What is Primary Cutaneous Large B-cell Lymphoma?
Primary Cutaneous Large B-cell Lymphoma (PCLBCL) is a rare type of non-Hodgkin lymphoma that starts in the B-cells of the immune system and primarily affects the skin. Unlike other B-cell lymphomas, it doesn’t usually affect other areas of the body at the time of diagnosis. This lymphoma is categorized into different subtypes, with the most common being the leg type and others including the follicle center lymphoma type and the intravascular large B-cell lymphoma type.
Symptoms
The symptoms of Primary Cutaneous Large B-cell Lymphoma include:
- Reddish or purple nodules or tumours on the skin, often on the legs but can appear anywhere on the body.
- The lesions may be painless and can vary in size.
- In some cases, the skin lesions might ulcerate or become painful.
- Systemic symptoms like fever, night sweats, and weight loss are less common in primary cutaneous forms compared to other types of lymphoma.
Disease Aetiology (Causes)
The exact cause of Primary Cutaneous Large B-cell Lymphoma is not well understood. Genetic factors and immune system dysregulation may play a role in its development, but specific environmental or lifestyle risk factors have not been conclusively identified.
Diagnosis
Diagnosing Primary Cutaneous Large B-cell Lymphoma involves:
- Skin Biopsy: A sample from the affected skin is essential for a definitive diagnosis.
- Immunophenotyping: To characterize the types of cells involved in the lymphoma.
- Imaging Studies: While PCLBCL is primarily a skin disease, imaging studies such as CT scans may be conducted to rule out internal involvement.
- Blood Tests: To assess overall health and look for signs of lymphoma.
Treatment
Treatment for Primary Cutaneous Large B-cell Lymphoma varies based on the type and extent of the disease:
- Radiotherapy: Often used for localized lesions.
- Chemotherapy: For more extensive skin involvement or for aggressive subtypes.
- Rituximab: A monoclonal antibody therapy, either alone or in combination with chemotherapy.
- Surgical Removal: In some cases, the lesions may be surgically excised.
- Skin-Directed Therapies: Such as topical corticosteroids or phototherapy, for less aggressive forms.
What support can we give for Primary Cutaneous Large B-cell Lymphoma?
Primary Cutaneous Large B-cell Lymphoma is a 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 Primary Cutaneous Large B-cell Lymphoma, 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:
