Sezary syndrome: Difference between revisions

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Revision as of 13:34, 13 May 2019

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

Overview

Sezary syndrome (SS) is one of the most common subtypes of cutaneous T cell lymphoma (CTCL). Sezary syndrome is an erythrodermic cutaneous T-cell lymphoma with a leukemic involvement of malignant T cells with or without lymphnod and visceral organ involvement. SS is closely related to mycosis fungoides (MF), and the two disorders are diagnosed and staged by the same criteria .Cutaneous T cell lymphoma arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response. Sezary Syndrome constitute the broad spectrum of cutaneous T cell lymphoma.

Historical Perspective

Sezary syndrome (SS) was first described by Albert Sézary in 1938.^[1]
The association between cell count of lymphocytes in the peripheral blood with grooved, lobulated (that is, “cerebriform”) nuclei and Sezary syndrome was made in the early to mid-20th century.^[2]

Classification

The staging of sezazry syndrome is based on the TNMB:^[3]
Sezary syndrome is defined by T4 erythroderma of body surface area (BSA) more than of 80 percent, Sezary cell is more than 1000 cells/microL in B2 involvement of peripheral blood staged of Sezary syndrome is based on the presence of nodal and/or visceral involvement^[4]


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 (B)
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 Sezary cells with positive clone

The staging of Sezary syndrome is based on the clinical stages:^[3]^[5]


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

The exact pathogenesis of Sezary syndrome is not fully understood.^[6]
Sezary syndrome is an erythrodermic cutaneous T-cell lymphoma with a leukemic involvement of malignant T cells.^[7]
Cutaneous T cell lymphoma arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response.^[8]
Sezary syndrome's patients have suppressed immunity, as the malignant cells produce type-2, T-cell (Th2) cytokines which suppress Th1 immunity by decreasing the production of IL-12. The role of IL-12 is to stimulate the production of interferon gamma and tumor necrosis factor-alpha (TNF-a), thus protecting against tumors.^[6]
The tumor cells originate from memory T cells or skin homing CD4+ T cells expressing cutaneous lymphocyte antigen (CLA) and chemokine receptors CCR4 and CCR7.^[9]
A few patients of Sezary syndrome are associated with human T-lymphotropic virus types 1 and 2 (HTLV1 and HTLV2) In Japan, Caribbean islands, and the Middle East.^[10]^[11]
In Sezary syndrome's patients blood flow cytometry shown measurement of the CD4+CD26- population of lymphocytes is a valuable tool for monitoring and CD26 seen in >90% of patients and CD7 loss in 50% of patients. ^[12]^[13]
In majority of Sezary syndrome's patients peripheral blood T cell seen CD3+, CD4+, CD7-, CD26-, CD8-.^[14]
In some Sezary syndrome patients CD8+ phenotype seen with retroviral infections (HTLV-1/2 or HIV).^[15]^[16]^[17]
Sezary syndrome cells are T-cells that have pathological quantities of mucopolysaccharides.
When Sezary cells move from the blood into the skin, the skin problem is seen and Sezary cells are finded in rash.
In sezary syndrome maybe one or more chromosomal abnormalities, such as the loss or gain of genetic.^[18]^[19]

61-year-old man presented in 1972 with unrelenting pruritus of six months’ duration. On the right is his peripheral blood film stained with Periodic Acid-Schiff (PAS) showing a neoplastic T cell (Sézary cell).

Microscopic pathology

Light microscopic findings : On microscopic histopathological analysis, movement of the atypical lymphocytes into the epidermis, atypical cells in intra epidermal, and infiltrate of inflammatory T cells are characteristic findings of Sezary syndrome.^[20]
Sezary cells are aypical mononuclear cells with moderately to highly grooved nuclei, termed seen in prepheral blood morphology^[21], Sezary cell is large than normal lymphocyt cell.^[22]
Clonality of the T cell receptor (TCR) gene rearrangement : Clonality of the T cell receptor (TCR) gene rearrangement by polymerase chain reaction.^[23]
High throughput TCR sequen is more sensitive technique than T cell receptor (TCR).^[24]

Causes

The cause of Sezary syndrome has not been identified.^[25]^[26]
Sezary syndrome might have one or more of the chromosomal abnormalities, such as the loss or gain of genetic.^[18]^[19]

Clinical Features

History

The majority of sezary syndrome patients present with developing lymphadenopathy and erythroderma for weeks to months.^[27]
Cutaneous T cell lymphoma is usually initially seen by dermatologists with patients presenting with skin lesions such as erythematous patches or plaque.^[28]
Sezary syndrome is known as leukemic form of cutaneous T cell lymphoma (CTCL) that associated with erythroderma.
Early clinical features of Sezary syndrome include:^[29]

In Sezary syndrome single or multiple lesions( thin erythematous plaques or flat patche) is a typucal skin involvement in the gluteal region or thighs. ^[30]
The lesions of Sezary syndrome can be pruritic or remain stable for many years, go into remission, or grow slowly^[31]
Patients with Sezary syndrome have a positive history of pruritic, infection, second malignancy such as hodgkin lymphoma, non-Hodgkin lymphoma, melanoma, urinary cancer.^[32]
Patients with Sezary syndrome often have a history of several years of eczematous or dermatitis skin lesions before the diagnosis is finally established.^[33]
The skin lesions then progress from the patch stage to the plaque stage to cutaneous tumors, skin is often pruritic and affected on quality of life of patients.^[34]

Signs

Common signs of Sezary syndrome include:^[35]

Widespread erythema
- In Sezary syndrome widespread erythema can be finely scaly, indurated, or even resemble livido reticularis
Indurated
Resemble livido reticularis
Erythema(Not seen in some patients)^[36]
The severity of erythema body surface area (BSA) involved may wax and wane(>80% of BSA)

Skin lesions

The other skin lesion symptoms of Sezary syndrome are:

Patches and plaques to erythroderma
Keratosis pilaris
Alopecia (hair loss)
Ectropion
Keratoderma
Hypertrophic nails
Erosions
Lichenification
Trouble regulating body temperature
Abnormalities of fingernails and toenails

Other Signs

Some patients with Sezary syndrome have^[37]^[38]

Differentiating Sezary syndrome from other Diseases

Sezary syndrome must be differentiated from other diseases that cause:^[39]^[40]^[41]^[42]^[43]
- Mycosis fangoides
  - Sezaruy syndrome is more symptoI contrast to patch or plaque MF, SS is much more symptomatic. Sezary syndrome patients tend to present with diffuse skin involvement,not like mycosis fungoides usually evolve through patches and plaques to erythroderma ^[44]
  - In Sezary syndrome infiltration of skin is generally much less dense than plaque in mycosis fungoides (MF)
- Eczema
- Psoriasis
- Pityriasis rubra pilaris
- dermatitis
- Hypereosinophilic syndrome
- Adult T-cell leukemia
- Atopic dermatitis
- Contact dermatitis
- Chronic actinic dermatitis
- Scabies
- Drug eruption
- Graft versus host disease

Epidemiology and Demographics

The prevalence of Sezary syndrome is exact unknown.^[45]
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.^[46]^[47]

Age

The median age at diagnosis of Sézary syndrome is 60 years of age(SS).^[48]
Sezary syndrome is more commonly observed among elderly patients.^[49]

Gender

Males are more commonly affected with Sezary syndrome than females(2:1).^[50]

Race

Sezary syndrome usually affects individuals of the whites race.^[51]
Sezary syndrome is rare disease that tends to affect Whites ^[51] but in this study African american has more percentage^[52]

Risk Factors

Common risk factors in the developme Sezary syndrome are AIDS, pneumonias , bacterial cutaneous infections were frequen.^[53]

Complications and Prognosis

Common complications of Sezary syndrome include infection, second malignancies ( Hodgkin lymphoma, Non-Hodgkin lymphoma, melanoma, urinary cancer) and especially lymphoma.^[54]^[55]^[56]
Prognosis is depended on stage of disease, elevated LDH, race, sex (male), peripheral eosinophilia and generally is poor.^[57]

