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Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency

Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE) is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI) tract m...

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Autores principales: Abdulkareem, Abdullateef, D'Souza, Ryan S., Mundorff, Joshua, Shrestha, Pragya, Shogbesan, Oluwaseun, Donato, Anthony
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5818943/
https://www.ncbi.nlm.nih.gov/pubmed/29545957
http://dx.doi.org/10.1155/2018/7809535
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author Abdulkareem, Abdullateef
D'Souza, Ryan S.
Mundorff, Joshua
Shrestha, Pragya
Shogbesan, Oluwaseun
Donato, Anthony
author_facet Abdulkareem, Abdullateef
D'Souza, Ryan S.
Mundorff, Joshua
Shrestha, Pragya
Shogbesan, Oluwaseun
Donato, Anthony
author_sort Abdulkareem, Abdullateef
collection PubMed
description Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE) is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI) tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. C1INH-AAE is typically under-diagnosed because of its rarity and its propensity to mimic more common abdominal conditions and allergic reactions. In this article, we present the case of a 62-year-old male with a history of recently diagnosed chronic lymphocytic leukemia (CLL) who presented to our hospital with recurrent abdominal pain, initially suspected to have Clostridium difficile colitis and diverticulitis. He received a final diagnosis of acquired angioedema due to C1 esterase inhibitor deficiency due to concomitant symptoms of lip swelling, cutaneous nonpitting edema of his lower extremities, and complement level deficiencies. He received acute treatment with C1 esterase replacement and icatibant and was maintained on C1 esterase infusions. He also underwent chemotherapy for his underlying CLL and did not experience further recurrence of his angioedema.
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spelling pubmed-58189432018-03-15 Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency Abdulkareem, Abdullateef D'Souza, Ryan S. Mundorff, Joshua Shrestha, Pragya Shogbesan, Oluwaseun Donato, Anthony Case Rep Hematol Case Report Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE) is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI) tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. C1INH-AAE is typically under-diagnosed because of its rarity and its propensity to mimic more common abdominal conditions and allergic reactions. In this article, we present the case of a 62-year-old male with a history of recently diagnosed chronic lymphocytic leukemia (CLL) who presented to our hospital with recurrent abdominal pain, initially suspected to have Clostridium difficile colitis and diverticulitis. He received a final diagnosis of acquired angioedema due to C1 esterase inhibitor deficiency due to concomitant symptoms of lip swelling, cutaneous nonpitting edema of his lower extremities, and complement level deficiencies. He received acute treatment with C1 esterase replacement and icatibant and was maintained on C1 esterase infusions. He also underwent chemotherapy for his underlying CLL and did not experience further recurrence of his angioedema. Hindawi 2018-01-10 /pmc/articles/PMC5818943/ /pubmed/29545957 http://dx.doi.org/10.1155/2018/7809535 Text en Copyright © 2018 Abdullateef Abdulkareem et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Abdulkareem, Abdullateef
D'Souza, Ryan S.
Mundorff, Joshua
Shrestha, Pragya
Shogbesan, Oluwaseun
Donato, Anthony
Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency
title Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency
title_full Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency
title_fullStr Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency
title_full_unstemmed Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency
title_short Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency
title_sort refractory abdominal pain in a patient with chronic lymphocytic leukemia: be wary of acquired angioedema due to c1 esterase inhibitor deficiency
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5818943/
https://www.ncbi.nlm.nih.gov/pubmed/29545957
http://dx.doi.org/10.1155/2018/7809535
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