Abstract

MOURA, Ana Luísa et al. Recurrent peripartum anaphylaxis as a manifestation of systemic mastocytosis. Rev Port Imunoalergologia [online]. 2020, vol.28, n.4, pp.241-246. ISSN 0871-9721.  https://doi.org/10.32932/rpia.2020.12.047.

Mastocytosis is a rare disease associated with high risk for severe anaphylactic reactions. Case description: Female patient that developed severe anaphylactic shock during a caesarian delivery at 27 years-old. The drugs administered in the perioperative period were lidocaine, ropivacaine, oxytocin, cefazolin and fentanyl. Skin tests were positive only to oxytocin (immediate reaction). One year later, a red-brown, pruriginous, maculopapular rash with Darier’s sign was noted in the torso and legs. The skin biopsy showed mast cells in the dermis, consistent with mastocytosis. On a second caesarian section, she had another episode of anaphylaxis with cardiorespiratory failure and generalized convulsions. The diagnosis of systemic mastocytosis was based on following criteria (WHO 2016): (1) basal serum tryptase levels 40,1 mg/L; (2) abnormal morphology of mast cells in bone marrow; and, (3) expression of CD25 on mast cells. Conclusion: The diagnosis of mastocytosis requires high clinical suspicion, particularly in cases of perioperative anaphylaxis.

Keywords : Anaphylactic shock; mast cell activation syndrome; perioperative anaphylaxis; systemic mastocytosis.

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