Diagnosis

The diagnosis of Sezary syndrome is made through examination of total body surface area (BSA) and the following of result of blood tests, biopsy of lymph node and bone marrow should be done.
Patients are presenting with maycosis fangoides (MF) may evolve to Sezary syndrome (SS) after 6-30 monthes. ^[58]
The ISCL/EORTC recommends diagnostic criteria:^[59]

Erythroderma covering body surface area (BSA) >80%
By PCR or southern blot analysis a clonal TCR rearrangement identified in blood
Sezary cell count >1000 cells/microL or one of the following two criteria :
1. Increased CD4+ or CD3+ cells with a CD4 to CD8 ≥ 10
2. Increased CD4+ cells with an abnormal phenotype ( CD4+CD7- ≥40 % or CD4+CD26- ≥30%)

CT scan may be helpful in the diagnosis of Sezary syndrome. Findings on CT scan a node larger than 1.5 cm.^[60]

Symptoms

Symptoms of Sezary syndrome may include the following:
- Most common symtom of Sezary syndrome is pruritus.
  - There is not related between pruritis and the degree of blood involvement or the extent and depth of erythema .^[61]
  - Pruritus may exacerbate sleep dysfunction, anxiety, and depression.^[62]

Physical Examination

Patients with Sezary syndrome usually appear with skin signs and symptoms.
Physical examination may be remarkable for:

Laboratory Findings

In sezary syndrome, B0, sezary cells are defined less than 5.
A majority of number atypical mononuclear cells with moderately to highly groove nuclei, termed Sezary cells concentration of peripheral blood of Sezary syndrome patients .^[63]
Other laboratory findings consistent with the diagnosis of Sezary syndrome include PCR, southern blot, and High throughput TCR sequencing, immunophenotyping confirming T cell origin (CD3+, CD4+), lymph node biopsy, peripheral blood test (morphology, felow cytometry)

Laboratory tests for cutaneous T cell lymphoma include:^[64]

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+
Bon marrow^[65]
- In advanced disease of Sezary syndrome, bone marrow involvement can be seen.
- Considered as a leukemic phase of cutaneous T-cell lymphoma without any bone marrow compromise.^[66]
Biopsy:
- More than 4 cm of punch

Treatment

The mainstay of therapy for Sezarey syndrome (SS) is similar to treatment for mycosis fungoides (MF).^[67]^[68]^[69]

Medical Therapy

Pharmacologic medical therapy is recommende radiotherapy, biological therapy mycosis fungoides (MF) for Sezary syndrome (SS) plus photopheresis (ECP) and low dose alemtuzumab, and the need for adjuvant treatment to control pruritus.^[70]
- In patients with severe pruritus; topical and systemic anti pruritics are recommended.^[71]
Systemic treatments are generally required in the leukemic blood involvement Sezary syndrome patients.^[71]
In new diagnosed Sezary syndrome patients with slowly progressive disease munomodulatory therapies are recommended.^[71]
Pharmacologic medical therapies for Sezary syndrome include primary treatment and secodanry treatment and allogenic hematopoietic cell transplantation
- Primary: Extracorporeal photopheresis (ECP), Retinoids (bexarotene, acitretin, isotretinoin, all-trans retinoic acid), Histone deacetylase (HDAC) inhibitors (vorinostat, romidepsin), Low dose methotrexate
- Secondary: Pegylated liposomal doxorubicin,Gemcitabine, Alemtuzumab, Chlorambucil, Fludarabine, Cladribine, Pentostatin, Intermediate dose methotrexate, Pralatrexate (low dose) , Brentuximab vedotin, Pembrolizumab


