Angioedema due to Acquired C1-Inhibitor Deficiency Associated With Monoclonal Gammopathies of Undetermined Significance Characteristics of a French National Cohort.
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Lahuna Constance, Defendi Federica, Bouillet Laurence, Boccon-Gibod Isabelle, Mekinian Arsene, Coppo Paul, Adamski Henri, Amarger Stephanie, Armengol Guillaume, Aubineau Magali, Bibes Beatrice, Blanchard-Delaunay Claire, Blaison Gilles, Brihaye Benoit, Cathebras Pascal, Caubet Olivier, Demoreuil Claire, Desblache Julien, Durupt Francois, Gayet Stephane, Gondran Guillaume, Hadjadj Jerome, Kalmi Galith, Kanny Gisele, Lacoste Marion, Launay David, Ly Kim Heang, McAvoy Chloé, Martin Ludovic, Ollivier Yann, Pelletier Fabien, Robbins Aylsa, Roos-Weil Damien, Fain Olivier, Gobert Delphine
Аннотация
OBJECTIVE: To describe the biological and clinical characteristics, evolution, and response to treatment of MGUS-associated AAE-C1-INH.
MATERIALS AND METHODS: We conducted a French national retrospective observational study on MGUS-associated acquired angioedema spanning a 30-year period.
RESULTS: Forty-one patients with MGUS-associated AAE-C1-INH at diagnosis were included; 68% displayed anti-C1-INH antibodies. The monoclonal component was an IgM in 24 patients, IgG in 11, and IgA in 6 patients. The mean age at first angioedema attack was 63 years (standard deviation [SD] = 13 years) and at diagnosis 66 years (SD = 11 years). A total of 88% patients benefited from acute attack treatments, and 77% from long-term prophylaxis, either danazol, tranexamic acid, or lanadelumab. Median follow-up was 7 years, during which 14 patients (33%) evolved into well-defined malignant hemopathies. Fifty percent of patients were given a hematological treatment, either rituximab alone, indicated by recurrent attacks of angioedema in patients with AAE-C1-INH with anti-C1-INH antibodies, or validated combinations of chemotherapies, indicated by evolution into a lymphoma in 7 patients and a myeloma in 3 patients. Fifteen patients (35%) were in clinical complete remission of angioedema at last visit, of whom 60% had an undetectable serum monoclonal immunoglobulin.
CONCLUSIONS: Complete remission of AAE-C1-INH is correlated to complete remission of the underlying hematological malignancy, as defined by an undetectable serum monoclonal immunoglobulin. In our MGUS-associated acquired angioedema cohort, we recorded an incidence of evolution into hematological malignancy of 4% per patient-year. It is therefore crucial to conduct full hematological workup during follow-up at an annual rate, and earlier if AAE relapses or if acute attack frequency increases.