Program: Oral and Poster Abstracts
Session: 622. Non-Hodgkin Lymphoma: Biology, excluding Therapy: Poster I
Patients and Methods: Seventy-two AAE patients were included.The diagnosis was based on a history of recurrent angioedema , which began during or after the fourth decade of life, absence of family history of angioedema, and detection of C1-INH functional levels below 50% of normal.Serum or plasma samples were stored at -80°C until tested. C1-INH, C4, C3, and C1q antigens were measured by radial immunodiffusion. C1-INH antigenic and C4 were quantified using radial immunodiffusion or nephelometry; C1-INH function was measured using an immunoenzimatic assay. Autoantibodies to C1-INH in serum were measured by enzyme-linked immunosorbent assay Hematoxyllin–eosin stained slides were reviewed. Lymphoprolipherative disorders were classified according to the WHO classification.
Results: Overall, 33.3% (24/72) of AAE patients had an underlying B-cell NHL. Most NHL (62,5%, 15/24) were diagnosed at onset of AAE or thereafter (3 months to 7 years), while in the remaining cases were diagnosed before the onset of AEE symptoms. According to the WHO classification, 29% (21/72) patients had indolent NHL and 4% (3/72) aggressive NHL (1 diffuse large B-cell lymphoma and 2 mantle cell lymphoma).Among patients with indolent NHL, most had splenic MZL (71%, 15/21), while the remaining had had LPL (n=3), SLL (n=2), or follicular lymphoma (n=1) .Serological evidence of hepatitis C virus infection was reported in 1 of 24 patients. Autoantibodies to C1-INH were detected in 54% (13/24) patients, including 12/15 ( 80%) of cases with a diagnosis of SMZL.Of the 20 patients with indolent B cell lymphoprolipherative disease 13/20 received systemic therapy. Chemotherapy was performed in 13/24 patients (2 R-CHOP,1 Rituximab –fludarabine,1 Chlorambucil, 5 Bendamustine R, 2 R-CVP, 2 CFX/Prednisone).Splenectomy without chemotherapy was performed in 3; 2 patients were treated with Rituximab alone. Thirteen patients experienced complement improvement or reduction in AAE symptoms after chemotherapy. Four patients received no therapy.
Conclusions: Our series is the largest reported to date and confirms that SMZL represents the most common histotype among AAE patients, with a frequency of 75% of INFBCL (15/20 indolent lymphoprolipherative disease) and of 62.5% of all lymphoprolipherative diseases identified.The post-germinal center origin of most of NHLs suggests that immune stimulation may contribute to lymphomagenesis.
Disclosures: No relevant conflicts of interest to declare.
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