Angioimmunoblastic T-cell lymphoma: clinical and laboratory features at diagnosis in 77 patients.

Medicine (Baltimore)

From Hospices Civils de Lyon, Department of Internal Medicine (FL, ID, HR), Department of Pathology (FB), Department of Cytogenetic and Molecular Biology (ECB), and Department of Hematology (BC, GS), Centre Hospitalier Lyon Sud, Pierre-Bénite; Department of Internal Medicine (AH), Hospital Edouard Herriot, Lyon; Department of Hematology (HG, PB) and Department of Pathology (CC), Centre Léon Bérard, Lyon; and Université Claude Bernard Lyon 1 (FL, FB, AH, ECB, BC, ID, HR, GS), Lyon, France.

Published: September 2007

We retrospectively analyzed 77 patients with pathologically diagnosed angioimmunoblastic T-cell lymphoma from a single city. There were 43 men and 34 women; the median age was 64.5 years (range, 30-91 yr). Average time between first symptoms of the disease and diagnosis was 3.6 months. At diagnosis, peripheral nodes were present in all but 1 patient, and were generalized in 90% of cases. Constitutional symptoms were reported in 77% of cases and spleen enlargement in 51%. A cutaneous eruption--morbilliform, urticarial, or more polymorphic--was present in 45% of patients; in one-third of them, the eruption occurred after drug administration. Other clinical manifestations included pleuritis (22%); arthralgia or arthritis (17%); ear, nose, and throat involvement (14%); central or peripheral neurologic manifestations (10%); and ascites (5%). Most patients presented with advanced disease at diagnosis (bone marrow involvement in 60% of cases). The main laboratory abnormalities were elevated lactate dehydrogenase levels (71%), inflammatory syndrome (67%), hypergammaglobulinemia (50%), anemia (51%), and lymphopenia (52%). Auto- or disimmune manifestations were reported in one-third of patients: autoimmune hemolytic anemia was present at diagnosis in 19% of patients and thrombocytopenic purpura in 7%. Documented vasculitis was described in 12% of cases. Clonality was analyzed in lymph nodes in 47 patients: T-cell and B-cell clones were found in 45 (96%) and 20 (45%) patients, respectively. Chromosomal abnormalities were identified in 62% of cases: trisomies 3, 5, 18, 19, additional X chromosome, and deletion of chromosome 7 were the most common abnormalities. The current study underlines the diversity of presenting manifestations of angioimmunoblastic T-cell lymphoma.

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http://dx.doi.org/10.1097/MD.0b013e3181573059DOI Listing

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