Sezary syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

Sezary syndrome is one of the most common subtypes of cutaneous T cell lymphoma (CTCL).

Historical Perspective

• Sezary syndrome (SS) was first described by Albert Sézary.

Classification

• [Disease name] may be classified according to [classification method] into [number] subtypes/groups:

• [group1]
• [group2]
• [group3]

• Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

• Cutaneous T cell lymphoma arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response.

• Sezary syndrome and mycosis fungoides are T-cell lymphomas that primary manifest as multiple cutaneous lesions.
• Sezary syndrome cells are T-cells that have pathological quantities of mucopolysaccharides.
• Sezary's syndrome is sometimes considered a late stage of mycosis fungoides.

Clinical Features

History

Patients with sezary syndrome have a positive history of oruritive, infectiopn, second malignancy such as hodgkin lymphoma, non-Hodgkin lymphoma, melanoma,Urinary cancer.

• [History finding 1]
• [History finding 2]
• [History finding 3]

Common Signs

Common symptoms of sezary syndrome include:

• Widespread erythema
• Indurated
• Resemble livido reticularis
• Erythema
• Body surface area (BSA) involved may wax and wan

Less Common Signs

Less common symptoms of [disease name] include

• Patches and plaques to erythroderma
• Keratosis pilaris
• Alopecia
• Ectropion
• Keratoderma
• Hypertrophic nails
• Erosions
• Lichenification

Differentiating [disease name] from other Diseases

• [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of sezary syndrom is exact unknown.
• In 2005 and 2009 the incidence of sezary syndrome was estimated to be 0/08 and 0/09 cases per 100,000 individuals in the United States.

Age

• [Disease name] is more commonly observed among patients aged [age range] years old.
• Sezary syndrome is more commonly observed among elderly patients.

Gender

• [Disease name] affects men and women equally.

• [Gender 1] are more commonly affected with [disease name] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• Sezary syndrome usually affects individuals of the whites race.
• [Race 2] individuals are less likely to develop [disease name]
• Sezary syndrome is rare disease that tends to affect Whites but in this study African american has more percentage

Region

• The majority of [disease name] cases are reported in [geographical region].

• .

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• [Disease name] is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Patients with [disease name] usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

• There are no [imaging study] findings associated with [disease name].

• [Imaging study 1] is the imaging modality of choice for [disease name].
• On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Staging

• [Disease name] may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• The mainstay of therapy for sezarey syndrome (SS) is similar to treatment for mycosis fungoides (MF).
• The mainstay of therapy for sezary syndrome is of extracorporeal photopheresis (ECP) and low dose alemtuzuma[medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action 1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].