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Angioimmunoblastic T-Cell Lymphoma

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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.  It is now a distinct entity under the newer WHO classification system

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.

Further Reading / In-Depth

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

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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

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This page is a work in progress - if you have more complete information, references, or other information please contact the author. The author is not in the medical field and does not warrant the correctness of the material on this page or the sites linked - please take online information and consult with your own medical team to make informed decisions.

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Updated October 30, 2005

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