<abstract xmlns="http://www.w3.org/1999/xhtml">

<p>Acquired angioedema due to C1-inhibitor deficiency is a very rare disorder that usually appears in patients with lymphoproliferative and/or autoimmune diseases. This type of swelling is bradykinin mediated and does not respond to antihistamines, corticosteroids, or epinephrine. The symptoms usually appear in patients older than 40 years with recurrent episodes of angioedema without wheals. The family history is negative. The swelling could affect any tissue, but most frequently is located at the face, lips, tongue, larynx, or extremities. In the gastrointestinal tract, it causes pain, nausea, vomiting, and diarrhea. The upper respiratory airway oedema is a potentially life-threatening condition due to asphyxiation. The oedema attacks may precede the symptoms of the causative disease for months or years. In most cases, the treatment of the underlying disease resolves the angioedema episodes. Here we report a case of C1-INH-AAE caused by non-Hodgkin lymphoma in a patient diagnosed many years before with myasthenia gravis whose angioedema symptoms resolved after the specific treatment of lymphoma.</p>
</abstract>

Acquired angioedema due to C1-inhibitor deficiency is a very rare disorder that usually appears in patients with lymphoproliferative and/or autoimmune diseases. This type of swelling is bradykinin mediated and does not respond to antihistamines, corticosteroids, or epinephrine. The symptoms usually appear in patients older than 40 years with recurrent episodes of angioedema without wheals. The family history is negative. The swelling could affect any tissue, but most frequently is located at the face, lips, tongue, larynx, or extremities. In the gastrointestinal tract, it causes pain, nausea, vomiting, and diarrhea. The upper respiratory airway oedema is a potentially life-threatening condition due to asphyxiation. The oedema attacks may precede the symptoms of the causative disease for months or years. In most cases, the treatment of the underlying disease resolves the angioedema episodes. Here we report a case of C1-INH-AAE caused by non-Hodgkin lymphoma in a patient diagnosed many years before with myasthenia gravis whose angioedema symptoms resolved after the specific treatment of lymphoma.

Acquired Angioedema Due to C1 inhibitor Deficiency Caused by Non-Hodgkin Lymphoma in a Patient with Myasthenia Gravis

George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Targu Mures

Department of Internal Medicine and Haematology

Revista Romana de Medicina de Laborator

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Acquired angioedema due to C1-inhibitor deficiency is a very rare disorder that usually appears in patients with lymphoproliferative and/or autoimmune...

Acquired Angioedema Due to C1 inhibitor Deficiency Caused by Non-Hodgkin Lymphoma in a Patient with Myasthenia Gravis

Publié en ligne: 22 oct. 2021

Pages: 453 - 456

Reçu: 07 sept. 2021

Accepté: 23 sept. 2021

DOI: https://doi.org/10.2478/rrlm-2021-0029

Mots clés
rare disease, bradykinin mediated angioedema, lymphoproliferative disease, autoimmune disease, angioedema attack

© 2021 Noémi Bara et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Acquired Angioedema Due to C1 inhibitor Deficiency Caused by Non-Hodgkin Lymphoma in a Patient with Myasthenia Gravis

Publié en ligne: 22 oct. 2021

Pages: 453 - 456

Reçu: 07 sept. 2021

Accepté: 23 sept. 2021

DOI: https://doi.org/10.2478/rrlm-2021-0029

Mots clésrare disease, bradykinin mediated angioedema, lymphoproliferative disease, autoimmune disease, angioedema attack

© 2021 Noémi Bara et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Mots clés
rare disease, bradykinin mediated angioedema, lymphoproliferative disease, autoimmune disease, angioedema attack