Case Presentation: A 22 year old man, originally from Guatemala, presented with recurrent episodes of right sided facial swelling associated with superficial oral ulcers and change in voice. He did not have any family history of angioedema and no known past history. At initial presentation, physical exam was significant for diffuse swelling of the right side of the face with remarkable swelling of both lips and periorbital region. He also had few superficial painful oral ulcers. Flexible laryngoscopy showed moderate supraglottic edema. Labs showed mild normocytic normochromic anemia, mild lymphopenia and mild hyper eosinophilia. Anti-nuclear antibody (ANA), Anti-neutrophil cytoplasmic antibody (ANCA), C1 esterase inhibitor, C1q, C2, C3, C4 levels were repeatedly normal. HIV serology was negative, serology for (herpes simplex virus) HSV-1 Ig G was positive, but HSV culture was negative. Initially he was empirically treated with steroids and acyclovir for presumed HSV infection. Despite, he presented with recurrent similar episodes and CT Neck revealed significant level II cervical lymphadenopathy, which on biopsy showed Natural Killer (NK)/T cell lymphoma, nasal type. Subsequent bone marrow biopsy did not show any atypical infiltrates. He was started on SMILE regimen chemotherapy which he tolerated for 5 cycles. His angioedema improved right after chemotherapy. Unfortunately, he developed neutropenic sepsis following sixth cycle of chemotherapy and he succumbed to his disease from septic shock and multi-organ failure.
Discussion: Acquired angioedema secondary to lymphoproliferative diseases or autoimmune diseases are known and in such cases, usually C1 esterase inhibitor level or functional activity of C 1 esterase inhibitor will be low along with decreased C2, C4 and C1q levels. Even though, we do not have functional assay of C1 esterase inhibitor activity for our patient, it would have been normal if tested, given normal C4 levels. All though, hereditary angioedema with normal C1 esterase inhibitor level and complement levels has been described, to the best of our knowledge, there is only one case of acquired angioedema secondary to a splenic marginal zone lymphoma with normal C1 esterase inhibitor and C4 levels was reported .
Conclusions: Adult onset angioedema should prompt physicians to evaluate for possible lymphoproliferative disorders, especially if the C1 esterase inhibitor levels are normal. Further molecular studies would be necessary to understand the pathophysiology of acquired angioedema with normal C1 esterase inhibitor levels and normal C2, C4 and C1q levels.
To cite this abstract:Sreenivasan, J; Baig, MA; Ayub, M; Ranka, S; Joshi, U; Lau, K; Moinuddin, S. Rare presentation of Natural Killer/T cell lymphoma as acquired angioedema. Abstract published at Hospital Medicine 2018; April 8-11; Orlando, Fla. Abstract 865. https://www.shmabstracts.com/abstract/rare-presentation-of-natural-killer-t-cell-lymphoma-as-acquired-angioedema/. Accessed July 22, 2019.