Scholarlink

Introduction <p>Mast cell disorders can cause allergy-like symptoms or elevated baseline serum tryptase levels (BST) through the release of mast cell mediators. Patients with previously unexplained symptoms attribute these to a&#xa0;mast cell disorder or histamine intolerance (HIT) and view this as the cause of their symptoms.</p> Methods <p>The scientific literature and our own experience were evaluated and summarized for this review to describe specific constellations of findings that require investigation for the diseases or characteristics mentioned in the title.</p> Results <p>Classic manifestations that should raise suspicion of mastocytosis include typical skin lesions, severe insect venom anaphylaxis, idiopathic anaphylaxis, or unexplained osteoporosis. Significantly elevated BST levels are also a&#xa0;criterion for systemic mastocytosis (SM). However, the most common cause of (often moderately) elevated BST is hereditary alpha-tryptasemia (HαT). To date, the increased prevalence of HαT has only been documented in cases of severe insect venom anaphylaxis, idiopathic anaphylaxis, and mastocytosis, while the majority of HαT carriers (5% of the population) do not differ from a&#xa0;population without HαT in terms of their symptoms. In the past, HIT was used as an explanatory model for multiple somatically unexplained symptoms; however, even after evaluating more recent studies, the existence of this condition must continue to be questioned. A&#xa0;diagnosis of mast cell activation syndrome (MCAS) is frequently suspected. The application of scientific diagnostic criteria in cases of suspected idiopathic MCAS confirmed this diagnosis almost exclusively in patients with idiopathic anaphylaxis.</p> Conclusion <p>In cases of polysymptomatic complaints without indicative leading symptoms and constellations of findings, the diagnosis of the aforementioned entities should be avoided.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Only specific combinations of findings indicate mastocytosis, hereditary α-tryptasemia, histamine intolerance, or mast cell activation syndrome

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年5月20日

查看全文
  • Knut Brockow,
  • Tobias Michael Franz,
  • Magdalena Saak,
  • Hannah Lorenz,
  • Julia Plötz,
  • Leonhard Stark,
  • Alexander Schäbitz,
  • Görkem Aydin,
  • Svenja Rupp,
  • Johannes Untch,
  • Tilo Biedermann

摘要

Introduction

Mast cell disorders can cause allergy-like symptoms or elevated baseline serum tryptase levels (BST) through the release of mast cell mediators. Patients with previously unexplained symptoms attribute these to a mast cell disorder or histamine intolerance (HIT) and view this as the cause of their symptoms.

Methods

The scientific literature and our own experience were evaluated and summarized for this review to describe specific constellations of findings that require investigation for the diseases or characteristics mentioned in the title.

Results

Classic manifestations that should raise suspicion of mastocytosis include typical skin lesions, severe insect venom anaphylaxis, idiopathic anaphylaxis, or unexplained osteoporosis. Significantly elevated BST levels are also a criterion for systemic mastocytosis (SM). However, the most common cause of (often moderately) elevated BST is hereditary alpha-tryptasemia (HαT). To date, the increased prevalence of HαT has only been documented in cases of severe insect venom anaphylaxis, idiopathic anaphylaxis, and mastocytosis, while the majority of HαT carriers (5% of the population) do not differ from a population without HαT in terms of their symptoms. In the past, HIT was used as an explanatory model for multiple somatically unexplained symptoms; however, even after evaluating more recent studies, the existence of this condition must continue to be questioned. A diagnosis of mast cell activation syndrome (MCAS) is frequently suspected. The application of scientific diagnostic criteria in cases of suspected idiopathic MCAS confirmed this diagnosis almost exclusively in patients with idiopathic anaphylaxis.

Conclusion

In cases of polysymptomatic complaints without indicative leading symptoms and constellations of findings, the diagnosis of the aforementioned entities should be avoided.