Radiation as a trigger of attacks in a misdiagnosed ...

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with hereditary angioedema and Hodgkin's disease. Understanding triggers associated with angioedema a acks can help towards a be er recogni>on of ...
Radiation as a trigger of attacks in a misdiagnosed patient with hereditary angioedema and Hodgkin’s disease Maria Palasopoulou, Gerasimina TsinG, Anastasios E Germenis, Mafhaios Speletas Department of Immunology & HistocompaKbility, School of Health Sciences, Faculty of Medicine, University of Thessaly, Larissa, Greece

BACKGROUND Understanding triggers associated with angioedema afacks can help towards a befer recogniGon of impending afacks and the implementaGon of prevenGve behavioral or treatment measures [1].

CONCLUSION Coincidence of C1-INH-HAE with Hodgkin’s lymphoma has not been reported as yet. However, the emergence of lymphoma in this paGent cannot be considered unrelated to such a longstanding administraGon of corGcosteroids [3]. RadiaGon and nivolumab treatment are reported as triggering factors of angioedema afacks for a first Gme in the literature. Finally, the reported case highlights the fact that awareness about angioedema remains sGll very confined at least in some areas. The low level of awareness can result not only in significant delays in diagnosis that aggravate paGents’ quality of life, but, even worse, in misdiagnoses leading to serious complicaGons of inappropriate treatment.

References 1.  ZOTTER Z, CSUKA D, SZABÓ E, CZALLER I, NÉBENFÜHRER Z, TEMESSZENTANDRÁSI G, FUST G, VARGA L, FARKAS H. The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficincy. Orphanet J Rare Dis 2014; 9:44. 2.  GOODMAN A, PATEL SP, KURZROCK R. PD-1-PD-L1 immune-checkpoint blockade in B-cell lymphomas. Nat Rev Clin Oncol 2017; 14:203-220. 3.  CARBONE A, SPINA M, GLOGHINI A, TIRELLI U. Classical Hodgkin's lymphoma arising in different host's condiGons: pathobiology parameters, therapeuGc opGons, and outcome. Am J Hematol 2011; 86:170-179.

CASE PRESENTATION A 34-year-old woman presented to our OutpaGent Clinic on December 2012 with a typical history of recurrent angioedema afacks of various severity since her age of 14 years. A mean frequency of 2.5 afacks per year was reported with involvement of her hands, face, neck, tongue (twice) and abdomen. Stress and infecGons had been recognized as triggering factors of these afacks. The clinical picture had been afributed to food allergy and, although ineffecGve, corGcosteroids were administered systemaGcally, conGnuously at least for the last 10 years. C1-INH-HAE was diagnosed amer the measurement of C4, anGgenic and funcGonal C1-inhibitor levels and the diagnosis was confirmed by SERPING1 genotyping that revealed a novel, de novo missense mutaGon (c.239C>G, p.A80G). On demand treatment with human C1-INH (Berinert®) or icaGbant (Firasyr®) resulted in successful control of the afacks and great improvement of the paGent’s quality of life. A second pregnancy, 8 months amer diagnosis, aggravated the frequency and the severity of afacks but terminated normally by on demand administraGon of human C1-INH. Twenty-one months amer the diagnosis of angioedema, the paGent suffered from a cervical lymphadenopathy and a diagnosis of Hodgkin’s lymphoma was performed. The lymphoma was proved refractory to an ABVD regimen (doxorubicin, bleomycin, vinblasGne, and dacarbazine) administered iniGally along with radiotherapy due to local disease as well as to the administraGon of nivolumab that followed. Nivolumab, recently approved for the treatment of Hodgkin’s refractory lymphoma, is a fully human IgG4 monoclonal anGbody directed against the negaGve immunoregulatory human cell surface receptor programmed death-1 (PD-1, PCD-1) with immune checkpoint inhibitory and anGneoplasGc acGviGes. Thus, PD-1 blockade with nivolumab results in response rates as high as 87% in paGents with relapsed and/or refractory Hodgkin’s lymphoma [2]. Lymphoma diagnosis was followed by exacerbaGon of angioedema. Especially, radiotherapy sessions were triggering severe edema afacks at the sites of radiaGon, a fact that necessitated the prophylacGc administraGon of human C1-INH before each session. Furthermore, the administraGon of nivolumab resulted also in exacerbaGon of angioedema afacks, especially in abdomen.