Korean J Med.  2018 Feb;93(1):68-73. 10.3904/kjm.2018.93.1.68.

Successful Remission of Recurrent Anaphylaxis after Omalizumab Administration in a Patient with Systemic Mastocytosis

Affiliations
  • 1Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea. helenmed@snu.ac.kr
  • 2Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Korea.
  • 3Department of Laboratory Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.

Abstract

Mastocytosis is a disorder characterized by abnormal mast cell proliferation and accumulation in one or more tissues. It presents in two major variants: cutaneous mastocytosis and systemic mastocytosis. Because the symptoms are related to mast cells, histamine receptor antagonists and leukotriene receptor antagonists are recommended as therapeutic options. Here, we report a 54-year-old male patient with a history of urticaria pigmentosa who presented with recurrent anaphylaxis. His serum tryptase level was 31.7 ng/mL and mast cell infiltration was observed in his bone marrow. He had frequent attacks of anaphylaxis despite treatment with ketotifen, levocetirizine, and montelukast. Symptoms related to systemic mastocytosis were controlled and the patient exhibited no recurrence of anaphylaxis following the introduction of monthly omalizumab injection. Omalizumab can be considered as a treatment option in patients with systemic mastocytosis unresponsive to conventional oral medications.

Keyword

Mastocytosis; Systemic; Anaphylaxis; Omalizumab

MeSH Terms

Anaphylaxis*
Bone Marrow
Humans
Ketotifen
Leukotriene Antagonists
Male
Mast Cells
Mastocytosis
Mastocytosis, Cutaneous
Mastocytosis, Systemic*
Middle Aged
Omalizumab*
Receptors, Histamine
Recurrence
Tryptases
Urticaria Pigmentosa
Ketotifen
Leukotriene Antagonists
Omalizumab
Receptors, Histamine
Tryptases
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