Transcription
Welcome to my channel. Médicos is perfection, Alice. Now let's talk about mantle cell lymphoma. If you don't know where the mantle is, please watch my video on the anatomy of the lymph node. Let's get started. [Music]
As you know, hematological malignancies are leukemias, lymphoma, and myeloma. And here are the books that I use. If you are a medical student, I strongly recommend this book and this one. See the link in the description.
Lymphoma is a solid tumor of the immune system. Could be in the lymph node, spleen. May cause us to see the lymphatic tissue. When it goes to the bone marrow, we call it a leukemia, all right, or a leukemic phase of a lymphoma. Lymphomas are Hodgkin's or non-Hodgkin's. Non-Hodgkin's are more common than Hodgkin's. And Hodgkin's could be aggressive or indolent, B or they could be T-cell. Non-Hodgkin's B-cell aggressive include mantle cell lymphoma, which is today's topic. So mantle cell lymphoma is an aggressive B-cell non-Hodgkin's lymphoma. Cool.
Lymphomas that grow in the mediastinum can lead to airway obstruction and superior vena cava syndrome by compression. Some lymphomas produce IgM. Some lymphomas produce less immunoglobulin. Non-Hodgkin's lymphoma could be aggressive or indolent. The most common aggressive is diffuse large B-cell lymphoma. The most common indolent is follicular lymphoma. Which one of these two is the most common? The answer is diffuse large B-cell. Mantle cell lymphoma is an aggressive non-Hodgkin's, and it constitutes around 6% of non-Hodgkin's lymphomas. Here are the aggressive subtypes. Here are the indolent subtypes. Aggressive subtypes such as mantle cell lymphoma. Patients are usually younger, more B symptoms, sensitive to chemo, treat with intention to cure. Median survival rate, unfortunately, is short. But first, when we say mantle cell lymphoma, where the flip is the mantle? So let's zoom in to this lymph node. Let's take a piece of this and let's zoom in. When we zoom in, we see the follicle. The follicle that has a germinal center is called the secondary follicle. Secondary follicle has the germinal center. It marginalizes some cells because this germinal center is continuing to grow, so it will marginalize some cells, pushing them towards the margin, and it will create an area in between the germinal center and the margin. We call this the mantle. The mantle is in the cortex. After the cortex, we have para-cortex, then medulla, then middle area sinuses. This will open into the hilum of the lymph node. The hilum is the exit. Cortex has B cells. Para-cortex, T cells. How about medulla? They have the plasma cells. Middle area sinuses, macrophages, as we have discussed before.
Let's use the Aristotelian method of deductive reasoning in philosophy or in logic. We start with premises, then we reach a conclusion. Since the mantle is in the cortex of the lymph node, and since the cortex has B lymphocytes, conclusion: therefore, mantle cell lymphoma is a B-cell lymphoma. Voila! C medicine makes sense. It makes logical perfect sense only with Médicos.
Mantle cell lymphoma: rare, aggressive B-cell non-Hodgkin's lymphoma. Median age is 63. Males more common than females, as the case with most lymphomas. There is a translocation between chromosomes 11 and 14, between the immunoglobulin heavy chain on chromosome 14 and BCL-1 gene on chromosome 11. The translocation will lead to overexpression of the BCL-1, not gene but protein. BCL-1 protein is also known as cyclin D1, same thing. Overexpression of this protein: proliferation, proliferation, proliferation, cancer. Mantle cell lymphoma is CD5 positive but CD23 negative.
Clinically speaking, signs and symptoms: palpable painless lymphadenopathy plus systemic symptoms, because it's a non-Hodgkin's lymphoma. Systemic symptoms or B-cell symptoms such as fever, weight loss, night sweats. Maybe we have bone marrow involvement and peripheral blood involvement. We call this a leukemic phase. GI tract involvement is common in mantle cell lymphoma.
How to diagnose any lymphoma? Biopsy. Should we do fine needle aspiration biopsy? Never. Use an excisional biopsy, or you can also use core needle biopsy to detect the architecture of the node, which in this case will have malignant cells in the mantle of the follicle. Immunohistochemistry: you will find CD5 positive. Staging by physical exam and CT scan, PET scan as usual. There is site opinion. Do a bone marrow biopsy to know the cause.
Treatment: if mantle cell lymphoma is localized, use combination chemotherapy plus radiation. Disseminated? Never use radiation. We use aggressive combination chemo plus autologous hematopoietic stem cell transplant if the patient is young. Elderly and asymptomatic? Observation and chemo, use a single agent, since the patient is very old and asymptomatic.
How to treat mantle cell lymphoma using chemo? We prefer these two together: Hyper-CVAD or CVAD plus rituximab. So, si-CVAD is cyclophosphamide, vincristine, adriamycin, and dexamethasone. We can use other drugs such as bortezomib, which is a protease inhibitor. Also a Bruton's tyrosine kinase inhibitor. I've told you about Bruton's agammaglobulinemia before, but let's refresh. Normally, we have the precursor cells, pro-B cells, pre-B cells, then immature B cells, then mature B cells, then plasma cells, then they will secrete antibodies, etc. There is an enzyme here called the Bruton's tyrosine kinase. So this drug called a Bruton's inhibitor will inhibit this enzyme. The cancer cells cannot grow, therefore less cancer. Wonderful. A Bruton's inhibitor. Anything that ends in "-nib" is a tyrosine kinase inhibitor. And Bruton's… 99% of doctors do not know this fact, but now because you watch Médicos, you are in the top 1%. Very well done.
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