Anaplastic Large Cell Lymphoma

What is BIA–ALCL?

BIA–ALCL is an abbreviation for Breast Implant-Associated Anaplastic Large Cell Lymphoma. A lymphoma is a cancer that affects the immune system. This particular lymphoma is referred to as “breast implant-associated” because it specifically affects patients who have, or at one time had, breast implants.  This description separates BIA-ALCL from ALCL (a non-implant-associated form of a similar lymphoma).

Anaplastic large cell lymphoma (ALCL) is a T-cell lymphoma, and a rare type of non-Hodgkin lymphoma. ALCL comprises approximately 16% of all T-cell lymphomas, and about 1% of all non-Hodgkin lymphomas. ALCL can initially appear either in the skin, the lymph nodes, or in organs throughout the body. In comparison, BIA-ALCL initially originates in the capsular tissue surrounding the breast implant.

BIA–ALCL is not breast cancer; it is not a cancer that originates in the breast tissue.  Because BIA–ALCL actually occurs in the capsular tissue surrounding the implant, related to abnormal white blood cells, it is classified as a lymphoproliferative disorder.

BIA–ALCL is a rare disease , consisting of a spectrum of diseases that can range from a slow developing and painless fluid collection around the breast implant (in the early-stage), to capsular tumors, to a potentially invasive and metastatic lymphoma (in the late stage). BIA-ALCL is not considered a benign disease at any stage. Ongoing research strives to better understand the entire disease process.

What is the risk for developing BIA–ALCL?

The risk of BIA–ALCL appears to only be associated with breast implants that have a textured surface.  At this time, there have been no documented cases of BIA–ALCL in patients with a history of only smooth surfaced breast implants.  This means that the risk for BIA–ALCL exists for any patient who has a textured surface breast implant in place now, or for any patient who has a history of having had a textured surface implant sometime in the past.  The risk of developing BIA–ALCL may be higher for implants that have a more aggressive surface texture.  The risk of developing BIA–ALCL does not vary depending upon the fill material and is the same for textured saline-filled or textured silicone gel-filled breast implants.  And BIA-ALCL can occur in either cosmetic or reconstructive patients.  Asian patients seemed to be significantly less likely to develop BIA–ALCL when compared to other ethnicities.

The risk of developing BIA–ALCL varies around the world.  Currently available information from the US, Canada, Netherlands, and Australia estimates the lifetime risk to be between (1/1,000) 1/3,817 and 1/30,000 with textured breast implants.  This range is due to the fact that this disease is not yet fully understood, and that there appears to be a genetic predisposition that causes some individuals to be more easily affected than others.

Another way to better understand this risk is to compare it to something more common. The relative risk of capsular counterculture is 100 – 3,000 times more common than BIA_ALCL.

What causes BIA-ALCL?

The exact course of BIA–ALCL is not well understood.  Researchers are intently studying this disease, but it appears that implant texturing, bacteriologic contamination (chronic inflammation), a genetic predisposition, and time likely all play an important role in the development of this disease.  It is thought that bacterial contamination of a textured surface implant, in patients who are genetically susceptible, can result in the development of BIA–ALCL.  A textured implant has a greater surface area than a smooth implant. The very small projections and depressions in this texturing provide increased surface area that allows for a higher number of bacteria around the implant.  These bacteria are more sequestered in this surface texturing and can lead to the formation of a biofilm. The biofilm is essentially a collection of bacteria, embedded in a slimy matrix of polymeric carbohydrates, proteins and lipids, that allow them to adhere to each other and the implant surface.  This biofilm stimulate a chronic inflammatory response and the proliferation of lymphocytes (white blood cells), which over time develops into anaplastic large cell lymphoma in some patients.  Researchers believe that there is a genetic predisposition that affects the development of BIA–ALCL.  Some at the cities/geographic areas of the world have very few reported cases, well BIA–ALCL is more prevalent in other areas/athletic populations.

There is a “time” element to the development of BIA–ALCL.  Because it is thought to develop from a chronic inflammatory reaction it does not occur shortly after implantation, but instead takes several years to manifest.  Cases of BIA–ALCL have occurred (have been diagnosed) between 2 – 28 years following surgery with the average time until presentation being about 8 years after surgery.

Currently, there is no test to screen for BIA–ALCL.

How common is BIA–ALCL?

BIA–ALCL is a rare disease.  As of February 2019, there have been 457 distinct cases of BIA–ALCL documented worldwide.  The American Society of Plastic Surgeons (ASPS) / FDA registry reports a total of 282 unique cases here in the US. The fill material of the implant does not play a role in the development of BIA–ALCL, and these have occurred in patients with both saline-filled and silicone gel-filled breast implants. And these cases are almost equally split between cosmetic breast augmentations and breast reconstructions for cancer.
However, the development of BIA–ALCL appears to be related to the surface characteristics of the breast implant; documented cases have occurred in patients who have (or had) a textured surface breast implant;

Risk stratification based upon implant surface texture

Based upon the known cases, the variable risk of developing BIA–ALCL can be further described based upon the degree of texturing of the implant surface. To better communicate these differences the implant surface texture has been divided into four grades.

Grade 1 : (smooth surface) –  There has not yet been a confirmed case of BIA–ALCL in a person with a history of only smooth implants.  It is important to note that one case of “smooth-only” BIA–ALCL was reported to the FDA; however, after further investigation it was later determined that the patient in this case previously had a textured breast implant.  The FDA has since amended its report.

