LYMPHOMATOID GRANULOMATOSIS - GRADE
III
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ALTERNATE NAMES
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Lymphomatoid Granulomatosis High Grade; Polymorphic Reticulosis; Lymph Angiitis and
Granulomatosis; Malignant Lymph Angiitis and Granulomatosis; Pulmonary Angiitis; Lymphoproliferative
Disease; LG High Grade; LG
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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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:
Skin findings may include:
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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Focal motor and sensory complaints; and
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ICD-9:
202.8
ICD-10: C86.0
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PROGRESSION
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Grade III LG is usually progressive and fatal.
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TREATMENT
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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%.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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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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Surgical procedure notes; and
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.05 A
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Grade III LG meets the criteria in listing 13.05 A upon confirmed diagnosis and without
regard to effectiveness of treatment.
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113.03
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Grade III LG meets the criteria in listing 113.03 upon confirmed diagnosis and without
regard to effectiveness of treatment.
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113.05 A
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Grade III LG meets the criteria in listing 113.05 A upon confirmed diagnosis and without
regard to effectiveness of treatment.
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Equals
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13.13 A 1
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Medically equals the criteria in listing 13.13 A 1 if grade III LG affects only the
central nervous system.
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113.13
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Medically equals the criteria in listing 113.13 if grade III LG affects only the central
nervous system.
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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