Abstract
BackgroundAcquired angioedema is a rare but recognized manifestation of lymphoproliferative disorders due to deficiency in C1 esterase inhibitor. Normal level of C1 esterase inhibitor proteins in association with angioedema due to lymphoproliferative disease is a rare and an uncommon finding caused by antibodies produced from the underlying disease. Antibodies cause inactivation of C1 esterase inhibitor, thus resulting in C1 esterase inhibitor dysfunction despite of normal quantity of C1 esterase inhibitor.Case presentationA 50-year-old Sri Lankan male presented with first episode of angioedema without any family history. Physical examination revealed mild pallor with swelling of tongue, lips and perioral region. On investigations, erythrocyte sedimentation rate was persistently high and bone marrow with immunohistochemistry revealed infiltration with B-cell type low grade non-Hodgkin lymphoma. Computed tomography scan of the chest and abdomen showed paratracheal and subcarinal lymphadenopathy and splenomegaly, with the findings being compatible with lymphoma. He had normal C1 esterase inhibitor protein level with reduced activity and low C1q, C4 levels indicating antibodies against C1 esterase inhibitor causing dysfunctional C1 esterase inhibitor.ConclusionAdult onset angioedema should prompt physicians to suspect underlying lymphoproliferative disorder despite of C1 esterase inhibitor protein level being normal. Though uncommon, presence of antibodies against C1 esterase inhibitor secondary to lymphoproliferative disorder should be considered in the presence of normal C1 esterase inhibitor protein levels with low functional capacity in the background of acquired angioedema.
Highlights
Acquired angioedema is a rare but recognized manifestation of lymphoproliferative disorders due to deficiency in C1 esterase inhibitor
Presence of antibodies against C1 esterase inhibitor secondary to lymphoproliferative disorder should be considered in the presence of normal C1 esterase inhibitor protein levels with low functional capacity in the background of acquired angioedema
It is a wellrecognized phenomenon associated with non-Hodgkin lymphoma (NHL) [5,6,7], commonly B cell type [1,8] as well as other lymphoproliferative disorders
Summary
This rare case of acquired angioedema as the first manifestation of non-Hodgkin lymphoma highlights the importance of having a high degree of suspicion to exclude occult malignancies or underlying disease in patients with adult onset angioedema. Consent Written informed consent was obtained from the patient for publication of this case report and any accompanying images. Authors’ contributions HW made the clinical diagnosis and supervised the manuscript drafting. SSCG drafted the first manuscript, reviewed the literature and was involved in direct management of the patient. All authors read and approved the final manuscript. Authors’ information HW (MBBS, MD, FRCP (Edin), FRCP (Lond), FCCP) is a Consultant Physician, Teaching Hospital, Kandy, Sri Lanka. SSCG (MBBS) is a Registrar in Medicine attached to the Teaching Hospital, Kandy, Sri Lanka
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