Angioimmunoblastic T-Cell Lymphoma

Angioimmunoblastic T-cell lymphoma (AITL) is a rare and often fast-growing form of peripheral T-cell lymphoma (PTCL). It accounts for about 20-30% of PTCLs and is more common in older people (median age at diagnosis is 65 years), though it can affect young adults as well.

Symptoms

Symptoms of AITL include:

• Fatigue
• High fever
• Night sweats
• Skin rash
• Autoimmune disorders (group of diseases where the immune system attacks the body’s own healthy tissues), such as autoimmune hemolytic anemia (AIHA; the body immune system attacks its own red blood cells) and immune thrombocytopenia (ITP; the body immune system attacks its own platelets).

Diagnosis

Diagnosing AITL requires taking a biopsy (sample of the tumor tissue) and looking at the cells under a microscope. A series of other tests may be done to determine the extent, or stage, of the disease. These can include blood tests, a computed tomography (CT) scan, a positron emission tomography (PET) scan, a magnetic resonance imaging (MRI) scan, and bone marrow biopsy.

This means that the disease has grown and/or affects many areas of the body (Figure 1) and requires treatment. Less-extensive disease, Stage I (localized disease that has not spread beyond one lymph node or other location) or Stage II (disease has spread only to nearby lymph nodes), is rare in AITL. In Stage III, affected lymph nodes are found both above and below the diaphragm (the muscle that separates the chest from the abdomen). In Stage IV, one or more organs beyond the lymph nodes are affected, such as the bone, bone marrow, skin, or liver.

Prognosis

A new prognostic tool (predicts how well the patient will do) called the AITL Score was recently developed to predict the outcome in patients with AITL. The AITL Score categorizes patients by level of risk (low-, intermediate- and high-risk). The risk categories are determined by age, Eastern Cooperative Oncology Group (ECOG) performance status (a scale of 0 to 5 that describes the patient’s ability to take care of themselves and perform daily activities, like walking or working), levels of C-reactive protein (CRP, a protein made in the liver in response to inflammation or tissue damage) and β2 microglobulin (a protein that is increased in some types of cancer, like lymphoma).

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