Anaplastic large cell lymphoma (ALCL) is a rare form of non-Hodgkin lymphoma. Like all non-Hodgkin lymphoma, ALCL is a type of cancer. With non-Hodgkin lymphoma, white blood cells called lymphocytes grow out of control. Lymphocytes are part of your immune system. They help protect your body from germs and diseases.
Lymphocytes include two main types of cells: B-cells (B lymphocytes) and T-cells (T lymphocytes). In ALCL, your T lymphocytes grow abnormally. The condition's name — anaplastic large cell lymphoma — describes how the abnormal T lymphocytes look when viewed under a microscope.
Anaplastic: “Anaplastic” is a word used to describe odd-looking cancer cells. The cancer cells in ALCL look abnormal when compared to healthy T lymphocytes.
Large cell: The abnormal T lymphocytes appear larger than normal T lymphocytes when viewed under a microscope.
Lymphoma: Lymphoma is cancer that involves the out-of-control growth of white blood cells called lymphocytes. ALCL forms from T lymphocytes.
What are the symptoms of anaplastic large cell lymphoma?
Symptoms vary depending on the type of ALCL.
Systemic ALCL
Both ALK-positive and ALK-negative ALCL often cause swollen lymph nodes at the site where the cancer’s growing. The most common areas are your neck, underarm and groin. Other symptoms include:
Fever.
Fatigue.
Night sweats.
Unexplained weight loss.
Most people are diagnosed when the cancer is more advanced. In advanced stages, the cancer may have spread to organs, like your lungs, liver and bone. You may experience symptoms based on which body parts are affected. For example, pressure in your chest and a frequent cough may signify the presence of the ALCL in your chest.
Primary cutaneous ALCL
Primary cutaneous ALCL often appears as a single abnormal growth or multiple growths on a single area of your skin. The most common sites are your face, abdomen, buttocks, arms and legs. Rarely (about 20% of the time), growths appear in multiple places. Symptoms include:
Abnormal reddish or reddish-brown growths that get bigger over time.
Large (often bigger than a quarter), raised bumps that may be itchy.
Growths that form ulcers and scab over.
BIA-associated ALCL
BIA-associated ALCL often causes noticeable breast changes. Symptoms include:
Breast pain.
Breast swelling.
Skin rash.
Swelling or a collection of fluid near your implant.
Solid mass (lump) near your implant.
Usually, just one breast changes, but you may notice changes in both breasts.
What causes anaplastic large cell lymphoma (ALCL)?
Anaplastic large cell lymphoma forms when lymphocytes multiply out of control and begin to overtake surrounding healthy tissue. Researchers haven’t yet found what causes lymphocytes to become malignant (cancerous). Still, they’ve identified several genetic mutations that frequently occur with ALCL. These mutations may cause healthy cells to transform into cancer cells.
For example, mutations in the ALK gene are the defining feature of ALK-positive ALCL. Other types of gene mutations are common with other forms of ALCL.
How is anaplastic large cell lymphoma treated?
Chemotherapy is the standard treatment for systemic ALCL. Surgery is the preferred treatment for primary cutaneous ALCL and BIA-ALCL.
Systemic ALCL
ALK-positive and ALK-negative anaplastic large cell lymphoma usually respond well to systemic chemotherapy. With systemic chemotherapy, drugs that destroy cancer cells are given in a vein and are distributed through your bloodstream to your body, killing cancer cells.
Chemotherapy treatments for systemic ALCL often include a combination of drugs that work together to destroy cancer cells. Drug combinations include:
BV-CHP: Drugs include brentuximab vedotin, cyclophosphamide, doxorubicin (hydroxydaunorubicin) and prednisone.
CHOP: Drugs include cyclophosphamide, doxorubicin (hydroxydaunorubicin), vincristine (Oncovin®) and prednisone.
CHEOP: Drugs include cyclophosphamide, doxorubicin (hydroxydaunorubicin), etoposide, vincristine (Oncovin®) and prednisone.
These treatments usually eliminate all signs of cancer (remission) in the short term. Still, the cancer may return. If your physician believes it’s likely that your cancer will return, they may recommend a stem cell transplant while you’re in remission. During a stem cell transplant, your healthcare provider replaces the cells destroyed during chemotherapy with healthy cells. These cells develop into healthy (noncancerous) blood cells.
If the cancer returns, your provider may recommend various additional chemotherapy treatments designed to treat lymphoma.
Primary cutaneous ALCL
Surgery is the most common treatment for primary cutaneous ALCL. You may need radiation therapy if the cancer has spread to your lymph nodes. Radiation directs energy toward the cancer cells, destroying them.
Primary cutaneous ALCL often returns within five years of treatment. You may need additional surgery and radiation therapy if it comes back.
You may need other forms of treatment if the localized tumors can’t be removed with surgery or if your cancer keeps returning. Treatments may include:
Oral methotrexate: A drug designed to treat non-Hodgkin lymphoma.
Bexarotene: A drug designed to treat T-cell lymphomas affecting your skin.
Brentuximab vedotin: A drug designed to treat systemic ALCL and other forms of ALCL that return.
Interferon: A drug that helps your immune system recognize and fight cancer cells, including lymphoma.
BIA-ALCL
Surgery to remove your implant and the surrounding cancer cells usually cures BIA-ALCL. If surgery isn’t an option, you may receive radiation therapy to destroy the cancer cells.
If the cancer returns or spreads beyond your breast, your healthcare provider may recommend chemotherapy treatments used to treat ALK-negative ALCL. Treatments may include CHOP chemotherapy and brentuximab vedotin.
How is anaplastic large cell lymphoma diagnosed?
Your healthcare provider will perform a physical exam to check for signs of ALCL, such as swelling in your lymph nodes. If they suspect ALCL, they’ll perform various tests and procedures.
Imaging: Imaging procedures allow your provider to identify the cancer’s location. The type of imaging you’ll need depends on the type of ALCL. A healthcare provider may perform a chest X-ray, CT scan or MRI to identify tumors. A PET/CT or PET scan can show if the cancer has spread throughout your body. A provider may perform a breast ultrasound if they suspect BIA-ALCL.
Bloodwork: A healthcare provider may perform various blood tests, like a complete blood count (CBC), to check for signs of ALCL. An abnormal amount of blood cells (red blood cells, white blood cells or platelets) may indicate ALCL. They may also check your blood for enzymes and other markers that may be signs of ALCL.
Biopsy: A biopsy is the only way to confirm an ALCL diagnosis. During a biopsy, your healthcare provider removes a tissue sample and examines the cells under a microscope. Studying the cells allows your provider to determine the type of ALCL and helps them plan the most effective treatments.
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