Abstract
Angioedema is a recurrent, non-pitting, non-pruritic, transitory swelling due to transient increase of endothelial permeability in the capillaries of the deep cutaneous and mucosal layers. Angioedema is generally categorized based on etiology, and characteristic lab findings are associated with each category. Cases of acquired angioedema associated with myeloproliferative disorders have been described in the literature, but these have been associated with a characteristic low C1q, a defining laboratory finding in acquired angioedema. Here we present a case of 68-year-old female with acquired angioedema that was not associated with low C1q, but was found to have Waldenström disease. Her angioedema responded dramatically to combination therapy consisting of bortezomib, rituximab, and dexamethasone.
References
Treon SP, Ioakimidis L, Soumerai JD, Patterson CJ, Sheehy P, Nelson M, et al. Primary therapy of Waldenström macroglobulinemia with bortezomib, dexamethasone, and rituximab: WMCTG clinical trial 05–180. J Clin Oncol. 2009;27(23):3830–5.
Richardson PG, Sonneveld P, Schuster MW, et al. Bortezomib or high-dose dexamethasone for relapsed multiple myeloma. New Engl J med. 2005;352:2487–98.
Zuraw B. Clinical practice: hereditary angioedema. N Engl J Med. 2008;359:1027–36.
Cicardi M, Zanichelli A. Acquired angioedema. Allergy Asthma Clin Immunol. 2010;6(1):14.
Zingale LC, Castelli R, Zanichelli A, Cicardi M. Acquired deficiency of the inhibitor of the first complement component: presentation, diagnosis, course, and conventional management. Immunol Allergy Clin North Am. 2006;26(4):669–90.
Caldwell JR, Ruddy S, Schur PH, Austen KF. Acquired C1 inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol. 1972;1:39–52.
Cicardi M, Zingale LC, Pappalardo E, Folcioni A, Agostoni A. Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies. Medicine (Baltimore). 2003;82(4):274–81.
Branellec A, Bouillet L, Javaud N, Mekinian A, Boccon-Gibod I, Blanchard-Delaunay C, et al. Acquired C1-inhibitor deficiency: 7 patients treated with rituximab. J Clin Immunol. 2012;32(5):936–41.
Tournilhac O, Santos DD, Xu L, Kutok J, Tai YT, Le Gouill S, et al. Mast cells in Waldenström’s macroglobulinemia support lymphoplasmacytic cell growth through CD154/CD40 signaling. Ann Oncol. 2006;17(8):1275–82.
Conflict of interest
The authors declare that they have no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Khanfar, A., Trikha, A., Bonds, R. et al. Angioedema with normal C1q and C1 inhibitor: an atypical presentation of Waldenström macroglobulinemia. Int J Hematol 97, 654–656 (2013). https://doi.org/10.1007/s12185-013-1330-z
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12185-013-1330-z