Lymphomatoid granulomatosis (LYG) is a rare blood vessel disease in which white blood cells are overproduced and cause lesions that destroy the arteries and veins.
LYG is most commonly found in the lung although also in the brain, kidney, liver, skin, and central nervous system.
LYG’s white blood cells contain the Epstein-Barr virus. LYG is also known as: Benign Lymph Angiitis and Granulomatosis, Malignant Lymph Angiitis and Granulomatosis, Pulmonary Angiitis, or Pulmonary Wegener’s Granulomatosis
Risk Factors
LYG is a rare disease, affecting less than 200,000 people in the United States. It is more common in males and in individuals over the age of 50. Patients with immunodeficiencies are at a higher risk of developing LYG.
Outlook
LYG, when it does occur, is typically fatal. The mortality rate is more than 60% within five years. The median survival is 14 months. Patients typically die of respiratory failure.
Prevention
Little can be done to prevent this disease; however, avoiding the Epstein-Barr Virus (human herpesvirus 4) is one preventative measure.
Signs & Symptoms
The following symptoms could be a sign of other ailments; however, severe and prolonged respiratory concerns in particular warrant deeper investigation into LYG.
- Fever
- Cough, often with blood
- Nose bleeds, stuffy nose, pain, perforated nasal septum
- Weight loss
- Seizures
- Hypertension
- Breathing difficulties/respiratory distress
- Chest pain
- Diarrhea
- Muscle and joint pain
- Skin spots, nodules, and/or ulcers
Other Signs & Symptoms
- Pneumonia
- Respiratory failure
- Malignant lymphoma
- Metastasis
Associated Disorders
B-cell Lymphoma (a form of non-Hodgkinís lymphoma)
Diagnosis & Staging
A proper diagnosis is likely to involve a biopsy, typically of the lung. There are two main types of LYG: B-lymphocytes and T-lymphocytes. The former tries to neutralize microorganisms, while the latter tries to destroy them.
Tests
- Chest radiographs
- CT scan
- Brain imaging
- Tissue biopsy
Overview of Staging
Staging of LYG is relatively irrelevant since the disease is fatal in nature.
Stages of Lymphomatoid Granulomatosis
LYG has three grades: I, II, and III. While the first two stages are non-cancerous, the latter is a subtype of large B-cell lymphoma.
Treatment
Some patients require no treatment and go into spontaneous remission; however, no therapeutic standards exist for LYG. LYG is also often resistant to chemotherapy.
Common Treatment Options
Treatment for LYG is generally restricted to radiation therapy or chemotherapy. More than half of patients seem to respond to corticosteroids, although recurrence is usual. If the disease is localized, radiation treatment can be useful. Surgery and chemotherapy has helped some patients.
Other Treatment Options
- Bone marrow transplants
- Interferon-alfa 2b treatments
- Antiviral therapy
- Rituximab
