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).

Sezary syndrome is a distinctive erythrodermic CTCL with a leukemic involvement of malignant T cells clonally matching that in the skin

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]

• 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:

The staging of sezazry syndrome is based on the TNMB:

Staging for mycosis fungoides and Sezary syndrome
Skin(T)
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(N)
N0	No clinically abnormal peripheral lymph nodes; biopsy not required
N1	Clinically abnormal lymph nodes; histopathology Dutch grade 1 or NCI LN0-2
N1a	Clone negative
N1b	Clone posetive
N2	Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3
N2a	Clone negatove
N2b	Clone posetive
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(M)
M0	No visceral organ involvement
M1	Visceral involvement (must have pathology confirmation and organ involved should be specified)
Blood
B0	0 Absence of significant blood involvement: 5% of peripheral blood lymphocytes are atypical (Sezary) cells B0a Clone negative B0b Clone positive
B1	Low blood tumour burden: >5% of peripheral blood lymphocytes are atypical (Sezary) 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

The staging of Sezary syndrome is based on the clinical stages:

clinical stages
Stage	T	N	M	B	DDS
IA	1	0	0	0/1	98
IB	2	0	0	0/1	89
IIA	1.2	1.2	0	0/1	89
IIB	3	0-2	0	0/1	56
IIIA	4	0-2	0	0	54
IIIB	4	0-2	0	1	48
IVA1	1-4	0-2	0	2	41
IVA2	1-4	3	0	0-2	23
IVB	1-4	0-3	1	0-2	18

• [5-year disease free survival (DSS)]

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.

Microscopic pathology

On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

causes

The cause of Sezary syndrome has not been identified.

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.

Cutaneous T cell lymphoma is usually initially seen by dermatologists with patients presenting with skin lesions such as erythematous patches or plaque.

Patients often have a history of several years of eczematous or dermatitic skin lesions before the diagnosis is finally established

The skin lesions then progress from the patch stage to the plaque stage to cutaneous tumors

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 Sezary syndrome from other Diseases

• Sezary syndrome must be differentiated from other diseases that cause:
  • Mycosis fangoides(Sezaruy syndrome vis more symptoIn contrast to patch/plaque MF, SS is much more symptomatic)
  • Eczema
  • Psoriasis

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

• The median age at diagnosis of Sézary syndrome is 60 years of age(SS).
• Sezary syndrome is more commonly observed among elderly patients.

Gender

• Males are more commonly affected with Sezary syndrome than females(2:1).

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].

• In sezary syndrome, B0, sezzary cells are defined less than 5.
• A majority of number atypical mononuclear cells with moderately to highly groove nuclei, termed Sézary cellsconcentration of peripheral blood of Sezary syndrome patients .
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Laboratory tests for cutaneous T cell lymphoma include:

• Complete blood count (CBC)
• Peripheral blood smear

• Atypical T-cells (Sezary cells)

• Blood chemistry studies
• Flow cytometry
• Immunohistochemistry
• Immunophenotyping: Beta F1+, CD2-/+, CD3+, CD3- (CD4-positive variant), CD4+ (CD4-positive variant), CD4-, CD5-, CD7+/-, CD8+, CD8- (CD4-positive variant), Granzyme B+, and Perforin+

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].

physical examination

Patients with Sezary syndrome in total body skin examination seen patch, plaque, tomur

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].