Primary treatment
Agent	Combination therapy	Comment
ECP	Systemic therapy can be combined with ECP: Retinoid, low dose methotrexate, HDAC inhibitor, interferon skin directed therapy (phototherapy, topicals, TSEBT)	this therapy is only in some specialized centers.
Retinoids (bexarotene, acitretin, isotretinoin, all-trans retinoic acid)	Systemic therapy can be combined such as: ECP Interferon Skin direct therapy( PUVA, TSEBT)	Teratogenic Side effects such as: Hyperlipidemia Central hypothyroidism (bexarotene)
Interferon alpha Interferon gamma	Systemic therapy can be combined : ECP Bexarotene ECP Skin directed therapy: Topicals, PUVA	It is useful for concomitant autoimmune conditions or solid transplant patients
Methotrexate (Low dose)	Systemic therapy can be combined: ECP Interferon HDAC inhibitor Skin directed therapy	Side effects: Teratogenic Mild generalized immunosuppression liver toxcity
Histone deacetylase HDAC inhibitors (vorinostat, romidepsin)	Systemic therapy can be combined: IECP Interferon Skin directed (TSEBT) therapy	Side effect: Fatigue Nausea Diarrhea Thrombocytopenia Nonspecific electrocardiogram effects
Secondary treatment
Secondary treatment recomanded after inadequate response, refractory disease or progression despite primary treatment ( Pegylated liposomal doxorubicin, Gemcitabine, Alemtuzumab, Chlorambucil, Fludarabine, Cladribine, Pentostatin, Methotrexate(intermediate dose), Low dose pralatrexate, Brentuximab vedotin, Pembrolizumab)

For immediate control in patients with rapidly progressive disease, chemotherapeutic agents or targeted therapies are recommanded. If possible immune enhancing or preserve agent recommanded to use before chemotherapy.^[71]
The predominant therapy for cutaneous T cell lymphoma is PUVA. Adjunctive chemotherapy, radiotherapy, biological therapy, retinoid therapy, and photophoresis may be required ^[72]

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].
Pneumococcal vaccination ld be systematically recommended
Prophylaxis with co-trimoxazole and valaciclovir is when the CD4 count is < 0·2 × 109 cells L-1

