Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature
Acquired angioedema due to C1-inhibitor deficiency (AAE-C1INH) is a rare condition characterized by the localized swelling of the deeper skin layers and mucous membranes, especially the face, lips, tongue, throat, and gastrointestinal tract. AAE-C1INH is strongly associated with lymphoproliferative...
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| Format: | Article |
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SAGE Publishing
2025-07-01
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| Series: | Journal of Primary Care & Community Health |
| Online Access: | https://doi.org/10.1177/21501319251360500 |
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| author | Juan Carlos Cardenas Rosales Ahmad Ridwan Carlos Ruiz-Orasma Santiago F. Galeano-Lovera Fernando Gil Lopez Jhonny Perusina Jacqueline D. Squire Liuyan Jiang Muhamad Alhaj Moustafa Dana M. Harris Salma Iftikhar Bala Munipalli |
| author_facet | Juan Carlos Cardenas Rosales Ahmad Ridwan Carlos Ruiz-Orasma Santiago F. Galeano-Lovera Fernando Gil Lopez Jhonny Perusina Jacqueline D. Squire Liuyan Jiang Muhamad Alhaj Moustafa Dana M. Harris Salma Iftikhar Bala Munipalli |
| author_sort | Juan Carlos Cardenas Rosales |
| collection | DOAJ |
| description | Acquired angioedema due to C1-inhibitor deficiency (AAE-C1INH) is a rare condition characterized by the localized swelling of the deeper skin layers and mucous membranes, especially the face, lips, tongue, throat, and gastrointestinal tract. AAE-C1INH is strongly associated with lymphoproliferative disorders, although it can also be linked to autoimmune conditions, solid tumors, infections, or even occur without an identifiable cause. We present the case of a 45-year-old female patient with complaints of recurrent abdominal pain, bloating, and joint swelling. Laboratory testing showed decreased C1q and C4 complement levels, and C1 esterase inhibitor levels, indicative of AAE-C1INH. Further work up confirmed a diagnosis of extranodal marginal zone lymphoma with involvement of the bone marrow and spleen. Treatment with rituximab led to resolution of angioedema symptoms and almost complete remission of underlying lymphoma. This case underscores the importance of evaluating an underlying lymphoproliferative disorder in AAE-C1INH. Therefore, the early participation of a multidisciplinary team including specialists in immunology, hematology, and oncology is necessary for appropriate management. |
| format | Article |
| id | doaj-art-b51f25bc7574444086dec7ee137d92ff |
| institution | DOAJ |
| issn | 2150-1327 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Journal of Primary Care & Community Health |
| spelling | doaj-art-b51f25bc7574444086dec7ee137d92ff2025-08-20T02:46:37ZengSAGE PublishingJournal of Primary Care & Community Health2150-13272025-07-011610.1177/21501319251360500Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of LiteratureJuan Carlos Cardenas Rosales0Ahmad Ridwan1Carlos Ruiz-Orasma2Santiago F. Galeano-Lovera3Fernando Gil Lopez4Jhonny Perusina5Jacqueline D. Squire6Liuyan Jiang7Muhamad Alhaj Moustafa8Dana M. Harris9Salma Iftikhar10Bala Munipalli11Mayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAMayo Clinic, Jacksonville, FL, USAAcquired angioedema due to C1-inhibitor deficiency (AAE-C1INH) is a rare condition characterized by the localized swelling of the deeper skin layers and mucous membranes, especially the face, lips, tongue, throat, and gastrointestinal tract. AAE-C1INH is strongly associated with lymphoproliferative disorders, although it can also be linked to autoimmune conditions, solid tumors, infections, or even occur without an identifiable cause. We present the case of a 45-year-old female patient with complaints of recurrent abdominal pain, bloating, and joint swelling. Laboratory testing showed decreased C1q and C4 complement levels, and C1 esterase inhibitor levels, indicative of AAE-C1INH. Further work up confirmed a diagnosis of extranodal marginal zone lymphoma with involvement of the bone marrow and spleen. Treatment with rituximab led to resolution of angioedema symptoms and almost complete remission of underlying lymphoma. This case underscores the importance of evaluating an underlying lymphoproliferative disorder in AAE-C1INH. Therefore, the early participation of a multidisciplinary team including specialists in immunology, hematology, and oncology is necessary for appropriate management.https://doi.org/10.1177/21501319251360500 |
| spellingShingle | Juan Carlos Cardenas Rosales Ahmad Ridwan Carlos Ruiz-Orasma Santiago F. Galeano-Lovera Fernando Gil Lopez Jhonny Perusina Jacqueline D. Squire Liuyan Jiang Muhamad Alhaj Moustafa Dana M. Harris Salma Iftikhar Bala Munipalli Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature Journal of Primary Care & Community Health |
| title | Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature |
| title_full | Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature |
| title_fullStr | Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature |
| title_full_unstemmed | Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature |
| title_short | Acquired Angioedema as the First Sign of Lymphoproliferative Disorder: Case Report and Review of Literature |
| title_sort | acquired angioedema as the first sign of lymphoproliferative disorder case report and review of literature |
| url | https://doi.org/10.1177/21501319251360500 |
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