What is Primary Cutaneous Anaplastic Large Ccell Lymphoma?
Primary cutaneous anaplastic large cell lymphoma is a type of skin cancer that usually appears as a single raised lesion larger than 2 cm, though multiple areas can also be affected. The cancer cells are confined to the skin but may occasionally invade deeper tissues. While this condition generally has a favorable prognosis, accurate diagnosis is crucial, as it can resemble other skin disorders or lymphomas.
Disease Cause
The exact cause isn’t fully understood, but it involves abnormal growth of T-cells.
Signs and Symptoms
The signs and symptoms of primary cutaneous anaplastic large cell lymphoma include:
- It primarily affects adults, with occurrences in children and teenagers being rare.
- The condition presents as large, red skin lesions, lumps, or tumours that develop gradually.
- These lesions can appear in one or more locations on the skin.
- The affected areas may cause itching or develop into sores.
Diagnosis
To diagnose primary cutaneous anaplastic large-cell lymphoma (pcALCL), doctors typically follow these steps:
- Medical History and Skin Examination: Review the patient’s medical history and conduct a thorough skin examination.
- Skin Biopsy: Analyse a skin sample for the presence of large, irregular CD30-positive cells.
- Genetic Testing: Examine cancer cells for specific gene alterations using a gene mutation test.
- Complete Blood Count: Perform a complete blood count to assess overall health and blood levels.
- Imaging Techniques: Use CT scans, MRI, or PET scans to check for the spread of cancer.
- Lymph Node Biopsy: If lymph nodes are swollen, test a sample for the presence of cancer cells.
- Bone Marrow Biopsy: Check if the cancer has spread to the bone marrow.
Treatment Options
The management of primary cutaneous anaplastic large-cell lymphoma generally involves:
- Surgery or Radiotherapy: For patients with one or a few skin nodules, removing the cancer cells through surgery or radiotherapy is often effective.
- Treatment for Advanced Disease: In more advanced or metastatic cases, targeted therapy with Brentuximab vedotin can be used, though it may cause nerve-related side effects.
- Other Drug Options: Medications like Pralatrexate, Bexarotene, or Methotrexate may also be prescribed.
- Chemotherapy: While CHOP chemotherapy is available, it is rarely used due to its potential to cause rapid relapse and significant side effects.
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