References

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↑ Wilcox RA (January 2016). "Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 91 (1): 151–65. doi:10.1002/ajh.24233. PMC 4715621. PMID 26607183.
↑ ^3.0 ^3.1 Trautinger, Franz; Eder, Johanna; Assaf, Chalid; Bagot, Martine; Cozzio, Antonio; Dummer, Reinhard; Gniadecki, Robert; Klemke, Claus-Detlev; Ortiz-Romero, Pablo L.; Papadavid, Evangelia; Pimpinelli, Nicola; Quaglino, Pietro; Ranki, Annamari; Scarisbrick, Julia; Stadler, Rudolf; Väkevä, Liisa; Vermeer, Maarten H.; Whittaker, Sean; Willemze, Rein; Knobler, Robert (2017). "European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome – Update 2017". European Journal of Cancer. 77: 57–74. doi:10.1016/j.ejca.2017.02.027. ISSN 0959-8049.
↑ Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, Zackheim H, Duvic M, Estrach T, Lamberg S, Wood G, Dummer R, Ranki A, Burg G, Heald P, Pittelkow M, Bernengo MG, Sterry W, Laroche L, Trautinger F, Whittaker S (September 2007). "Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)". Blood. 110 (6): 1713–22. doi:10.1182/blood-2007-03-055749. PMID 17540844.
↑ Jawed, Sarah I.; Myskowski, Patricia L.; Horwitz, Steven; Moskowitz, Alison; Querfeld, Christiane (2014). "Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome)". Journal of the American Academy of Dermatology. 70 (2): 205.e1–205.e16. doi:10.1016/j.jaad.2013.07.049. ISSN 0190-9622.
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↑ ^18.0 ^18.1 Woollard WJ, Pullabhatla V, Lorenc A, Patel VM, Butler RM, Bayega A, Begum N, Bakr F, Dedhia K, Fisher J, Aguilar-Duran S, Flanagan C, Ghasemi AA, Hoffmann RM, Castillo-Mosquera N, Nuttall EA, Paul A, Roberts CA, Solomonidis EG, Tarrant R, Yoxall A, Beyers CZ, Ferreira S, Tosi I, Simpson MA, de Rinaldis E, Mitchell TJ, Whittaker SJ (June 2016). "Candidate driver genes involved in genome maintenance and DNA repair in Sézary syndrome". Blood. 127 (26): 3387–97. doi:10.1182/blood-2016-02-699843. PMID 27121473.
↑ ^19.0 ^19.1 Wilcox RA (January 2016). "Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 91 (1): 151–65. doi:10.1002/ajh.24233. PMC 4715621. PMID 26607183.
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↑ Wong HK, Mishra A, Hake T, Porcu P (October 2011). "Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)". Br. J. Haematol. 155 (2): 150–66. doi:10.1111/j.1365-2141.2011.08852.x. PMC 4309373. PMID 21883142.
↑ Woollard WJ, Pullabhatla V, Lorenc A, Patel VM, Butler RM, Bayega A, Begum N, Bakr F, Dedhia K, Fisher J, Aguilar-Duran S, Flanagan C, Ghasemi AA, Hoffmann RM, Castillo-Mosquera N, Nuttall EA, Paul A, Roberts CA, Solomonidis EG, Tarrant R, Yoxall A, Beyers CZ, Ferreira S, Tosi I, Simpson MA, de Rinaldis E, Mitchell TJ, Whittaker SJ (June 2016). "Candidate driver genes involved in genome maintenance and DNA repair in Sézary syndrome". Blood. 127 (26): 3387–97. doi:10.1182/blood-2016-02-699843. PMID 27121473.
↑ Wilcox RA (January 2016). "Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 91 (1): 151–65. doi:10.1002/ajh.24233. PMC 4715621. PMID 26607183.
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↑ Kim YJ, Shin HJ, Won CH, Chang SE, Lee MW, Choi JH, Lee WJ (June 2018). "The Incidence of Other Primary Cancers in Patients with Cutaneous Lymphoma". Ann Dermatol. 30 (3): 335–341. doi:10.5021/ad.2018.30.3.335. PMC 5929952. PMID 29853749.
↑ "Extracorporeal photophoresis: an evidence-based analysis". Ont Health Technol Assess Ser. 6 (6): 1–82. 2006. PMC 3379535. PMID 23074497.
↑ Demierre MF, Gan S, Jones J, Miller DR (November 2006). "Significant impact of cutaneous T-cell lymphoma on patients' quality of life: results of a 2005 National Cutaneous Lymphoma Foundation Survey". Cancer. 107 (10): 2504–11. doi:10.1002/cncr.22252. PMID 17048251.
↑ Olek-Hrab K, Silny W (March 2014). "Diagnostics in mycosis fungoides and Sezary syndrome". Rep Pract Oncol Radiother. 19 (2): 72–6. doi:10.1016/j.rpor.2013.11.001. PMC 4054990. PMID 24936324.
↑ Thompson, Agnieszka K.; Killian, Jill M.; Weaver, Amy L.; Pittelkow, Mark R.; Davis, Mark D.P. (2017). "Sézary syndrome without erythroderma: A review of 16 cases at Mayo Clinic". Journal of the American Academy of Dermatology. 76 (4): 683–688. doi:10.1016/j.jaad.2016.10.029. ISSN 0190-9622.