It bears emphasizing for clarification that there have been numerous cases in which patients have had smooth surfaced implants at the time they were diagnosed with BIA–ALCL.  Thorough evaluation of their surgical history has shown that all of these patients had a textured breast implant in the past (prior to their smooth implant).

Grade 2 : (Micro-texture; Mentor Siltex and similar devices) –  1:82,000

Grade 3 : (Macro-textured; Natrelle/Inamed/McGhan Biocell and similar devices) –  1:3,200

Grade 4 : (Polyurethane foam; Silimed polyurethane) –  1:2800 ; this incidence is based upon data from an Australian study that encompasses only Silimed polyurethane implants, that had a manufacturing defect and have since been removed from their marketplace. Polyurethane foam-covered implants have not been available  in the US since the early 1990s.

What are the symptoms of BIA–ALCL?

The initial symptom of BIA–ALCL is usually a painless swelling of the affected breast due to a (seroma) fluid collection that occurs around the breast implant.  This may occur between 2 and 28 years after implant insertion, but the average time frame appears to be about 8 years following surgery.

In addition to a seroma, BIA–ALCL can also present as firmness in the breast, a lump in the breast or armpit, or a painful breast.  When diagnosed early BIA–ALCL can be effectively treated.  It is therefore important for patients to seek routine annual follow-up with their physician, and to notify their physician if they have any concerns or symptoms.

How is BIA-ALCL diagnosed?

A blood test is not currently available to screen for BIA–ALCL.  Women who do not have any symptoms should receive their routine annual mammograms and breast examinations. 

Any woman who experiences swelling in her breast, or a lump in her breast, should undergo evaluation by a plastic surgeon. This may include diagnostic imaging studies such as a mammogram and/or ultrasound.  If there is fluid around the breast implant, this fluid should be aspirated under ultrasound guidance and then sent to the laboratory for specific analysis.  The tests should include cytology, an assessment of the type of cells within the fluid, and CD30 immunohistochemistry. CD30 is a cell surface protein expressed by activated white blood cells (T cells and B cells). The CD30 protein is expressed on many types of lymphomas, including ALCL. Although the presence of CD 30 cells is not diagnostic for BIA–ALCL, it is a marker for activated T cells.  If the seroma is CD30 positive and the cytology is negative this may represent a precursor to BIA–ALCL, and should be treated with a total capsulectomy and implant removal.  If the seroma fluid is CD30 negative, and the cytology is also negative, then this should be considered a benign seroma.  The majority of seromas are benign and not indicative of BIA–ALCL.

If BIA–ALCL is confirmed, a PET scan or MRI and CT scans are utilized to help stage the disease. 

How is BIA-ALCL treated and what is the prognosis?

The current recommendations for the treatment of BIA–ALCL are bilateral en bloc capsulectomies – removal of the entire breast capsule along with the textured breast implant.  Capsulectomy is a common procedure performed by plastic surgeons during breast revision surgeries,  to treat capsular contracture, and to treat a failed silicone gel implant.  Patient’s then have the option of utilizing a smooth implants if they desire. 

The prognosis for BIA–ALCL is excellent if diagnosed early. Total capsulectomy has successfully treated all early-stage cases of BIA–ALCL, and the majority of patients require no additional treatment.  However, if the disease is allowed to spread to the lymph nodes, invade into adjacent tissues, or metastasize,  chemotherapy and radiation therapy will be necessary.

Have there been any deaths associated with BIA-ALCL?

There have been 21 confirmed deaths worldwide that are attributed to BIA–ALCL since the disease was first reported nearly 20 years ago; five deaths have been reported n the US. BIA-ALCL-related deaths have been reported in Argentina, Australia, Brasil, Canada, France, Netherlands, New Zealand, Sweden, United Kingdom, and the United States. All patients who died had either incomplete capsule removal, or the disease had spread prior to surgical treatment.

Do I need to have my textured breast implants removed?

In regards to textured breast implants, it is worth emphasizing that neither the FDA nor any plastic surgery society currently recommends the removal of textured breast implants to prevent BIA–ALCL in the absence of symptoms.  If you have concerns about your breast implants, you should speak with your surgeon to discuss the risks and benefits of implant removal.  

In the event that a decision is made to remove a textured breast implant in an attempt to prevent BIA–ALCL, simply exchanging the textured implant to a smooth implant does not completely reduce the risk for BIA–ALCL. To reduce the risk of BIA–ALCL a total capsulectomy along with an exchange to a smooth implant is required.

Any woman who does not know what type of breast implant she has should speak with the surgeon who performed her surgery.

Do the plastic surgery societies recommend against the use of textured implants?

There is no ban on the use of textured breast implants. The available data does not support discontinuing the use of textured breast implants, and neither the American Society of Plastic Surgeons nor The American Society for Aesthetic Plastic Surgery recommends against the use of textured breast implants. Many options are available to patient’s in terms of implant size, implant shape, and implant surface texture. in some situations, a textured implant may be a preferred option. As is always the case, it is important for each patient and her surgeon to thoroughly discuss all options available, as well as the risks and benefits involved. It is imperative that each patient make an informed decision based upon an individual assessment of her needs and the risks involved.

(Stay tuned for more info shortly)

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