Systemic mastocytosis is a rare disorder characterised by tissue infiltration of morphologically abnormal mast cells and has been associated with severe anaphylactoid reactions during general anaesthesia. We report the case of a 43-year-old woman who developed a severe anaphylactoid reaction to iodinated contrast media. Persistently elevated serum tryptase levels led to further evaluation and the eventual diagnosis of systemic mastocytosis. This case highlights the importance of repeated measurements of serum tryptase levels following severe anaphylactoid reactions. The anaesthetist should also be aware of the propensity of these patients to develop severe anaphylactoid reactions during general anaesthesia and use treatment strategies to minimise this risk.
IshizakaT., ConradD.H., SchulmanE.S., SterkA.R., KoC.G., IshizakaK.IgE-mediated triggering signals for mediator release from human mast cells and basophils. Fed Proc1984; 43: 2840–2845.
5.
DesboroughJ.P., TaylorI., HattersleyA., GardenA., WolffA., BloomS.R.Massive histamine release in a patient with systemic mastocytosis. Br J Anaesth1990; 65: 833–836.
6.
HoskingM.P., WarnerM.A.Sudden intraoperative hypotension in a patient with asymptomatic urticaria pigmentosa. Anesth Analg1987; 66: 344–346.
7.
VaughanS.T., JonesG.N.Systemic mastocytosis presenting as profound cardiovascular collapse during anaesthesia. Anaesthesia1998; 53: 804–807.
8.
LarocheD., Aimone-GastinI., DuboisF., HuetH., GerardP., VergnaudM.C.Mechanisms of severe, immediate reactions to iodinated contrast material. Radiology1998; 209: 183–190.
9.
EscribanoL., AkinC., CastellsM., OrfaoA., MetcalfeD.D.Mastocytosis: current concepts in diagnosis and treatment. Ann Hematol2002; 81: 677–690.
10.
ValentP., AkinC., SperrW.R., HornyH.P., ArockM., LechnerK.Diagnosis and treatment of systemic mastocytosis: state of the art. Br J Haematol2003; 122: 695–717.
11.
FlorianS., KrauthM.T., Simonitsch-KluppI., SperrW.R., Fritsche-PolanzR., SonneckK.Indolent systemic mastocytosis with elevated serum tryptase, absence of skin lesions, and recurrent severe anaphylactoid episodes. Int Arch Allergy Immunol2005; 136: 273–280.
12.
KrishnaswamyG., KelleyJ., JohnsonD., YoungbergG., StoneW., HuangS.K.The human mast cell: functions in physiology and disease. Front Biosci2001; 6: D1109–D1127.
13.
PayneV., KamP.C.Mast cell tryptase: a review of its physiology and clinical significance. Anaesthesia2004; 59: 695–703.
CastellsM.C., IraniA.M., SchwartzL.B.Evaluation of human peripheral blood leukocytes for mast cell tryptase. J Immunol1987; 138: 2184–2189.
16.
LarocheD., VergnaudM.C., SillardB., SoufarapisH., BricardH.Biochemical markers of anaphylactoid reactions to drugs. Comparison of plasma histamine and tryptase. Anesthesiology1991; 75: 945–949.
17.
SchwartzL.B.Diagnostic value of tryptase in anaphylaxis and mastocytosis. Immunol Allergy Clin North Am2006; 26: 451–463.
18.
ValentP., SperrW.R., SchwartzL.B., HornyH.P.Diagnosis and classification of mast cell proliferative disorders: delineation from immunologic diseases and non-mast cell hematopoietic neoplasms. J Allergy Clin Immunol2004; 114: 3–11; quiz 12.
19.
ParrisW.C.V., ScottH.W., SmithB.E.Anesthetic management of systemic mastocytosis: experience with 42 cases. Anesth Analg1986; 65: S117.
20.
BorgeatA., RuetschY.A.Anesthesia in a patient with malignant systemic mastocytosis using a total intravenous anesthetic technique. Anesth Analg1998; 86: 442–444.
MackseyL.F., WhiteB.Anesthetic management in a pediatric patient with noonan syndrome, mastocytosis, and von Willebrand disease: a case report. AANA Journal2007; 75: 261–264.
24.
ParrisW.C.V., SandidgeP.C., PetrinelyG.Anesthetic management of mastocytosis. Anesthesiol Rev1981; 8: 32–35.
25.
ScottH.W., ParrisW.C., SandidgeP.C., OatesJ.A., RobertsL.J.Hazards in operative management of patients with systemic mastocytosis. Ann Surg1983; 197: 507–514.
26.
SmithG.B., GusbergR.J., JordanR.H., KimB.Histamine levels and cardiovascular responses during splenectomy and splenorenal shunt formation in a patient with systemic mastocytosis. Anaesthesia1987; 42: 861–867.
27.
ColemanM.A., LiberthsonR.R., CroneR.K., LevineF.H.General anesthesia in a child with urticaria pigmentosa. Anesth Analg1980; 59: 704–706.
JamesP.D., KrafchikB.R., JohnstonA.E.Cutaneous mastocytosis in children: anaesthetic considerations. Can J Anaesth1987; 34: 522–524.
30.
VilleneuveV., KaufmanI., WeeksS., DeschampsA.Anesthetic management of a labouring parturient with urticaria pigmentosa. Can J Anaesth2006; 53: 380–384.
31.
MetcalfeD.D.The treatment of mastocytosis: an overview. J Invest Dermatol1991; 96: 55S–56S; discussion.
32.
DaeronM., SterkA.R., HirataF., IshizakaT.Biochemical analysis of glucocorticoid-induced inhibition of IgE-mediated histamine release from mouse mast cells. J Immunol1982; 129: 1212–1218.
33.
WorobecA.S.Treatment of systemic mast cell disorders. Hematol Oncol Clin North Am2000; 14: 659–687, vii.
34.
HoranR.F., ShefferA.L., AustenK.F.Cromolyn sodium in the management of systemic mastocytosis. J Allergy Clin Immunol1990; 85: 852–855.
35.
MossJ., RosowC.E.Histamine release by narcotics and muscle relaxants in humans. Anesthesiology1983; 59: 330–339.