↑ Sausville EA, Eddy JL, Makuch RW, Fischmann AB, Schechter GP, Matthews M, Glatstein E, Ihde DC, Kaye F, Veach SR (September 1988). "Histopathologic staging at initial diagnosis of mycosis fungoides and the Sézary syndrome. Definition of three distinctive prognostic groups". Ann. Intern. Med. 109 (5): 372–82. PMID 3408055.
↑ Beylot-Barry M, Parrens M, Delaunay M, Thiebault R, Vergier B, DeMascarel A, Dubus P, Beylot C, Merlio JP (June 2005). "Is bone marrow biopsy necessary in patients with mycosis fungoides and Sézary syndrome? A histological and molecular study at diagnosis and during follow-up". Br. J. Dermatol. 152 (6): 1378–9. doi:10.1111/j.1365-2133.2005.06621.x. PMID 15949023.
↑ Yamashita T, Abbade LP, Marques ME, Marques SA (2012). "Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update". An Bras Dermatol. 87 (6): 817–28, quiz 829–30. PMC 3699909. PMID 23197199.
↑ Olek-Hrab K, Silny W (March 2014). "Diagnostics in mycosis fungoides and Sezary syndrome". Rep Pract Oncol Radiother. 19 (2): 72–6. doi:10.1016/j.rpor.2013.11.001. PMC 4054990. PMID 24936324.
↑ Klemke CD, Brade J, Weckesser S, Sachse MM, Booken N, Neumaier M, Goerdt S, Nebe TC (September 2008). "The diagnosis of Sézary syndrome on peripheral blood by flow cytometry requires the use of multiple markers". Br. J. Dermatol. 159 (4): 871–80. doi:10.1111/j.1365-2133.2008.08739.x. PMID 18652582.
↑ Scala E, Abeni D, Palazzo P, Liso M, Pomponi D, Lombardo G, Picchio MC, Narducci MG, Russo G, Mari A (2012). "Specific IgE toward allergenic molecules is a new prognostic marker in patients with Sézary syndrome". Int. Arch. Allergy Immunol. 157 (2): 159–67. doi:10.1159/000327553. PMID 21985996.
↑ Chu AC, Robinson D, Hawk JL, Meacham R, Spittle MF, Smith NP (February 1986). "Immunologic differentiation of the Sézary syndrome due to cutaneous T-cell lymphoma and chronic actinic dermatitis". J. Invest. Dermatol. 86 (2): 134–7. PMID 3528307.
↑ Chand K, Sayal SK, Chand S (April 2007). "Cutaneous T-Cell Lymphoma (Mycosis Fungoides)". Med J Armed Forces India. 63 (2): 188–90. doi:10.1016/S0377-1237(07)80076-1. PMC 4925357. PMID 27407986.
↑ Wilcox RA (January 2016). "Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 91 (1): 151–65. doi:10.1002/ajh.24233. PMC 4715621. PMID 26607183.
↑ Bradford PT, Devesa SS, Anderson WF, Toro JR (May 2009). "Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases". Blood. 113 (21): 5064–73. doi:10.1182/blood-2008-10-184168. PMC 2686177. PMID 19279331.
↑ Saunes M, Nilsen TI, Johannesen TB (February 2009). "Incidence of primary cutaneous T-cell lymphoma in Norway". Br. J. Dermatol. 160 (2): 376–9. doi:10.1111/j.1365-2133.2008.08852.x. PMID 18808419.
↑ Wilcox, Ryan A. (2016). "Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management". American Journal of Hematology. 91 (1): 151–165. doi:10.1002/ajh.24233. ISSN 0361-8609.
↑ Al Hothali GI (June 2013). "Review of the treatment of mycosis fungoides and Sézary syndrome: A stage-based approach". Int J Health Sci (Qassim). 7 (2): 220–39. PMC 3883611. PMID 24421750.
↑ Horesh N, Horowitz NA (October 2014). "Does gender matter in non-hodgkin lymphoma? Differences in epidemiology, clinical behavior, and therapy". Rambam Maimonides Med J. 5 (4): e0038. doi:10.5041/RMMJ.10172. PMC 4222427. PMID 25386354.
↑ ^51.0 ^51.1 Criscione VD, Weinstock MA (July 2007). "Incidence of cutaneous T-cell lymphoma in the United States, 1973-2002". Arch Dermatol. 143 (7): 854–9. doi:10.1001/archderm.143.7.854. PMID 17638728.
↑ Desai M, Liu S, Parker S (February 2015). "Clinical characteristics, prognostic factors, and survival of 393 patients with mycosis fungoides and Sézary syndrome in the southeastern United States: a single-institution cohort". J. Am. Acad. Dermatol. 72 (2): 276–85. doi:10.1016/j.jaad.2014.10.019. PMID 25458019.
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↑ Huang KP, Weinstock MA, Clarke CA, McMillan A, Hoppe RT, Kim YH (January 2007). "Second lymphomas and other malignant neoplasms in patients with mycosis fungoides and Sezary syndrome: evidence from population-based and clinical cohorts". Arch Dermatol. 143 (1): 45–50. doi:10.1001/archderm.143.1.45. PMID 17224541.
↑ Herro E, Dicaudo DJ, Davis MD, Weaver AL, Swanson DL (August 2009). "Review of contemporaneous mycosis fungoides and B-cell malignancy at Mayo Clinic". J. Am. Acad. Dermatol. 61 (2): 271–5. doi:10.1016/j.jaad.2009.03.033. PMID 19481294.
↑ Olsen EA, Delzell E, Jegasothy BV (February 1984). "Second malignancies in cutaneous T cell lymphoma". J. Am. Acad. Dermatol. 10 (2 Pt 1): 197–204. PMID 6609176.
