Angioimmunoblastic T-Cell Lymphoma

Abstract

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.

Targeting intratumoral B cells with rituximab in addition to CHOP in angioimmunoblastic T-cell lymphoma. A clinicobiological study of the GELA

“…2 It is clinically characterized by widespread lymphadenopathy, and commonly, extranodal disease, immune-mediated hemolysis, and polyclonal hypergammaglobulinemia. 3 The recent identification of the follicular helper T cell (TFH) as the cell of origin of this lymphoma 4,5 represents a major step in the understanding of the clinicobiological characteristics of the disease. 6 This T-cell subset promotes positive selection, proliferation and differentiation of germinal center antigen-specific B cells.…”

Extranodal Marginal Zone Lymphoma–like Presentations of Angioimmunoblastic T-Cell Lymphoma

“…The majority of cases of AITL are associated with EBV, which can be detected with the use of in-situ hybridization studies (EBER) or immunohistochemistry 7,9,22–24 . Biologically, EBV enters the B-cells and CD21 positive follicular dendritic cells (FDC) and immortalizes them 25 .…”

“…Later, Watanabe et al 6 hypothesized that AITL was a proliferation of hyperactive B-cells. We now know that AITL is the second most common type of peripheral T-cell lymphoma (PTCL) worldwide, and in some countries the most common form 1,7–9 . Clinically, AITL usually presents with a constellation of findings that include diffuse lymphadenopathy, hepatosplenomegaly and constitutional symptoms (fever, chills, weight loss, etc).…”

Angioimmunoblastic T Cell Lymphoma: An Unusual Presentation of Posttransplant Lymphoproliferative Disorder in a Pediatric Patient

“…In a study of 77 patients, Lachenal et al [27 ]reported neurologic symptoms, including confusion, loss of hearing or vision, motor and sensory polyneuritis and aphasia, in 8 patients (10%). In 2 of these patients, tumor cells were identified in the CSF and lymphocytic meningoencephalitis was present in 1 patient.…”