Sézary Syndrome

views updated

Sézary syndrome

Definition

Sézary syndrome is a type of cutaneous T-cell lymphoma , characterized by skin abnormalities, extreme itching , enlarged lymph glands, and abnormal blood cells.

Description

Sézary syndrome is a type of lymphoma, which is a disease where lymphocytes (a type of white blood cell) increase to very large numbers in a person's blood. Sézary syndrome is a type of lymphoma known as a cutaneous T-cell lymphoma, meaning that it is a disease where the white blood cells known as T-lymphocytes increase to large numbers.

Sézary syndrome can affect many organs. In early stage disease, the skin is the only organ affected; however, later stage disease can affect other organ systems.

Demographics

Sézary syndrome is relatively rare, affecting about one in one million people. The incidence of the syndrome increases with age, with most cases appearing in people in their 50s or 60s. Men appear to be affected more often than women, and black males appear to be at higher risk of developing the syndrome than white males.

Causes and symptoms

There are no known causes of Sézary syndrome. Early in the course of study of the syndrome, it was thought that exposure to certain chemicals could trigger the disease. However, later studies have not shown any relation between industrial chemical exposure and Sézary syndrome

The symptoms of Sézary syndrome can be very subtle; because of this, it is often not diagnosed for many years. Early symptoms include skin lesions that can look like eczema and psoriasis. Later symptoms can include skin tumors, especially in body folds. Enlarged lymph glands in the neck, armpits, and groin can accompany the skin tumors. Later in the course of Sézary syndrome symptoms may relate to other areas of disease involvement.

Diagnosis

The diagnosis of Sézary syndrome is made by careful clinical evaluation. Generally, a patient with Sézary syndrome seeks treatment for skin lesions that are not responsive to ordinary medications. If the doctor suspects a cutaneous T-cell lymphoma, a blood test is ordered to see if there are any abnormalities, such as an increase or decrease in lymphocytes and the presence or absence of Sézary cells, which are certain white blood cells with a distinctive shape when viewed under a microscope. Finally, a sample (biopsy ) of one the skin lesions is done to see if the lesion is part of Sézary syndrome or caused by some other disease.

Clinical staging, treatment, prognosis

Staging for cutaneous T-cell lymphoma, including Sézary syndrome, is based on the extent of skin involvement and the presence or absence of other manifestations of the syndrome. Stage I is characterized by mild skin involvement. In stage II there is extensive skin involvement, including skin tumors. Patients in stage III and IV have extensive skin involvement, blood abnormalities including Sézary cells, and swollen lymph nodes.

There are multiple therapies for Sézary syndrome. However, unless the disease is in an early stage, the chances for a complete cure are small. Nonspecific treatment includes skin lubricants and moisturizers to help treat the skin irritation and dryness that is common with the syndrome. Low potency steroid creams or ointments may be used to help treat itching and skin inflammation.

The first therapy used with some success against Sézary syndrome is mechlorethamine or nitrogen mustard. It is applied daily to the entire skin surface (except for sensitive areas such as eyelids and genitalia) for six to twelve months, then three times a week for one to two years more. Several studies have investiagted the effectiveness of nitrogen mustard therapy, and have found that in stage I or II disease, the therapy causes complete remission in 60-80% of patients. Side effects are minimal, but dry skin, irritation, and change in skin pigmentation can occur.

Another treatment that has been used for many years, especially for stage II and III disease, is electron beam radiation therapy . Treatment with electron beam radiation therapy has been used since 1953, with good response rates seen in 50-70% of patients. Side effects can include excessive skin dryness, skin blistering, loss of hair on treated areas, and increased risk of skin cancer.

ECP, or photophoresis, has been approved by the FDA as a treatment for Sézary syndrome. In this mode of treatment, phototherapy with ultraviolet light is combined with leukapheresis. In leukapheresis, a person's blood is taken out and passed through special filters that remove circulating Sézary cells; the cells are treated with ultraviolet radiation, then reinfused into the patient. Response rates range from 55% to 75%, with some reports showing a 15-25% cure rate. Side effects can include nausea and fever .

Systemic chemotherapy is often used in patients who are in later stages of the disease. Using standard cancer chemotherapeutic agents such as cyclophosphamide , vincristine , and doxorubicin , response rates up to 19 months have been seen. No studies have shown an increased survival rate in patients getting aggressive, high-dose chemotherapy versus those getting more standard doses.

The prognosis for patients with Sézary syndrome is based on placing the patient in one of three categories: good, intermediate, or poor. Patients with good prognosis have the condition limited to their skin. Their general survival time is more than 10 years. Patients in the intermediate category have skin lesions including tumors and plaques, but no blood involvement. Their survival time is five years. Patients in the poor risk category have extensive skin lesions along with blood abnormalities, including high levels of Sézary cells. Patients in this category, even with extensive treatment, generally have survival rates of only one year or less.

Coping with cancer treatment

There are multiple ways to help patients cope with side effects brought about by the treatment of Sézary syndrome. Lubricants can be used to help dryness, scaling, and itching of the skin caused by the use of topical treatments such as nitrogen mustard and electron beam therapy. Symptoms such as nausea and vomiting , caused by ECP and systemic chemotherapy, can be treated with standard anti-nausea and vomiting medication.

Clinical trials

In 2001, clinical trials are underway to investigate several forms of innovative treatment for cutaneous T-cell lymphoma and Sézary syndrome. Interferon has been used with some success in both early and late stage disease. Common side effects include a decrease in white blood cells and chronic fatigue . The use of monoclonal antibodies in treating late stage disease (III and IV) has been recently studied. Early studies have shown response rates of around 30%. Side effects include allergies to the monoclonal antibodies, fever, and fatigue.