Angioimmunoblastic lymphadenopathy with dysproteinemia: emphasis on pathogenesis and treatment

Acta Haematologica
S Sallah, G A Gagnon

Abstract

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder characterized by diffuse lymphadenopathy, fever, hepatosplenomegaly, hemolytic anemia, and polyclonal hypergammaglobulinemia. Morphologically, the involved lymph nodes demonstrate complete effacement of the normal architecture, prominent neovascularization and infiltration by immunoblasts and plasma cells. Other terms that have been used to describe this entity include diffuse plasmacytic sarcomatosis, immunoblastic lymphadenopathy, lymphogranulomatosis X, and immunologic aberrations in idiopathic reticulosis. Initially, AILD was thought to be a disease of B-cell origin that represented reactive immune response to unknown stimulus and high potential for malignant transformation. It is now evident that AILD in 80% of cases follows an aggressive course with short median survival, especially, if complete response with chemotherapy is not achieved. Immunologic and molecular studies have demonstrated that the majority of AILD cases are T-cell clonal disorders. Despite the numerous reports on the role of Epstein-Barr virus in this disorder, it is unknown whether the presence of this virus is associated with the immune defect that acco...Continue Reading

Citations

Feb 5, 2005·Respirology : Official Journal of the Asian Pacific Society of Respirology·Toshiyuki YamagataMasakazu Ichinose
Feb 19, 2005·International Journal of Hematology·Hitoshi OhnoYataro Yoshida
Jul 26, 2011·Leukemia & Lymphoma·Korinna JöhrensIoannis Anagnostopoulos
Jul 28, 2015·Current Medical Research and Opinion·Magali Van den BerghConstantin A Dasanu
Dec 18, 2013·Seminars in Ultrasound, CT, and MR·Carlos S RestrepoMicheal N Koss
Apr 3, 2012·Hematology/oncology Clinics of North America·Gabriela Motyckova, David P Steensma
Jan 2, 2007·Cancer Letters·Amit W Panwalkar, James O Armitage
Jul 22, 2004·The Oncologist·Jennifer R Brown, Arthur T Skarin
Oct 16, 2016·International Journal of Clinical Oncology·Helen Ma, Maher Abdul-Hay
Oct 8, 2005·Blood·Mujahid A RizviSteven T Rosen
Mar 25, 1999·Hospital Practice·D T ParnassaH M Adelman
Jul 6, 2004·Current Treatment Options in Oncology·Andrew M Evens, Ronald B Gartenhaus

❮ Previous
Next ❯

Related Concepts

Related Feeds

B cell Differentiation

Depending on the signal received through the B cell receptor and other receptors, B cells differentiate into follicular or marginal zone B cells. Here is the latest research pertaining to this differentiation process.