Management of labor and delivery by spinal and epidural analgesia in a woman with systemic mastocytosis: a case report

Systemic mastocytosis is a life-threatening disease in which mast cell mediator release can lead to general symptoms. The most common triggers are stress and pain during labor and delivery. We report the management of labor and delivery in a case with severe systemic mastocytosis by epidural analgesia.

Differences and Similarities in the Mechanisms and Clinical Expression of Bradykinin-Mediated vs. Mast Cell–Mediated Angioedema

Angioedema (AE), transient localized swelling due to extravasated fluid, is commonly classified as mast cell mediator-induced, bradykinin-mediated or of unknown cause. AE often occurs more than once, and it is these recurrent forms of AE that are challenging for patients and physicians, and they are the ones we focus on and refer to as AE in this review. Since effective treatment depends on the causative mediator, reliable and early diagnosis is essential. Although their clinical presentations bear similarities, many forms of angioedema exhibit specific patterns of clinical appearance or disease history that may aid in diagnosis. Here, we describe the most common differences and similarities in the mechanisms and clinical features of bradykinin-mediated and mast cell mediator-induced types of angioedema. We first provide an overview of the diseases that manifest with mast cell mediator-induced versus bradykinin-mediated angioedema as well as their respective underlying pathogenesis. We then compare these diseases for key clinical features, including angioedema location, course and duration of swelling, attack frequency, prevalence and relevance of prodromal signs and symptoms, triggers of angioedema attacks, and other signs and symptoms including wheals, age of onset, and duration. Our review and comparison of the clinical profiles of different types of angioedema incorporate our own clinical experience as well as published information. Our aim is to highlight that mast cell mediator-induced and bradykinin-mediated angioedema types share common features but are different in many aspects. Knowledge of the differences in underlying pathomechanisms and clinical profiles between different types of angioedema can help with the diagnostic approach in affected patients and facilitate targeted and effective treatment.

A Practical Guide for Treatment of Pain in Patients with Systemic Mast Cell Activation Disease

Systemic mast cell activation disease (MCAD, a subclass of mastocytosis), which has a prevalence of around 17% (at least in the German population), is characterized by accumulation of genetically altered dysfunctional mast cells with abnormal release of these cells’ mediators. Since mast cells affect functions in potentially every organ system, often without causing abnormalities in routine laboratory or radiologic testing, this disease has to be considered routinely in the differential diagnosis of patients with chronic multisystem polymorbidity of a generally inflammatory and allergic theme. Pain in its different manifestations is a common symptom in MCAD found in more than three-quarters of the MCAD patients. Because of the specific mast cell-related causes of pain in MCAD it should be treated specifically, if possible, deduced from their putative mast cell mediator-related causes. As yet, there is no official guideline for treatment of MCAD at all. The present review focuses on mast cell mediator-induced acute and chronic pain and the current state of analgesic drug therapy options in MCAD. Due to the high prevalence of MCAD, many physicians are often faced with the issue of pain management in MCAD patients. Hence, our practical guide should contribute to the improvement of patient care.