↑ Berg S, Villasenor-Park J, Haun P, Kim EJ (June 2017). "Multidisciplinary Management of Mycosis Fungoides/Sézary Syndrome". Curr Hematol Malig Rep. 12 (3): 234–243. doi:10.1007/s11899-017-0387-9. PMID 28540671.
↑ Hurabielle C, Michel L, Ram-Wolff C, Battistella M, Jean-Louis F, Beylot-Barry M, d'Incan M, Bensussan A, Bagot M (January 2016). "Expression of Sézary Biomarkers in the Blood of Patients with Erythrodermic Mycosis Fungoides". J. Invest. Dermatol. 136 (1): 317–20. doi:10.1038/JID.2015.360. PMID 26763453.
↑ Elise Olsen, Eric Vonderheid, Nicola Pimpinelli, Rein Willemze, Youn Kim, Robert Knobler, Herschel Zackheim, Madeleine Duvic, Teresa Estrach, Stanford Lamberg, Gary Wood, Reinhard Dummer, Annamari Ranki, Gunter Burg, Peter Heald, Mark Pittelkow, Maria-Grazia Bernengo, Wolfram Sterry, Liliane Laroche, Franz Trautinger & Sean Whittaker (2007). "Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)". Blood. 110 (6): 1713–1722. doi:10.1182/blood-2007-03-055749. PMID 17540844. Unknown parameter |month= ignored (help)
↑ Haththotuwa R, Zilinskiene L, Oliff J, Vydianath B, Amel-Kashipaz R, Stevens A, Shah F, Chaganti S, Scarisbrick J (September 2017). "Biopsy correlation of surface area vs. single-axis measurements on computed tomography scan of lymph nodes in patients with erythrodermic mycosis fungoides and Sézary syndrome". Br. J. Dermatol. 177 (3): 877–878. doi:10.1111/bjd.15266. PMID 28012157.
↑ Vij A, Duvic M (August 2012). "Prevalence and severity of pruritus in cutaneous T cell lymphoma". Int. J. Dermatol. 51 (8): 930–4. doi:10.1111/j.1365-4632.2011.05188.x. PMID 22788808.
↑ Ferreira BI, Abreu JL, Reis JP, Figueiredo AM (June 2016). "Psoriasis and Associated Psychiatric Disorders: A Systematic Review on Etiopathogenesis and Clinical Correlation". J Clin Aesthet Dermatol. 9 (6): 36–43. PMC 4928455. PMID 27386050.
↑ Olek-Hrab K, Silny W (March 2014). "Diagnostics in mycosis fungoides and Sezary syndrome". Rep Pract Oncol Radiother. 19 (2): 72–6. doi:10.1016/j.rpor.2013.11.001. PMC 4054990. PMID 24936324.
↑ Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016
↑ Foss, Francine M.; Girardi, Michael (2017). "Mycosis Fungoides and Sezary Syndrome". Hematology/Oncology Clinics of North America. 31 (2): 297–315. doi:10.1016/j.hoc.2016.11.008. ISSN 0889-8588.
↑ Sibaud, Vincent; Beylot-Barry, Marie; Thiébaut, Rodolphe; Parrens, Marie; Vergier, Béatrice; Delaunay, Michèle; Beylot, Claire; Chêne, Geneviève; Ferrer, Jacky; de Mascarel, Antoine; Dubus, Pierre; Merlio, Jean Philippe (2003). "Bone Marrow Histopathologic and Molecular Staging in Epidermotropic T-Cell Lymphomas". American Journal of Clinical Pathology. 119 (3): 414–423. doi:10.1309/QH6XLRF3MVUF2M8M. ISSN 0002-9173.
↑ Janiga, Jenna; Kentley, Jonathan; Nabhan, Chadi; Abdulla, Farah (2018). "Current systemic therapeutic options for advanced mycosis fungoides and Sézary syndrome". Leukemia & Lymphoma. 59 (3): 562–577. doi:10.1080/10428194.2017.1347650. ISSN 1042-8194.
↑ Olsen EA, Rook AH, Zic J, Kim Y, Porcu P, Querfeld C, Wood G, Demierre MF, Pittelkow M, Wilson LD, Pinter-Brown L, Advani R, Parker S, Kim EJ, Junkins-Hopkins JM, Foss F, Cacchio P, Duvic M (February 2011). "Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)". J. Am. Acad. Dermatol. 64 (2): 352–404. doi:10.1016/j.jaad.2010.08.037. PMID 21145619.
↑ Whittaker SJ, Marsden JR, Spittle M, Russell Jones R (December 2003). "Joint British Association of Dermatologists and U.K. Cutaneous Lymphoma Group guidelines for the management of primary cutaneous T-cell lymphomas". Br. J. Dermatol. 149 (6): 1095–1107. PMID 14696593.
↑ Quaglino P, Fierro MT, Rossotto GL, Savoia P, Bernengo MG (February 2004). "Treatment of advanced mycosis fungoides/Sézary syndrome with fludarabine and potential adjunctive benefit to subsequent extracorporeal photochemotherapy". Br. J. Dermatol. 150 (2): 327–36. PMID 14996105.
↑ ^71.0 ^71.1 ^71.2 ^71.3 Olsen EA, Rook AH, Zic J, Kim Y, Porcu P, Querfeld C, Wood G, Demierre MF, Pittelkow M, Wilson LD, Pinter-Brown L, Advani R, Parker S, Kim EJ, Junkins-Hopkins JM, Foss F, Cacchio P, Duvic M (February 2011). "Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)". J. Am. Acad. Dermatol. 64 (2): 352–404. doi:10.1016/j.jaad.2010.08.037. PMID 21145619.
↑ Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016

