LYMPHOMATOID GRANULOMATOSIS - GRADE III

ALTERNATE NAMES

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

DESCRIPTION

Lymphomatoid granulomatosis (LG) is a rare, progressive malignant neoplastic disease in adults and children (although it is most common in individuals 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 (EBV) 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 (CNS) involvement.

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

Diagnostic testing: Pathologic examination of tissue biopsy confirms the diagnosis. Imaging includes bone and chest X-rays, lymphangiography, computed tomography (CT) scan, and magnetic resonance imaging (MRI).

Physical findings: Pulmonary findings may include:

• •

  Cough;

• •

  Shortness of breath; and

• •

  Chest pain.

Skin findings may include:

• •

  Small red bumps;

• •

  Lumps under the skin;

• •

  Ulcers;

• •

  Thickened patches; or

• •

  Inflamed hair follicles.

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

Neurological findings may include:

• •

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

• •

  Seizures;

• •

  Altered cognition;

• •

  Focal motor and sensory complaints; and

• •

  Stroke syndromes.

ICD-9: 202.8

ICD-10: C86.0

PROGRESSION

Grade III LG is usually progressive and fatal.

TREATMENT

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:

• •

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

• •

  Results of imaging tests;

• •

  Biopsy results;

• •

  Surgical procedure notes; and

• •

  Pathology reports.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

13.05 A

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

113.03

Grade III LG meets the criteria in listing 113.03 upon confirmed diagnosis and without regard to effectiveness of treatment.

113.05 A

Grade III LG meets the criteria in listing 113.05 A upon confirmed diagnosis and without regard to effectiveness of treatment

Equals

13.13 A 1

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

113.13

Medically equals the criteria in listing 113.13 if grade III LG affects only the CNS