Medical algorithm: Peri‐operative management of mastocytosis patients
Authors: Inger Femke Astra Bocca‐Tjeertes, Annick A. J. M. van de Ven, Gerard H. Koppelman, Aline B. Sprikkelman, Hanneke J. N. G. Oude Elberink
Journal: Allergy (2021)

Mastocytosis is a clonal disorder characterized by the proliferation and accumulation of mast cells (MCs) in various tissue types, preferentially skin and bone marrow (BM). Mastocytosis consists of cutaneous and systemic forms in both pediatric and adult patients. Both the excess and increased propensity of MCs to release mediators leads to a higher frequency and severity of immediate hypersensitivity reactions.1-4 In the peri-operative setting, systemic mastocytosis is associated with a higher risk of severe anaphylaxis.1-7 Here, drugs such as nonsteroidal anti-inflammatory drugs, opioids and a broad range of anesthetics, temperature change, friction/pressure to the skin, and stress can cause mainly non–IgE-mediated MC (mediator) release.1-7 Prospective controlled studies on peri-operative hypersensitivity reactions in mastocytosis are lacking.8 Nevertheless, expert-based advice has been given on prohibited drugs.1 Moreover, antimediator therapy such as anti-H1, anti-H2, and corticosteroids is commonly prescribed peri-operatively.1-7 Albeit common practice, evidence supporting the value of premedication with antihistamines and corticosteroids is lacking, nor is there evidence of the benefit of specific drug regimens. However, there is also no evidence to the contrary. The potentially serious adverse outcomes in the peri-operative setting cause uncertainty in both doctor and patient. Position papers have discussed the available evidence in detail, resulting in recommendations for mastocytosis patients undergoing surgery under general anesthesia.3, 4 We provide an algorithm which serves as a tool for the practical management of both cutaneous and systemic, pediatric, and adult mastocytosis patients in need of procedures requiring (local or general) anesthesia in line with these recommendations (Figure 1).3, 4 Preferably, surgery in patients with systemic mastocytosis requiring general anesthesia should take place in a mastocytosis reference center. The medical team should be aware of and able to counteract severe allergic reactions before, during, and after surgery. To limit uncertainties, an individual plan should be made for each patient considering previous events and including decisions on premedication, type of anesthesia, and intra- and postoperative analgesia. In patients that have no record of anesthesia or analgesia, or when in doubt, medication with low capacity to elicit MC mediator release is preferred. Given uncertainties in premedication strategies, it may be useful to limit premedication to a risk group with previous anaphylaxis and/or extensive skin involvement in which excessive MC mediator release is most expected, undergoing general anesthesia. Premedication can consist of antihistamines and corticosteroids. If premedication is chosen to be administered, antihistamines can be given orally or intravenously shortly (<30 min) before starting anesthesia. Corticosteroids may be added, especially if H1 and H2 antihistamines are not available. The choice of corticosteroids depends on the clinical setting; elective procedures allow for oral administration 12 and 2 h before surgery, while emergency procedures depend on intravenous use. The choice of corticosteroids is preferably also made upon the desired duration of effect. Additionally, the prevention of nonspecific triggers is as much needed as any other precautions. Consider benzodiazepines for stress reduction and its probable direct beneficial regulatory effect on MCs.8, 9 Cutaneous MC proliferation in mastocytosis patients requires extra safety measures (Figure 1). Most pediatric and many adult patients have cutaneous lesions, of which some with blistering. Friction and pressure to the skin can cause excessive MC mediator release. Therefore, applying plasters or adhesives on lesions should be avoided and the use of adhesive remover is recommended upon (slow) removal. During surgery, anaphylaxis may consist of hypotension, often without skin involvement, or respiratory events. In case of hypotension, consider anaphylaxis as a cause. Following surgery, MC mediator release may still occur in the next 24 h.7 Therefore, patients should restart their regular antimediators as soon as possible and monitored carefully. Whereas this algorithm is based on clinical guidelines, published evidence, and the authors' experience, future prospective controlled studies on peri-operative hypersensitivity reactions are needed to further improve peri-operative management of mastocytosis patients. Dr. Bocca-Tjeertes has nothing to disclose. Dr. van de Ven has nothing to disclose. Prof dr. Koppelman reports grants from Lung Foundation of the Netherlands, GSK, TEVA the Netherlands, Ubbo Emmius Foundation, TETRI foundation, Vertex, European Union (H2020 program), outside the submitted work; and he has participated in advisory boards to GSK and PURE-IMS (Money to institution, outside the submitted work). Dr. Sprikkelman reports grants from Aimmune, outside the submitted work. Dr. Oude Elberink has nothing to disclose.