What is Lymphomatiod Granulomatosis?
Lymphomatoid granulomatosis is an uncommon Epstein- Barr virus-associated disease, characterized by the excessive production of lymphocytes (white blood cells) which infiltrate or accumulate within the tissues in the form of nodules or lesions. These lesions cause the destruction of blood vessels in these tissues. Lungs are among the most commonly affected sites. Others, most commonly affected sites and organs are skin, liver, kidneys, brain, and spinal cord. Lymphomatoid granulomatosis is a characteristic type of B- cell lymphoma (cancer of white blood cells) which is triggered in response to the suppression of the immune system of the individual and as a result of infection with Epstein –Barr virus.
There is no exact well-known cause is found, however, it is more prevalent in individuals having HIV (Human immunodeficiency virus). It is also associated with Epstein –Barr virus infection.
It is a rare disease and its prevalence is unknown. It is mostly diagnosed or developed after the age of 50-60 years. It is more common in males with a male to female ratio is 2:1.
Signs and Symptoms
Signs and symptoms vary according to the involvement of organs. In most cases, it involves multiple organs of the body. Usually, lungs are most commonly affected while, less commonly involved sites are skin(50%), CNS(25%), liver, and kidney. In advance cases, it also spread to the lymph nodes, spleen, and bone marrow.
Respiratory Signs and Symptoms
The patient may suffer from the following signs and symptoms if lymphomatoid granulomatosis involving the lungs. These are.
- Coughing up blood or blood mixed with sputum
- Difficulty in breathing
Skin Signs and Symptoms
The patient may suffer from the following signs and symptoms if lymphomatoid granulomatosis involved the skin. These are.
- Painful skin lesions
- Erythema nodosum ( Red, painful lump or lesion)
Central Nervous System (Brain and Spinal cord) Signs and Symptoms
The patient may suffer from the following signs and symptoms if lymphomatoid granulomatosis involved the CNS. These are.
- Alteration in consciousness
- Loss of balance while standing or walking
- Slurred or stumbling speech
Liver Signs and Symptoms
The patient may suffer from the following signs and symptoms if lymphomatoid granulomatosis involved the Liver. These are.
- Painful enlargement of the liver
- Night sweats
- Weight loss
Diagnosis and Treatment
Diagnostic tests are:
- Complete blood count( CBC) with peripheral smear
- ESR ( Erythrocyte sedimentation rate)
- Liver function tests (LFTs)
- Renal Function tests (RFTs)
- Urine complete examination
- EBV test or EBV antibodies
- Chest –X-Ray, CT-Scan, MRI, and USG
- Biopsy of the suspected organ, skin biopsy in case of skin lesions
Treatment: Patients experiencing a benign course of the disease do not need treatment. Patients with benign lymphomatoid granulomatosis should be kept on observation. Corticosteroids may be highly suggested with or without chemotherapy. Treat symptomatic or progressive disease. Overall, treatment includes corticosteroids with anti-cancerous drugs (e.g., cyclophosphamide).
What Support can we Give for Lymphomatoid Granulomatosis?
Lymphomatoid Granulomatosis is rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. So, we provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Lymphomatoid Granulomatosis, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
You can help us with your donation:
Without a Ribbon is a charity that works hard to aid those who suffer from rare cancers. You can help our cause in a variety of ways:
Donations — Without a Ribbon is grateful when we receive every donation. Giving to Without a Ribbon helps us to provide ongoing support, organise the annual gathering and subsidise the costs of our Warriors attending these conferences.
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