What does Sézary syndrome look like?

Most patients with Sézary syndrome have extensive red, itchy rash covering at least 80 percent of the body. In some cases, thicker, red patches (or plaques) and tumors may also appear. In addition, these symptoms may be accompanied by changes in the nails, hair, eyelids, and the presence of enlarged lymph nodes.

What does Sézary syndrome feel like?

People with Sézary syndrome develop a red, severely itchy rash (erythroderma) that covers large portions of their body. Sézary cells are found in the rash. However, the skin cells themselves are not cancerous; the skin problems result when Sézary cells move from the blood into the skin.

How do you diagnose Sézary syndrome?

The diagnosis is made by erythroderma involving greater than 80% BSA, clonal TCR rearrangement confirmed by PCR or Southern blot, and absolute Sezary cell count of at least 1000 cells/microL, or one of the following 2 criteria: Increased CD4+ or CD3+ with CD4/CD8 ratio of 10 or more.

What is the difference between mycosis fungoides and Sézary syndrome?

Mycosis fungoides and Sézary syndrome are types of cutaneous T-cell lymphoma. A sign of mycosis fungoides is a red rash on the skin. In Sézary syndrome, cancerous T-cells are found in the blood. Tests that examine the skin and blood are used to diagnose mycosis fungoides and Sézary syndrome.

How long can you live with Sézary syndrome?

Median survival for patients with Sézary syndrome has been reported to be 2-4 years, although the median survival was 2.9 years among patients defined by 2011 criteria for the disease. The disease-specific 5-year survival rate has been reported to be 24%.

Answers from the Experts: Sezary Syndrome and Transformed Mycosis Fungoides

What are the stages of Sézary syndrome?

When there is a high number of Sézary cells in the blood, the disease is called Sézary syndrome. Stage IV is divided into stages IVA1, IVA2, and IVB. In stage IVA1, patches, papules, plaques, or tumors may cover any amount of the skin surface, and 80% or more of the skin surface may be reddened.

Is Sézary syndrome terminal?

Sézary syndrome has features of both an aggressive and chronic lymphoma. Short of allogeneic stem cell transplantation, the disease is not felt to be curable and survival is shortened for most affected patients. However, there are multiple therapies with response rates in the 30 to 50% range.

What does mycosis fungoides look like on the skin?

In its earliest form, mycosis fungoides often looks like a red rash (or scaly patch of skin). It begins on skin that gets little sun, such as the upper thigh, buttocks, back, belly, groin, chest, or breasts.

Does mycosis fungoides show up in blood work?

A sign of mycosis fungoides is a red rash on the skin. In Sézary syndrome, cancerous T-cells are found in the blood. Tests that examine the skin and blood are used to diagnose mycosis fungoides and Sézary syndrome.

What is the life expectancy of someone with mycosis fungoides?

The overall survival and disease-specific survivals of our 525 patients with MF are shown in Figure 1. The median survival was 11.4 years, and the actuarial overall survival rates at 5, 10, and 30 years were 68%, 53%, and 17%, respectively. The median follow-up time was 5.5 years (range, 0.1-38.5 years).

What causes mycosis fungoides to flare up?

Mycosis fungoides occurs when T-cell lymphocytes become cancerous. When these cancerous lymphocytes circulate in the blood, they are called Sézary cells. Sézary syndrome occurs when you have large numbers of T-cell lymphocytes — called Sezary cells — in the blood that can go to the skin and lymph nodes.

What does a rash from lymphoma look like?

When lymphoma affects the skin, it may cause a rash that appears as one or more scaly, reddish-to-purple patches, plaques, or nodules. A lymphoma rash, such as mycosis fungoides (MF), can be easy to confuse with other skin conditions, such as psoriasis or eczema, which can cause similar symptoms.

What does lymphoma of the skin look like?

The lesions are often itchy, scaly, and red to purple. The lymphoma might show up as more than one type of lesion and on different parts of the skin (often in areas not exposed to the sun). Some skin lymphomas appear as a rash over some or most of the body (known as erythroderma).

Where do you itch with lymphoma?

Cytokines can irritate nerve endings in the skin, which can in turn cause persistent itching. Many individuals experience this itchiness in their hands, lower legs or feet, while others feel it throughout their entire body. Patients often report that the itching tends to worsen while they are lying in bed at night.

What can mycosis fungoides be mistaken for?

Mycosis fungoides is often mistaken for common skin conditions such as psoriasis, chronic dermatitis or chronic fungal infection. The experts at Stanford's Cutaneous Lymphoma Program have the experience necessary to provide an accurate diagnosis for this condition.

Does mycosis make you tired?

The majority of respondents had mycosis fungoides (89%). Respondents were bothered by skin redness (94%) and by the extent of symptoms that affected their choice of clothing (63%). For most patients, the disease had a functional impact, rendering them tired or affecting their sleep.

What does a cancerous rash look like?

As the cancer progresses, an indentation may form in the center of the lesion, where it may begin to ooze or bleed. Initially, a rash caused by basal cell carcinoma may resemble dermatitis, psoriasis or eczema. However, unlike a noncancerous skin rash, a cancerous skin rash will usually not resolve on its own.

What does cutaneous T-cell lymphoma look like?

Signs and symptoms of cutaneous T-cell lymphoma include: Round patches of skin that may be raised or scaly and might be itchy. Patches of skin that appear lighter in color than surrounding skin. Lumps that form on the skin and may break open.

Who is at risk for mycosis fungoides?

Age: Mycosis fungoides occurs most often in patients older than age 60, however can be seen at any age. Sex: Mycosis fungoides is twice as common in men as women. Race: Mycosis fungoides is somewhat more common in blacks.

What does a lesion look like?

Skin lesions are areas of skin that look different from the surrounding area. They are often bumps or patches, and many issues can cause them. The American Society for Dermatologic Surgery describe a skin lesion as an abnormal lump, bump, ulcer, sore, or colored area of the skin.

How long can a person live with T-cell lymphoma?

Among all histological type subgroups, the prognosis of NK/T-cell lymphoma was the worst with the 3-year survival rate of only 25%u the 3-year survival rate was 40% in unspecified peripheral T-cell lymphoma group,and 85% in angioimmunoblast T-cell lymphoma group.

Can Sézary syndrome be cured?

But in Sézary syndrome, cancerous lymphocytes (Sézary cells) spread from the skin to the blood. The cancer can also travel to the lymph nodes and other areas of the body. Sézary syndrome is an aggressive disease, meaning it spreads fast. Treatment can help manage the effects of the disease, but there's no cure.

How long can you live with skin lymphoma?

Patients who have stage IIB disease with cutaneous tumors have a median survival rate of 3.2 years (10-year survival rate of 42%) Patients who have stage III disease (generalized erythroderma) have a median survival rate of 4-6 years (10-year survival rate of 83%)

What are the warning signs of lymphoma?

What does the start of lymphedema look like?

Common signs and symptoms of lymphedema can include: Swelling in part of the body (such as your breast, chest, shoulder, arm, or leg) Skin feeling tight or hard, changing in texture, looking red, or feeling hot. New aching, tingling, numbness, or other discomfort in the area.