Prognostic factors in angioimmunoblastic lymphadenopathy

Abstract

In order to identify prognostic factors in angioimmunoblastic lymphadenopathy (AIL), 30 directly diagnosed patients were prospectively followed for more than 42 months. Age and sex distribution, clinical and laboratory findings and evolution were not different from previously reported series. Median duration of survival was 24 months. Parameters associated with a longer survival in our series were localized adenopathies (P = 0.01) and the achievement of a remission (P < 0.0001). Features associated with a shorter survival included drug exposure in relation to the onset of the disease (P = 0.02), rash (P < O.OOOl), lymph node eosinophilia (P = 0.03) and elevated serum lactic dehydrogenase (P = 0.03). Drug exposure and rash were, however, significantly dependent (P = 0.02). In addition, lymphopenia, the presence of circulating immune complexes, and the absence of polyclonal hypergammaglobulinemia may indicate a poor prognosis, although the significance level is not achieved in this short series. None of the parameters tested was significantly related to the lymphomatous transformation of AIL, which occurred in four cases. It is concluded that multicentric prospective studies of AIL are necessary in order to better define this disorder, to find prognostic factors, and to optimize therapy. NGIOIMMUNOBLASTIC LYMPHADENOPATHY (AIL) isA a lymphoid disorder first described in 1974 by Frizzera et al. ' Despite its apparent histologic benignity,',* AIL usually behaves as a rather aggressive disease with frequent organ in~olvement,l-~ and median survival is only 30 months in the largest series published to date.5 Transformation of AIL into a true malignant lymphoma is observed in up to 18% of the case^.^,^ The evolution of the disease remains, however, rather unpredictable and up to 20% of the patients may undergo a spontaneous complete remissi~n.',~+~ The treatment of AIL is still controversial and various therapies have been recommended including initial abstention,'S2 predni~one,',~,~-~ and ~hemotherapy.~.~,'~ It would therefore be of great importance to define precise prognostic factors in order to optimize the therapeutic approach in each case. Due to the rare occurrence of the disease, only one series of more than 30 cases has been published.495 Most of the cases reported in the literature were diagnosed by the retrospective review of lymph node biopsy specimens from We present here the results of a statistical search for prognostic factors in our own series of 30 directly diagnosed and prospectively followed patients with a minimal follow-up of 42 months. Materials and MethodsOur series includes 30 patients in whom AIL was directly diagnosed between 1975 and 198 1. Angioimmunoblastic lymphadenopathy was diagnosed histologically on lymph node biopsy specimens obtained before treatment. All biopsy specimens were reviewed by one of us (P.A.B.) and fulfilled the morphologic criteria established by Frizzera et al.': diffuse obliteration of the nodal architecture by a lymphoid cellular infiltrate including lymphocytes, pl...

Heterozygosity for Roquinsan leads to angioimmunoblastic T-cell lymphoma-like tumors in mice

“…7,8 Roquin san/ϩ mice displayed a slight but significant (P Ͻ .05) increase in spleen weight compared with control Roquin ϩ/ϩ mice ( Figure larger than lymph nodes from Roquin ϩ/ϩ control mice (data not shown).…”

“…Sinusoidal dilatation was also evident, as well as vessels filled with T cells. A prominent feature present in most AITL cases, but absent in the tumors of Roquin san/ϩ mice, is prominent expansion of the FDC network, 3,7,8,27 as determined by an absence of CD21 ϩ dendritic cells in lymph node sections (data not shown). Together, this histologic appearance is highly reminiscent of AITL.…”

“…6 Patients commonly present with lymphadenopathy, hepatosplenomegaly, skin rash, fever, hemolytic anemia, and hypergammaglobulinemia. 7,8 Histologic features of AITL typically include effacement of the lymph node architecture, prominent arborization of endothelial venules, and an expanded extrafollicular meshwork of follicular dendritic cells (FDCs).…”

Clinicopathologic Characteristics of Angioimmunoblastic T-Cell Lymphoma: Analysis of the International Peripheral T-Cell Lymphoma Project

“…23, 24 The Kiel Lymphoma Study Group analyzed the prognostic impact of clinical observations and laboratory findings at presentation and found that survival was significantly related to age (P ϭ .032), stage (P ϭ .037), systemic symptoms (P ϭ .007), skin rash/pruritus (P ϭ .038), edema (P ϭ .030), ascites (P ϭ .013), high LDH (P ϭ .007), and decreased hemoglobin (P ϭ .020). 9 Archimbaud et al 24 identified prognostic factors associated with shorter survival, including rash (P Ͻ .001), lymph node eosinophilia (P ϭ .03), elevated LDH (P ϭ .03), and drug exposure in relation to the onset of the disease (P ϭ .02); parameters associated with longer survival were localized lymphadenopathy (P ϭ .01) and achievement of remission (P Ͻ .001).…”

“…9 Archimbaud et al 24 identified prognostic factors associated with shorter survival, including rash (P Ͻ .001), lymph node eosinophilia (P ϭ .03), elevated LDH (P ϭ .03), and drug exposure in relation to the onset of the disease (P ϭ .02); parameters associated with longer survival were localized lymphadenopathy (P ϭ .01) and achievement of remission (P Ͻ .001). In our study, an alternative prognostic model for AITL was constructed using the five clinical prognostic factors: age Ͼ 60 years, PS Ն 2, ENSs Ͼ one, B symptoms, and platelet count Ͻ 150 ϫ 10 9 /L.…”