Lymphomatoid Granulomatosis (LG) is a rare, progressive malignant neoplastic disease in adults and children (although
it is most common in people in the 5th and 6th 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, PHYSICAL FINDINGS, AND ICD-9-CM CODING
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 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, weakness or numbness affecting
facial muscles or hands and feet, seizures, altered cognition, focal motor and sensory
complaints, and stroke syndromes.
Suggested MER for evaluation: The adjudicator needs medical evidence from treating sources and hospitals. This
evidence may include clinical history and examination that describe the diagnostic
features of the impairment, imaging tests, biopsies, surgical procedures, and pathology