Forum - Issue 2 - 2021

Cutaneous Lymphoma Foundation FORUM 2021 Issue 2 SÉZARY SYNDROME PART ONE: OVERVIEW & DIAGNOSIS Sézary syndrome is the leukemia version of cutaneous T-cell lymphoma. The main difference between Sézary syndrome and mycosis fungoides are the cells of origin. In patients with Sézary syndrome, the cells of origin are called central memory T-cells, which means that they circulate centrally or within your bloodstream. In mycosis fungoides, the cells of origin are called skin resident effector memory T-cells, which means that they’re largely locked in the skin. Though the cause of Sézary syndrome is still unknown, there have been some associa- tions that have been observed with a small list of medications. These are associations or clusterings, they are not proof of causation. Sézary syndrome does not appear to have a genetic link and is not linked to any unique mutation. It is thought to be an epigenetic phenomenon which means that a complex combi- nation of genetic makeup and lifetime environ- mental exposures result in disease. With Sézary syndrome, most of the malignant cells are in the bloodstream; as such, erythro- derma is a known hallmark of the disease, meaning diffuse redness with some scaling or peeling of the skin over most of the body. Many patients will also experience hair loss affect - ing the hair on the scalp, face, or body. Another thing that we see in Sézary syndrome is some- thing called hyperkeratosis, which means that the hands and feet on the palms and soles become very thick and brittle and patients may develop cracks or fissures. These skin changes are unfortunately associated with intense itching or stinging which is severe for many patients and creates the largest challenge in symptommanage- ment in Sézary syndrome. Swollen lymph nodes may also occur and can most commonly be felt in the neck, underarms, and groin. The diffuse and intense skin inflammation pulls blood to the skin and robs the body of heat resulting in patients feel - ing cold or experiencing shivering or chills. When diagnosing Sézary syndrome, skin biopsy is not as useful as it is with cutaneous T-cell lymphoma mycosis fungoides, because again, the majority of the malignant cells are in the blood rather than the skin. While a skin biopsy is traditionally done and it can give us some useful information, the bulk of our diagnostic information is going to come from an assessment of the blood. This is performed using a flow cytometry machine which From the President and the Chief Executive Officer. ............................................................. 3 Catalyst Research Grant Awardees................... 4 CRISPR Therapeutics Research............................ 5 My Cutaneous Lymphoma Experience........... 6 Frequently Asked Questions.......................... 8 Many Ways to Support .................................... 9 ISCL Scientific Meeting Highlights................. 10 Get Your Move On 2021....................Backpage Sézary Syndrome...continued on page 7 Cutaneous Lymphoma Foundation

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