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

If the staging system involves specific and characteristic findings and features:

According to the TNMB (tumoure, node, metastasis,blood) classification , there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

The staging of sezazry syndrome is based on the TNMB:

Mycosis Fungoides Cooperative Group TNMB classification of cutaneous T cell lymphoma (CTCL)

Staging for mycosis fungoides and Sezary syndrome
Skin
T1	Limited patches, papules, and/or plaques covering <10% of the skin surface. May further stratify into T1a (patch only) versus T1b (plaque patch)
T2	Patches, papules, or plaques covering 10% of the skin surface. May further stratify into T2a (patch only) versus T2b (plaque patch).
T3	One or more tumours (1-cm diameter)
T4	Confluence of erythema covering 80% body surface area

Skin (T)
T1	Limited patches*, papules, and/or plaques¶ covering <10 percent of the skin surface; may further stratify into T1a (patch only) versus T1b (plaque ± patch)
T2	Patches, papules, or plaques covering ≥10 percent of the skin surface; may further stratify into T2a (patch only) versus T2b (plaque ± patch)
T3	One or more tumorsΔ (≥1 cm diameter)
T4	Confluence of erythema covering ≥80 percent body surface area
Node (N)
N0	No clinically abnormal lymph nodes◊; biopsy not required
N1	Clinically abnormal lymph nodes; histopathology Dutch grade 1 or NCI LN0-2
N1a	Clone negative§
N1b	Clone positive§
N2	Clinically abnormal lymph nodes; histopathology Dutch grade 2 or NCI LN3
N2a	Clone negative§
N2b	Clone positive§
N3	Clinically abnormal lymph nodes; histopathology Dutch grades 3-4 or NCI LN4; clone positive or negative
NX	Clinically abnormal lymph nodes; no histologic confirmation
Visceral (M)
M0	No visceral organ involvement
M1	Visceral involvement (must have pathology confirmation¥ and organ involved should be specified)
Blood (B)
B0	No significant blood involvement: ≤5 percent of Sézary cells. For clinical trials, B0 may also be defined as <250/microL Sézary cells; CD4+CD26- or CD4+CD7- cells or CD4+CD26- and CD4+CD7- cells <15 percent by flow cytometry.
B0a	Clone negative
B0b	Clone positive
B1	Low blood tumor burden: Does not meet the criteria of B0 or B2
B1a	Clone negative
B1b	Clone positive
B2	High blood tumor burden: Positive clone‡ plus one of the following: ≥1000/microL Sézary cells; CD4/CD8 ≥10; CD4+CD7- cells ≥40 percent; or CD4+CD26- cells ≥30 percent. For clinical trials, B2 may also be defined as >1000/microL CD4+CD26- or CD4+CD7- cells.

Skin T1 Limited patches, papules, and/or plaques covering <10% of the skin surface. May further stratify into T1a (patch only) versus T1b (plaque patch). T2 Patches, papules, or plaques covering 10% of the skin surface. May further stratify into T2a (patch only) versus T2b (plaque patch). T3 One or more tumours (1-cm diameter) T4 Confluence of erythema covering 80% body surface area Node [181,182] N0 No clinically abnormal peripheral lymph nodes; biopsy not required N1 Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0e2 N1a Clone negative N1b Clone positive N2 Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3 N2a Clone negative N2b Clone positive N3 Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3e4 or NCI LN4; clone positive or negative Nx Clinically abnormal peripheral lymph nodes; no histologic confirmation Visceral M0 No visceral organ involvement M1 Visceral involvement (must have pathology confirmation and organ involved should be specified) Blood B0 Absence of significant blood involvement: �5% of peripheral blood lymphocytes are atypical (Se´zary) cells B0a Clone negative B0b Clone positive B1 Low blood tumour burden: >5% of peripheral blood lymphocytes are atypical (Se´zary) cells but does not meet the criteria of B2 B1a Clone negative B1b Clone positive B2 High blood tumour burden: 1000/mL Se´zary cells with positive clone SS is staged as T4 N2

OR

There is no established system for the staging of [malignancy name]. If the staging system involves specific and characteristic findings and features:

According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

The staging of [malignancy name] is based on the [staging system].

OR

There is no established system for the staging of [malignancy name].

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(Not seen in some patients)
• 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].

Medical Therapy

The predominant therapy for cutaneous T cell lymphoma is PUVA. Adjunctive chemotherapy, radiotherapy, biological therapy, retinoid therapy, and photophoresis may be required.