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@@ Line 182: / Line 182: @@
 ==Causes==
 * The cause of Sezary syndrome has not been identified.<ref name="pmid21883142">{{cite journal |vauthors=Wong HK, Mishra A, Hake T, Porcu P |title=Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome) |journal=Br. J. Haematol. |volume=155 |issue=2 |pages=150–66 |date=October 2011 |pmid=21883142 |pmc=4309373 |doi=10.1111/j.1365-2141.2011.08852.x |url=}}</ref><ref name="pmid27121473">{{cite journal |vauthors=Woollard WJ, Pullabhatla V, Lorenc A, Patel VM, Butler RM, Bayega A, Begum N, Bakr F, Dedhia K, Fisher J, Aguilar-Duran S, Flanagan C, Ghasemi AA, Hoffmann RM, Castillo-Mosquera N, Nuttall EA, Paul A, Roberts CA, Solomonidis EG, Tarrant R, Yoxall A, Beyers CZ, Ferreira S, Tosi I, Simpson MA, de Rinaldis E, Mitchell TJ, Whittaker SJ |title=Candidate driver genes involved in genome maintenance and DNA repair in Sézary syndrome |journal=Blood |volume=127 |issue=26 |pages=3387–97 |date=June 2016 |pmid=27121473 |doi=10.1182/blood-2016-02-699843 |url=}}</ref>
-* In sezary syndrome maybe one or more [[chromosomal]] [[abnormalities]], such as the loss or gain of [[Genetics|genetic]].<ref name="pmid271214732" /><ref name="pmid266071832" />
+* Sezary syndrome might have one or more of the [[chromosomal]] [[abnormalities]], such as the loss or gain of [[Genetics|genetic]].<ref name="pmid271214732" /><ref name="pmid266071832" />
 ==Clinical Features==