS). A critical role of vitamin D (VD) in the stabilization of mast cells (MCs) with deficiency of VD resulting in MC activation has been demonstrated. If so, supplementation of VD would be a potential therapeutic approach in the treatment of those IBS patients. Methods We investigated in the present study for the first time systematically whether the VD level in 100 MCAS patients differed from that in the German general population (Ggp) and made a first attempt to elucidate potential reasons for possible differences by simultaneously determining the blood levels of heparin and cholesterol. Results In contrast to the Ggp, the VD level was detected in a sufficient range (> 30 ng/mL) in 53 % of the MCAS patients (Ggp 8 %), and only 34 % had values in the range of deficiency (< 20 ng/mL; GgP 75 %). There was no correlation between VD blood level and heparin and cholesterol blood levels. Conclusions The demonstration that in the majority of MCAS patients the VD level is not in a deficient range argues against an essential contribution of VD deficiency to the high prevalence of MCAS in Germany. Our findings do not exclude the possibility of smaller effects of VD level on MC activation in vivo. However, if such effects are present, the effect sizes seem to be too small to become identifiable in the multifactorial process of disease development.

F1000Research ◽  
2017 ◽  
Vol 6 ◽  
pp. 1889 ◽  
Author(s):  
Gerhard J. Molderings ◽  
Thomas Zienkiewicz ◽  
Jürgen Homann ◽  
Markus Menzen ◽  
Lawrence B. Afrin

Background:  It has been shown repeatedly that mast cells can promote or prevent cancer development and growth. If development and/or progression of a solid cancer is substantially influenced by mast cell activity, the frequencies of occurrence of solid cancers in patients with primary mast cells disorders would be expected to differ from the corresponding prevalence data in the general population. In fact, a recent study demonstrated that patients with systemic mastocytosis (i.e., a rare neoplastic variant of the primary mast cell activation disease) have increased risk for solid cancers, in particular melanoma and non-melanoma skin cancers. The aim of the present study is to examine whether the risk of solid cancer is increased in systemic mast cell activation syndrome (MCAS), the common systemic variant of mast cell activation disease. Methods: In the present descriptive study, we have analysed a large (n=828) patient group with MCAS, consisting of cohorts from Germany and the USA, for occurrence of solid forms of cancer and compared the frequencies of the different cancers with corresponding prevalence data for German and U.S. general populations. Results: Sixty-eight of the 828 MCAS patients (46 female, 22 male) had developed a solid tumor before the diagnosis of MCAS was made. Comparison of the frequencies of the malignancies in the MCAS patients with their prevalence in the general population revealed a significantly increased prevalence for melanoma and cancers of the breast, cervix uteri, ovary, lung, and thyroid in MCAS patients. Conclusions: Our data support the view that mast cells may promote development of certain malignant tumors. These findings indicate a need for increased surveillance of certain types of cancer in MCAS patients irrespective of its individual clinical presentation.


Diagnosis ◽  
2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Lawrence B. Afrin ◽  
Mary B. Ackerley ◽  
Linda S. Bluestein ◽  
Joseph H. Brewer ◽  
Jill B. Brook ◽  
...  

AbstractThe concept that disease rooted principally in chronic aberrant constitutive and reactive activation of mast cells (MCs), without the gross MC neoplasia in mastocytosis, first emerged in the 1980s, but only in the last decade has recognition of “mast cell activation syndrome” (MCAS) grown significantly. Two principal proposals for diagnostic criteria have emerged. One, originally published in 2012, is labeled by its authors as a “consensus” (re-termed here as “consensus-1”). Another sizable contingent of investigators and practitioners favor a different approach (originally published in 2011, newly termed here as “consensus-2”), resembling “consensus-1” in some respects but differing in others, leading to substantial differences between these proposals in the numbers of patients qualifying for diagnosis (and thus treatment). Overdiagnosis by “consensus-2” criteria has potential to be problematic, but underdiagnosis by “consensus-1” criteria seems the far larger problem given (1) increasing appreciation that MCAS is prevalent (up to 17% of the general population), and (2) most MCAS patients, regardless of illness duration prior to diagnosis, can eventually identify treatment yielding sustained improvement. We analyze these proposals (and others) and suggest that, until careful research provides more definitive answers, diagnosis by either proposal is valid, reasonable, and helpful.


2021 ◽  
pp. 135910532110145
Author(s):  
Jennifer Nicoloro SantaBarbara ◽  
Marci Lobel

Individuals with Mast Cell Activation Syndrome (MCAS), a rare chronic disease, experience unpredictable physical symptoms and diagnostic challenges resulting in poor emotional states. The prevalence and correlates of depressive symptoms were examined among 125 participants who completed the CES-D and relevant instruments. The majority reported a clinically-significant level of depression which was especially common among younger participants and those who reported greater loneliness or more disease-specific stressors. Greater magnitude of depressive symptoms was associated with greater illness intrusiveness, less social support, and lower optimism. Results highlight the value of interventions targeting loneliness and stressors unique to this population.


2017 ◽  
Vol 99 (2) ◽  
pp. 190-193 ◽  
Author(s):  
Lawrence B. Afrin ◽  
Roger W. Fox ◽  
Susan L. Zito ◽  
Leo Choe ◽  
Sarah C. Glover

2021 ◽  
Vol 127 (5) ◽  
pp. S49
Author(s):  
M. Hakim ◽  
D. Kurian ◽  
U. Rehman ◽  
S. Kazmi ◽  
A. Ghazi

2021 ◽  
Author(s):  
Isabelle Brock ◽  
Nicole Eng ◽  
Anne Maitland

Abstract Mast Cell Activation syndrome (MCAS) is a clinical condition, defined by the combination of 1) typical symptoms, 2) laboratory abnormalities and 3) response to treatment. Patients present with episodic symptoms of aberrant mast cell activation, such as abdominal cramping, asthma, hypotensive episodes, tachycardia, anaphylaxis, unexplained arrhythmias, and neurologic/psychiatric symptoms. Both clonal and nonclonal mast cell activation syndromes have been described, with a greater prevalence of non-clonal MCAS among the pediatric and adult population. Numerous extrinsic triggers of mast cell activation (MCA) are described, but recent reports point to nonatopic triggers, as the predominant, extrinsic stimulants of MCA in the adult population. The etiology of MCAS is unclear, though recent studies point to the disruption of the epithelium by infection, toxic exposures or physical trauma, and perturbation the tight regulation of these innate immune cells, associated to the epithelial borders. Here we describe a geriatric patient with adult onset MCAS, following a significant toxic exposure, scombroid poisoning. We also review the relevant literature regarding MCAS diagnosis and management as well as potential mechanisms for this hypersensitivity syndrome in adults.


Sign in / Sign up

Export Citation Format

Share Document