![]() You can also see on the slide that those smaller pieces can sort of move off on their own, and we call those free light chains. These immunoglobulins can be intact so that they're kind of made of four pieces: two smaller pieces, which are called light chains and two heavier pieces, which are called heavy chains. And again, in health, these are really important, and they protect us. Those are called antibodies, or immunoglobulins. And the job of these plasma cells is they make these proteins that are represented by kind of those Y's that you can see on the slides. They help us fight off infection, and that's what happens in health. In the bone marrow, there are a number of different types of cells. And so AL is actually a bone marrow disease. So that's a shot of part of the pelvis, where a lot of bone marrow is. And so here's a cartoon of a person, and you can see a blow-up that there's bone marrow. So we're going to move on now to what this is. It's something that's acquired, and if a person has it, it's not something that they should worry about that their family members are going to be affected. The good news is that AL is not a hereditary form of amyloid. The bulk of the diseases that I have listed there are actually hereditary forms. You can see that there are a number of other types of amyloids, which are very different diseases, but they share in common that they have these fibrils, which under the microscope are perceived as what we call amyloid. And this is a disease that can affect the heart, the kidney, the liver, the nerves, the skin, soft tissues. ![]() AL, the building block protein, or precursor protein, is made of immunoglobulin, most commonly immunoglobulin light chain. Amyloid is a disease where different proteins, for whatever reason, and we don't always understand it very well, decide to basically group together in what we call amyloid fibrils. It's one of many different types of amyloidosis, and here you can see a list of a number of different types of amyloidoses. So just a very brief introduction about what AL amyloidosis is. I'm a hematologist, a professor of medicine and laboratory medicine, and today I'd like to talk to you about AL amyloidosis treatment, including high-dose chemotherapy with stem cell transplantation. ANGELA DISPENZIERI: Hello, my name is Angela Dispenzieri, and I am a doctor at the Mayo Clinic. What to do if diagnosed with amyloidosis (17:04).Learn more about amyloidosis or schedule an appointment. Proper treatment can improve quality of life and longevity for patients with AL amyloidosis. It's important to work with a specialist to establish the specific type of amyloidosis, so that treatment can be designed to fit each patient's needs.ĪL amyloidosis is treated through a combination of chemotherapy drugs or by means of stem cell transplant. There are many different types of amyloid medications. AL amyloidosis is unlike other forms of amyloidosis in that it is not hereditary. The fibrils are then deposited into various tissues, including the heart, kidneys, liver and nerves. Mayo Clinic hematologist Angela Dispenzieri, M.D., discusses immunoglobulin light chain amyloidosis - also called AL amyloidosis - treatment, including high-dose chemotherapy with stem cell transplantation.Īmyloidosis is a rare disease that occurs when proteins in the body group together to form amyloid fibrils.
0 Comments
Leave a Reply. |