LYMPHOMATOID GRANULOMATOSIS - GRADE III

Lymphomatoid Granulomatosis High Grade; Polymorphic Reticulosis; Lymph Angiitis and Granulomatosis; Malignant Lymph Angiitis and Granulomatosis; Pulmonary Angiitis; Lymphoproliferative Disease; LG High Grade; LG

Lymphomatoid Granulomatosis (LG) is a rare, progressive malignant neoplastic disease in adults and children (although it is most common in people in the fifth and sixth decade), where nodular lesions destroy blood vessels. In addition, the lungs are usually affected. LG is composed of B-cells positive for Epstein Barr Virus and mixed with reactive T-cells. It often occurs in association with an underlying immunodeficiency state such as rheumatoid arthritis, organ transplantation, and human immune deficiency virus (HIV) infection. A grading system from Grade I to Grade III for LG is based on the number of atypical lymphocytes, EBV-positive B-cells and amount of tissue destruction (necrosis). The advanced form (grade III) of LG is approached clinically as a subtype of diffuse large B-cell non-Hodgkin lymphoma (NHL). The clinical features reflect systemic multi-organ disease with lung, skin, and central nervous system involvement.

Diagnostic testing: Pathologic examination of tissue biopsy confirms the diagnosis. Imaging includes bone and chest X-rays, lymphangiography, CT scan, and MRI.

Physical findings: Pulmonary findings may include:

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  Cough;

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  Shortness of breath; and

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  Chest pain.

Skin findings may include:

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  Small red bumps;

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  Lumps under the skin;

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  Ulcers;

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  Thickened patches; or

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  Inflamed hair follicles.

Skin lesions usually do not cause symptoms but they can be tender or itchy.

Neurological findings may include:

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  Cranial and peripheral nerve defects resulting in unsteadiness, blurred vision, weakness or numbness affecting facial muscles or hands and feet;

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  Seizures;

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  Altered cognition;

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  Focal motor and sensory complaints; and

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  Stroke syndromes.

ICD-9: 202.8

Grade III LG is usually progressive and fatal.

There is no standard treatment for grade III LG, although surgery and chemotherapy may improve pain relief and neurological symptoms. Median survival is about 14 months; five-year survival is less than about 30%.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical history and examination that describe the diagnostic features of the impairment;

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  Results of imaging tests;

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  Biopsy results;

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  Surgical procedure notes; and

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  Pathology reports.

Suggested Listings for Evaluation:

REMARKS

Grade III LG meets the criteria in listing 13.05 A upon confirmed diagnosis and without regard to effectiveness of treatment.

Medically equals the criteria in listing 13.13 A 1 if grade III LG affects only the central nervous system.

Medically equals the criteria in listing 113.13 if grade III LG affects only the central nervous system.