Medical therapy for cutaneous T cell lymphoma

Stage	PUVA	Topical chemotherapy	Systemic chemotherapy	Radiotherapy	Biological therapy	Retinoid therapy	Photopheresis
Stage I	May be given By itself Or with interferon alfa	May be offered	May be offered	To 1 or 2 skin lesions (local radiation therapy) Total skin electron beam therapy (TSEB)	May be given By itself Or with topical chemotherapy	May be offered	---------
Stage II	May be given By itself Or with interferon alfa	May be offered	May be offered	To 1 or 2 skin lesions (local radiation therapy) Total skin electron beam therapy	May be given By itself Or with topical chemotherapy	May be offered	---------
Stage III	May be given By itself Or with interferon alfa Or systemic chemotherapy	May be offered	May be combined with other skin-focussed therapies	Total skin electron beam therapy As palliative therapy to reduce the size of tumours or relieve symptoms	May be given By itself Or with topical chemotherapy	May be offered	May be offered
Stage IV	May be given By itself Or with interferon alfa Or systemic chemotherapy	May be offered	May be offered	Total skin electron beam therapy (TSEB) As palliative therapy to reduce the size of tumours or relieve symptoms	May be given By itself Or with topical chemotherapy	May be offered	May be given By itself Or with total skin electron beam therapy
Recurrent cutaneous T cell lymphoma	May be offered	May be offered	May be offered	Total skin electron beam therapy Radiation therapy to bulky tumours or lymph nodes	May be offered	---------	---------

Treatment for cutaneous T cell lymphoma

Treatment	Description
Phototherapy or Ultraviolet light therapy
PUVA (psoralen and ultraviolet A light therapy)	Treatment consists of giving a drug called psoralen and then a certain amount of ultraviolet A light is used on the skin Psoralen makes the skin very sensitive to the effects of UVA light, which helps destroy the lymphoma cells Psoralen is taken as a pill, usually about 2 hours before the skin is treated with the UVA light PUVA is effective for treating thick patches and plaques PUVA treatments are given much the same as a tanning session under a sunlamp Treatments are given several times (often 3 times) a week at first When the person responds, then the number of treatments is usually decreased Treatments may need to be continued on a regular basis for several months (maintenance therapy) PUVA treatment is sometimes called photochemotherapy
Ultraviolet B (UVB) light	UVB therapy is effective in treating skin patches or thin plaques Psoralen is not used with UVB treatment Treatment with UVB phototherapy may also be given several times a week
Chemotherapy
Topical chemotherapy	Is usually used to treat limited disease or early stage cutaneous T cell lymphoma because it is a local therapy Mechlorethamine Carmustine
Systemic chemotherapy	Is used to treat cutaneous T cell lymphoma that is more advanced, that has relapsed, or that no longer seems to be responding to other treatments Most common chemotherapy pills Methotrexate Chlorambucil Etoposide Intravenous chemotherapy drugs Fludarabine Cladribine Pentostatin Pegylated liposomal doxorubicin Gemcitabine
Radiation therapy
Local external beam radiation therapy	May be used if only 1 or 2 small areas of skin are affected It may also be used to treat patches that remain after PUVA treatment
Total skin electron beam (TSEB) therapy	May be used to treat larger areas of skin Usually given only once to treat a person with cutaneous T cell lymphoma But can sometimes be repeated using reduced doses if cutaneous T cell lymphoma recurs Can cause a sunburn-like reaction and people may lose their finger nails, toe nails and hair Requires special equipment and may not be available in all treatment centres
Biological therapy
Interferon alfa	Interferon alfa is injected under the skin into the fatty tissue (subcutaneously) to help boost the immune response It may be used alone or in combination with other treatments, such as PUVA
Denileukin diftitox	Is a newer drug that is a combination of the biological therapy drug interleukin-2 and the diphtheria toxin The interleukin finds the cutaneous T cell lymphoma cells and the diphtheria toxin kills the cells
Retinoid therapy
Retinoids	Retinoids are drugs that are similar to vitamin A and interfere with cell growth Retinoids may be applied to the skin or may be taken by mouth (orally) Bexarotene is one retinoid drug that may be used Bexarotene comes in a gel form that can be put on the skin It is used for early stage cutaneous T cell lymphoma with limited skin involvement It can also be taken as a pill and is used for people with extensive skin involvement or who relapse
Photopheresis
Photopheresis	Involves running a person's blood from a vein in their arm through a machine that exposes it to ultraviolet A light Similar to PUVA treatment, psoralen is used to make the cancerous white blood cells in the blood more sensitive to the effects of UVA light The treated blood is then returned (reinfused) back into the body This treatment is used for sezary syndrome or for progressing cutaneous T cell lymphoma Often need to be repeated several times May also be called extracorporeal photochemotherapy (ECP)

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].