A Rare Case of Sezary Syndrome Cutaneous T-Cell Lymphoma with Complete Response_ Crimson Publishers

A Rare Case of Sezary Syndrome Cutaneous T-Cell Lymphoma with Complete Response by Hojouj Mohammad IM in Journal of Biotechnology & Bioresearch_ Journal of Biotechnology

Abstract

Cutaneous T cell lymphoma is one of non-Hodgkin’s lymphoma. Several types of cutaneous T-cell lymphoma exist. The most common type is mycosis fungoides. Sezary syndrome is a less common type that causes skin redness over the entire body. The common symptoms of Sezary syndrome are erythroderma, leukemia with circulating mononuclear cells having convoluted nuclei and lymph node enlargement due to infiltration by similar cells.

Lymphoma starts in the lymphocytes, or the cells in the immune system that are normally found in the lymphatic system. If lymphocytes start growing out of control, they can build up and form a cancerous collection of cells in the skin that can lead to lymphoma. Patients with skin lymphoma might experience a variety of vague or unusual symptoms, including:

1.                  Peeled skin

2.                  Rash, which sometimes can itch

3.                  Mushroom-like skin lesions

4.                  Swollen lymph nodes, or painless lumps, in the skin

5.                  Unexplained fevers and weight loss

Skin lymphoma usually is well behaved and doesn’t spread throughout the body. However, it has the potential to do so in approximately 15 to 20 percent of cases. At the specialized skin lymphoma clinic at UT Southwestern, we offer a range of treatment options to effectively beat the two specific types of skin lymphoma and their various subtypes.

Sezary syndrome is an aggressive form of cutaneous T-cell lymphoma which is a group of disorders that occur when T-cells (a type of white blood cell) become cancerous and affect the skin. It is characterized by a widespread red rash that may cover most of the body, the presence of cancerous T cells (called Sezary cells) in the blood, and abnormally enlarged lymph nodes. Other signs and symptoms may include intense itchiness, scaling and peeling of the skin; fever; weight loss; hair loss; outward turning of the eyelids (ectropion); palmoplantar keratoderma; malformation of the nails; and hepatosplenomegaly. The exact cause of Sezarysyndrome is currently unknown. Treatment varies based on the signs and symptoms present in each person and the severity of the condition.

Prognosis: 

The long-term outlook (prognosis) for people with Sezary syndrome is generally poor. Sezary syndrome is difficult to cure. Treatment is usually palliative, with the intention of relief of symptoms and improvement in the quality of life. Median survival for patients with Sezary syndrome has been reported to be 2 to 4 years after development of the condition, although survival has improved with newer treatments. The disease-specific 5-year survival rate has been reported to be 24%. We present 1 case of Sezary syndrome which happened in our hospital with complete response.

Keywords: Cutaneous T-cell lymphoma; Rare cancers; Pruritus; Sezary syndrome; Chemotherapy

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