Lymphocytic lymphoma and acquired C1 esterase inhibitor deficiency

Abstract

We report a 59-year-old woman who had progressive symptoms of angioedema for 3 years, culminating in an attack of laryngeal stridor. C1 esterase inhibitor deficiency was confirmed and treatment with tranexamic acid produced considerable clinical benefit and improvement in complement levels. Two years later she developed clinical evidence of lymphocytic lymphoma. Splenectomy resulted in rapid correction of complement abnormalities. The patient received 6 months of chemotherapy after surgery and remained asymptomatic with normal complement levels 4 years later.

Angioedema of the Tongue Due to Acquired C1 Esterase Inhibitor Deficiency

“…Prior to elective surgery, the attenuated androgens danazol and stanozolol act to increase the hepatic production of C1 esterase inhibitor. Danazol appears to be safe and effective but levels of C2, C4 and C1q may remain low despite treatment 13 . It has been used for long-term prophylaxis of hereditary angioedema, but treatment had been associated with hepatocellular carcinoma 14 .…”

Acquired C 1-inhibitor deficiency with angioedema due to pleomorphic immunocytoma in a patient with three malignant tumors: long-term follow-up data and presentation of an additional case

“…In our patients, concomitant hematological abnormalities and splenomegaly relatively promptly led to the correct diagnosis. However, very often the correct diagnosis was made only after a latency period of several years [9]. Probably, in many of the patients reported with acquired C 1-inhibitor deficiency without underlying disease [3], neoplasm will be diagnosed during follow-up.…”

Angioedema in the emergency department: A presentation of lymphoma

“…Waldenstroms macroglobulinemia is a low‐grade B cell lymphoproliferative disorder characterized by an IgM paraprotein, classified as lymphoplasmacytic lymphoma in the recent World Health Organization classification 5 . Waldenstroms and acquired C1 esterase inhibitor deficiency is a rare association, with only a few cases being reported 6,7 …”