Clinical manifestation of angioimmunoblastic T-cell lymphoma with exuberant plasmacytosis

Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of non-Hodgkin lymphoma characterized by aggressive symptoms and various abnormal laboratory test results. One of the rare immunologic abnormalities in AITL is exuberant polyclonal plasmacytosis, but its clinical significance has not been evaluated. This report concerns three AITL cases with exuberant polyclonal plasmacytosis and investigates its clinical impact by comparison with 12 patients without plasmacytosis. Our study found that the performance status (PS) of the former was significantly worse and their serum immunoglobulin levels were significantly higher. All other parameters, including B symptoms, various prognostic scores, blood cell counts other than plasmacyte, and serum levels of lactate dehydrogenase, C-reactive protein and soluble interleukin-2 receptor, showed no significant differences. More importantly, although the diagnosis of AITL with plasmacytosis was not straightforward in our series, outcomes of treatment with conventional chemotherapy or immunosuppressive therapy with cyclosporine A were favorable. To conclude, AITL should be considered a candidate underlying disease of exuberant polyclonal plasmacytosis. Provided a correct diagnosis is made early and is followed by adequate treatment, the prognosis for AITL with plasmacytosis may not be worse than that for those without plasmacytosis despite the severe exhaustion at first presentation.

Angioimmunoblastic T-Cell Lymphoma with Coexisting Plasma Cell Myeloma: A Case Report and Review of the Literature

“…The reactive plasmacytes commonly exhibited a CD19 + , CD38 + , CD138 -/+ , CD10 -, CD20 -, and CD56 -immunophenotype (Sakai et al 2007;Yamane et al 2007;Ahsanuddin et al 2011;Nagoshi et al 2013). In addition, 4 of 12 patients demonstrated proliferation of monoclonal plasma cells that were relatively mature in morphology; that originated from EBV-negative clones; and that were always associated with AITL lesions at the lymphoma site, but not in the bone marrow (Zettl et al 2002;Balague et al 2007;Huppmann et al 2013).…”

“…However, the potential mechanism involved in the development and progression of the concomitant plasma cell proliferation remains to be clarified. High levels of cytokines, such as IL-6, IL-10 and tumor necrosis factor-α, may serve as possible contributing factors (Balague et al 2007;Sakai et al 2007;Yamane et al 2007;Ahsanuddin et al 2011;Huppmann et al 2013;Nagoshi et al 2013). …”

“…Histopathologically, AITL derives from a unique subset of T cells which are called the follicular helper T (TFH) cells, and displays distinctive features that are characterized by effacement of the lymph node architecture, a paracortical polymorphic infiltrate, the proliferation of arborizing high endothelial venules (HEVs) and follicular dendritic cells (FDCs), and occasional background expansion of Epstein-Barr virus (EBV)-harboring B immunoblasts (Dogan et al 2003(Dogan et al , 2008Dunleavy et al 2007). Recently, several research groups focused on the behavior of plasma cells in AITL, which ranged from reactive plasmacytosis to striking clonal proliferation (Balague et al 2007;Sakai et al 2007;Yamane et al 2007;Ahsanuddin et al 2011;Huppmann et al 2013;Nagoshi et al 2013). However, the role of the plasma cell as a component of the microenvironment in AITL and the functional interaction between plasma cells and tumor cells have not yet been deciphered (Gaulard and de Leval 2014).…”

Reactive Plasmacytosis at the Onset of Angioimmunoblastic T-Cell Lymphoma. Case Report

“…Прогностическое значение реактивного плазмо-цитозаприАИТЛдосихпорнеясно.Вкрупномяпонскомретроспективномисследованиипрогностических факторов при АИТЛ поликлональный плазмоцитоз не рассматривался из-за своей редкости в качестве факторапрогноза [26].Вдругойработеяпонскихколлег,описывающей15больныхАИТЛ,у3изкоторых наблюдалсяреактивныйплазмоцитоз,былопоказано, что пролиферация поликлональных плазматических клетоксопряженасболеевысокойактивностьюлактатдегидрогеназы, высоким уровнем С-реактивного белкаисывороточныхиммуноглобулинов.Уостальных 12 больных без реактивного плазмоцитоза был отмеченболеевысокийсоматическийстатусименее выраженысимптомыинтоксикации.Эффективность стандартноголеченияубольныхАИТЛвгруппахсреактивнымплазмоцитозомибезнегобылаодинаковой [27].Внашемслучаенаблюдалиcьвыраженныесимптомы интоксикации, низкий соматический статус, высокаяактивностьлактатдегидрогеназыиревматоидного фактора, высокий уровень С-реактивного белка,сывороточныхиммуноглобулинов,циркулирующихиммунныхкомплексов,анемия(до43г/л),тромбоцитопения. Противоопухолевая терапия позволила достигнутьдлительнойполнойремиссии.…”

Angioimmunoblastic T-Cell Lymphoma: Clinical Aspects and Recent Advances in Biology and Therapy

“…Usual manifestations also involve pruritic skin rash, hepatosplenomegaly, and generalized lympha-denopathy [5][6][7]. Hematological features may include autoimmune hemolytic anemia, thrombocytopenia and polyclonal hypergammaglobulinemia [8,9]. Furthermore, bone marrow involvement is reported in up to 70% of the cases and it tends to correlate with B symptoms, hepatosplenomegaly, abnormal laboratory findings, and higher levels of circulating tumor cells [10].…”