What is Angioimmunoblastic lymphadenopathy?

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder characterized by diffuse lymphadenopathy, fever, hepatosplenomegaly, hemolytic anemia, and polyclonal hypergammaglobulinemia.

What is the cause of Angioimmunoblastic?

Suspected risk factors include several viruses including the Epstein-Barr virus, cytomegalovirus, hepatitis C virus, human herpes viruses 6 and 8, and the human immunodeficiency virus. Certain infectious agents including tuberculosis and Cryptococcus have also been linked to AITL.

What are the symptoms of Angioimmunoblastic T-cell lymphoma?

Symptoms of AITL include high fever, night sweats, skin rash, and autoimmune disorders such as autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). As a result of these autoimmune disorders, the body’s immune system attacks its own cells and tissues, such as red blood cells (AIHA) or platelets (ITP).

What is the survival rate for AITL?

AITL can occasionally spontaneously remit, though almost never does so permanently. The complete remission rate with CHOP is 64% with a median survival of 19 months. The 5 year overall survival is 32% with a 5 year failure free survival of 18%. Survival is dependent on the International Prognostic Index (IPI) score.

How is lymphoma caused?

Doctors aren’t sure what causes lymphoma. But it begins when a disease-fighting white blood cell called a lymphocyte develops a genetic mutation. The mutation tells the cell to multiply rapidly, causing many diseased lymphocytes that continue multiplying.

What is Dermatopathic lymphadenopathy?

Dermatopathic lymphadenopathy is characterized by expansion of the lymph node paracortex with numerous Langerhans cells and macrophages containing phagocytosed melanin. In infiltrated lymph nodes, aggregates of small to medium-sized lymphoid cells with cerebriform nuclei are seen in the paracortex.

Is AITL curable?

In general, relapsed lymphoma remains potentially curable; however, required approaches are significantly more aggressive and therefore riskier than the initial treatment. AITL cure remains elusive in the majority of patients.

What’s the meaning of Angioimmunoblastic T-cell lymphoma?

Angioimmunoblastic lymphoma is a type of peripheral T-cell lymphoma. It is a high grade (aggressive) lymphoma that affects blood cells called T cells. It is also called angioimmunoblastic T-cell lymphoma (AITL). High grade lymphomas tend to grow more quickly than low grade lymphomas.

What does Angioimmunoblastic T-cell lymphoma mean?

Listen to pronunciation. (AN-jee-oh-IH-myoo-noh-BLAS-tik T-sel lim-FOH-muh) An aggressive (fast-growing) type of T-cell non-Hodgkin lymphoma marked by enlarged lymph nodes and hypergammaglobulinemia (increased antibodies in the blood).

How do you treat Angioimmunoblastic lymphoma?

For most patients with AITL, the initial goal of treatment is curative; therefore, an aggressive approach with combination chemotherapy followed often by consolidation with autologous stem-cell transplant (ASCT) is used.

What can lymphoma be mistaken for?

Alcoholism.