ALTERNATE NAMES

AILD; AITL; Angioimmunoblastic Lymphadenopathy with Dysproteinemia; Immunoblastic Lymphadenopathy; Lymphogranulomatosis

DESCRIPTION

Angioimmunoblastic T-Cell Lymphoma (AITL) is a rare and highly aggressive cancer of the lymphatic system. AITL spreads throughout the body by converting T-cell lymphocytes (immune cells that dispose of bacteria and viruses) into malignant cells.

AITL is unique among non-Hodgkin lymphomas for its disabling effect on the immune system; the symptoms that arise from the dysfunctional immune response are often more severe than those caused by the tumor itself.

The cause of AITL is poorly understood, although there appears to be a correlation with Epstein-Barr virus infection.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing:

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  Blood chemistry analysis;

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  Biopsy of lymph nodes and bone marrow;

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  Computed tomography (CT) scan; and

• •

  Positron emission tomography (PET) scan

Physical findings: Symptoms of AITL include:

• •

  Fever;

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  Weight loss;

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  Excessive sweating, especially at night;

• •

  Swollen lymph nodes;

• •

  Skin rash, with or without itching; and

• •

  Fatigue.

ICD-9: 202.7

ICD-10: C86.5

PROGRESSION

Although many patients with AITL will achieve remission with the initial course of treatment, most will relapse. Median survival is 2-3 years from initial diagnosis.

TREATMENT

Bone marrow transplant has shown to lead to more positive outcomes in patients with AITL than standard radiation and chemotherapy regimens. Corticosteroids are used to control symptoms related to immune dysfunction.

DETERMINATION

LISTING

REMARKS

Meets

Equals

13.05 D

AITL equals 13.05 D even when bone marrow transplant surgery is performed.