Angioimmunoblastic lymphoma (AIL) is an aggressive T-cell lymphoma representing about 1-2% of all types of Non-Hodgkin's Lymphoma in the US. It has in the past been called Angioimmunoblastic Lymphadenopathy With Dysproteinemia (AILD). Initially was thought to represent an abnormal reaction of the immune system but it is now known to be a form of lymphoma.
Presentation
AIL is a type of peripheral T-cell lymphoma that is clinically characterized by high fever, night sweats, weight loss, skin rash, a positive Coomb’s test, polyclonal hypergammaglobulinemia, and generalized lymphadenopathy that sometimes has cutaneous (skin) involvement.
This malignancy usually occurs in adults. Patients are usually aged 40-90 years (median around 65) and are more often male.
As AIL progresses, hepatosplenomegaly, hemolytic anemia, and polyclonal hypergammaglobulinemia may develop. The skin is involved in approximately 40-50% of patients. Other symptoms can include weight loss, hepatomegaly, polyclonal hyperglobulinemia, and generalized adenopathy (lymph swelling).
Treatment
Although steroid therapy initially is beneficial in many patients, the disease usually progresses to another form of lymphoma (e.g., to high-grade T-cell immunoblastic lymphoma or Epstein-Barr virus positive diffuse large b-cell lymphoma).
Stem Cell transplantation has been selectively used for advanced cases.
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Resources
Angioimmunoblastic Lymphadenopathy With Dysproteinemia - e-Medicine
Non-Hodgkin's Lymphoma - US National Cancer Institute
Non-Hodgkin's Lymphoma – oncologychannel
Abstract: Long-term disease-free survival in patients with angioimmunoblastic T-cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation. Schetelig J et al. Haematologica. 2003 Nov; 88(11): 1272-8
Abstract: Angioimmunoblastic T-cell lymphoma. Ferry JA, Adv Anat Pathol. 2002 Sep; 9(5): 273-9
The Hematopathology of Lymphoma
Abstract: Peripheral T-cell lymphoma (excluding anaplastic large-cell lymphoma): results from the Non-Hodgkin's Lymphoma Classification Project. Rudiger T. et al. Ann Oncol. 2002 Jan; 13(1): 140-9.
Non-Hodgkin's Lymphoma for Professionals - US National Cancer Institute
Related Articles
Non-Hodgkin's Lymphoma Information Pages:
Non-Hodgkin's Lymphomas
Non-Hodgkin's Lymphoma: Diagnosis
Non-Hodgkin's Lymphoma: Aggressive Lymphomas
Non-Hodgkin's Lymphoma: Indolent Lymphomas
Non-Hodgkin's Lymphoma: Treatment
Non-Hodgkin's Lymphoma: Resources
Bone Marrow and Stem Cell Transplants
Books
Non-Hodgkin's Lymphomas, Peter M. Mauch (Editor), James O. Armitage (Editor), et al., 2004.
Section IV: Pathology, Biology, Clinical Evaluation, and Treatment Section
Chapter 26: Mature Nodal and Extranodal T-Cell and Non-Hodgkin's Cell Lymphomas (including Angioimmunoblastic Lymphoma)
Excellent but Technical:
An Illustrated Guide to Skin Lymphomas, Cerroni, Gatter, Kerl, 2nd. Ed, 2005
Chapter 8 Other Cutaneous T-Cell Lymphomas
Angioimmunoblastic T-Cell Lymphoma pp 86-88
