Transcription
Mycosis fungoides: the rash that could be cancer, and the lymphoma that is first diagnosed not by an internist, but by a dermatologist. This is Médicos is Perfection Ellis, and let's get started.
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If you have watched my previous video (and of course you should), on adult T-cell leukemia lymphoma, we have learned that T-cell malignancies will have a skin infiltration or a rash. Just a quick reminder: if you can help me reach 25,000 subscribers on YouTube, I'll start a new series on one of these systems, and we will vote on it. I'll let you choose, and you can choose more than one, and we can do them together. Thank you so much in advance. Please consider sharing this video if you like them.
Now, mycosis fungoides is a wrong name, why? Because it means fungus, and this is cancer; this is not fungus, although I wish it were, because it would have been easier to cure and treat. Mycosis fungoides is by far the most common cutaneous T-cell lymphoma; neoplastic proliferation of the CD4 helper T-cells. Again, when there is a T-cell malignancy, there will be skin invasion first; they come in patches, then plaques, then generalized erythroderma. Sometimes they form a micro abscess. The lightly lymphoma will go to the lymph node and to the visceral organs, plus you'll find some circulating tumor cells in the blood. And now we call this Sézary syndrome. The guy was French, so we should say it's Sézary syndrome; French is always cool. So if you remember my analogy to the lemon and the lemonade, same stuff here, but instead of the lemon being the lymphoma, now it's a skin invasion or infiltration. So first we have a skin problem, such as a rash, and we call this mycosis fungoides. Later on in the disease, these cells will go into the blood (leukemia) and visceral involvement, and we call that Sézary syndrome. From the lemon to the lemonade; from the localized to the generalized; from the mycosis to the Sézary.
Usually, mycosis fungoides affects men in their mid-50s, most common in African-Americans, and nobody knows why. Diagnosed by dermatologists, usual because it's a rash first; differential diagnosis: eczema or psoriasis; that's why it's difficult to diagnose. Characteristic finding: this cell called Sézary cell, thanks to the scientist who first discovered it.
So let's do it again: first we have mycosis fungoides, skin involvement by what? By the neoplastic CD4 helper T-cells. This skin involvement is called cutaneous T-cell lymphoma, and it's very itchy. Start with patches, then plaques, generalized erythroderm; from the localized to the generalized. We do not have lymph node involvement yet; it will come later. They preferably, or they come first in the buttocks; okay, that's why these cells sometimes are called buttock cells; what a weird name. Then we go from the single to the multiple to the nodules, then complete deformation. Then after this, we go to the systemic Sézary syndrome: lymphadenopathy; now we have lymph node involvement, usually of course generalized, mononucleosis, viscera lymphomas, and leukemias (LLs) in the blood. Note: same cells that were found in the skin are the exact same cells that are now found in the blood. These are called the Sézary cells; they are atypical CD4 positive T lymphocytes. To diagnose it, you will need an experienced dermatologist to do a skin biopsy and a good pathologist to report these Sézary cells, also known as buttock cells. They have this nice cerebriform nucleus, which looks like a brain. I don't know, like the souls I enjoy with the brain, I don't know. Then we have, later when it's Sézary syndrome, the peripheral smear, because it's now in the blood; we will find the same exact cells; we call them Sézary cells; they are atypical lymphocytes.
Treatment: early, which means mycosis fungoides: radiation, boom! It's localized; give it a beam of radiation. We call this total skin electron beam radiation. Later, Sézary syndrome: unfortunately, there is no cure; there is only palliative therapy, which means we try to help the patient symptomatically, but there is no cure. Topical steroids, phototherapy, retinoid, cytotoxic therapy. I hope in the future we can find a cure. Don't forget retinoids are the same stuff that treat acne and the same stuff that treat acute promyelocytic leukemia, which is AML subtype M3.
So let's wrap it up: early CD4 T-cells in the skin only, in the skin; this is mycosis fungoides lymphoma. In the lymph nodes and visceral organs, plus circulating tumor cells called Sézary cells or buttock cells; now we have Sézary syndrome. In the upcoming videos, we will talk about lymphomas as well as myeloproliferative disorders, such as multiple myeloma, essential thrombocytosis, primary myelofibrosis, etc. And don't forget to subscribe to my YouTube channel, hit the bell. I would like to see you on Facebook or Twitter, where you can ask me personal, private questions, and I answer almost all of them. Thank you so much for watching. It's Médicos is Perfection Ellis, and as always, be safe, stay happy, and study hard.