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 (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 |
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]
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]
- Mimic psoriasis
- Chronic eczema
- Atopic dermatitis
- Leprosy
- Lichenoid pityriasis
- 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
- Mycosis fangoides
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
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 :
- 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]
- Most common symtom of Sezary syndrome is pruritus.
Physical Examination
- Patients with Sezary syndrome usually appear with skin signs and symptoms.
- Physical examination may be remarkable for:
- Skin lesions
- Enlarged lymph nodes
- Fingernails and toenails abnormalities
- lower eyelides
- trouble regulating body temperature
- Splenomegaly
- Hepatomegaly
- Gastrointestinal trac
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]
- 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
- 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
- ↑ Steffen C (August 2006). "The man behind the eponym dermatology in historical perspective: Albert Sézary and the Sézary syndrome". Am J Dermatopathol. 28 (4): 357–67. PMID 16871044.
- ↑ 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.
- ↑ 6.0 6.1 Saulite, Ieva; Hoetzenecker, Wolfram; Weidinger, Stephan; Cozzio, Antonio; Guenova, Emmanuella; Wehkamp, Ulrike (2016). "Sézary Syndrome and Atopic Dermatitis: Comparison of Immunological Aspects and Targets". BioMed Research International. 2016: 1–15. doi:10.1155/2016/9717530. ISSN 2314-6133.
- ↑ Balfour EM, Glusac EJ, Heald P, Talley LL, Smoller BR (August 2003). "Sezary syndrome: cutaneous immunoperoxidase double-labeling technique demonstrates CD4/CD8 ratio non-specificity". J. Cutan. Pathol. 30 (7): 437–42. PMID 12859741.
- ↑ 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.
- ↑ Fuhlbrigge RC, Kieffer JD, Armerding D, Kupper TS (October 1997). "Cutaneous lymphocyte antigen is a specialized form of PSGL-1 expressed on skin-homing T cells". Nature. 389 (6654): 978–81. doi:10.1038/40166. PMID 9353122.
- ↑ Graham RL, Burch M, Krause JR (July 2014). "Adult T-cell leukemia/lymphoma". Proc (Bayl Univ Med Cent). 27 (3): 235–8. PMC 4059578. PMID 24982574.
- ↑ 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.
- ↑ Introcaso CE, Hess SD, Kamoun M, Ubriani R, Gelfand JM, Rook AH (September 2005). "Association of change in clinical status and change in the percentage of the CD4+CD26- lymphocyte population in patients with Sézary syndrome". J. Am. Acad. Dermatol. 53 (3): 428–34. doi:10.1016/j.jaad.2005.06.001. PMID 16112348.
- ↑ Gorczyca, Wojciech; Weisberger, James; Liu, Z.; Tsang, Patricia; Hossein, Monowar; Wu, C. Daniel; Dong, Henry; Wong, John Y.L.; Tugulea, Sorina; Dee, Simpson; Melamed, Myron R.; Darzynkiewicz, Zbigniew (2002). "An approach to diagnosis of T-cell lymphoproliferative disorders by flow cytometry". Cytometry. 50 (3): 177–190. doi:10.1002/cyto.10003. ISSN 0196-4763.
- ↑ Pulitzer M (September 2017). "Cutaneous T-cell Lymphoma". Clin. Lab. Med. 37 (3): 527–546. doi:10.1016/j.cll.2017.06.006. PMC 5710803. PMID 28802499.
- ↑ Kim EJ, Hess S, Richardson SK, Newton S, Showe LC, Benoit BM, Ubriani R, Vittorio CC, Junkins-Hopkins JM, Wysocka M, Rook AH (April 2005). "Immunopathogenesis and therapy of cutaneous T cell lymphoma". J. Clin. Invest. 115 (4): 798–812. doi:10.1172/JCI24826. PMC 1070436. PMID 15841167.
- ↑ Kashanchi, Fatah; Coelho-dos-Reis, Jordana Grazziela Alves; Passos, Livia; Duarte, Mariana Costa; Araújo, Marcelo Grossi; Campi-Azevedo, Ana Carolina; Teixeira-Carvalho, Andréa; Peruhype-Magalhães, Vanessa; Trindade, Bruno Caetano; dos Santos Dias, Raquel; Martins, Marina Lobato; Carneiro-Proietti, Anna Barbara de Freitas; Guedes, Antônio Carlos; Gonçalves, Denise Utsch; Martins-Filho, Olindo Assis (2013). "Immunological Profile of HTLV-1-Infected Patients Associated with Infectious or Autoimmune Dermatological Disorders". PLoS Neglected Tropical Diseases. 7 (7): e2328. doi:10.1371/journal.pntd.0002328. ISSN 1935-2735.
- ↑ Myskowski, Patricia L. (1991). "Cutaneous T-Cell Lymphoma and Human Immunodeficiency Virus". Archives of Dermatology. 127 (7): 1045. doi:10.1001/archderm.1991.01680060119017. ISSN 0003-987X.
- ↑ 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.
- ↑ Arafah M, Zaidi SN, Kfoury HK, Al Rikabi A, Al Ghamdi K (March 2012). "The Histological Spectrum of Early Mycosis Fungoides: A Study of 58 Saudi Arab patients". Oman Med J. 27 (2): 134–9. doi:10.5001/omj.2012.28. PMC 3321345. PMID 22496939.
- ↑ Chu AC, Morris JF (May 1989). "Sézary cell morphology induced in peripheral blood lymphocytes: re-evaluation". Blood. 73 (6): 1603–7. PMID 2653459.
- ↑ Vonderheid EC, Bernengo MG, Burg G, Duvic M, Heald P, Laroche L, Olsen E, Pittelkow M, Russell-Jones R, Takigawa M, Willemze R (January 2002). "Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas". J. Am. Acad. Dermatol. 46 (1): 95–106. PMID 11756953.
- ↑ Guitart J (September 2005). "Beyond clonal detection: defining the T-cell clone". Arch Dermatol. 141 (9): 1159–60. doi:10.1001/archderm.141.9.1159. PMID 16172316.
- ↑ Kirsch IR, Watanabe R, O'Malley JT, Williamson DW, Scott LL, Elco CP, Teague JE, Gehad A, Lowry EL, LeBoeuf NR, Krueger JG, Robins HS, Kupper TS, Clark RA (October 2015). "TCR sequencing facilitates diagnosis and identifies mature T cells as the cell of origin in CTCL". Sci Transl Med. 7 (308): 308ra158. doi:10.1126/scitranslmed.aaa9122. PMC 4765389. PMID 26446955.
- ↑ 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.
- ↑ Sokołowska-Wojdyło M, Olek-Hrab K, Ruckemann-Dziurdzińska K (October 2015). "Primary cutaneous lymphomas: diagnosis and treatment". Postepy Dermatol Alergol. 32 (5): 368–83. doi:10.5114/pdia.2015.54749. PMC 4692822. PMID 26759546.
- ↑ 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.
- ↑ Olsen, Elise A. (2015). "Evaluation, Diagnosis, and Staging of Cutaneous Lymphoma". Dermatologic Clinics. 33 (4): 643–654. doi:10.1016/j.det.2015.06.001. ISSN 0733-8635.
- ↑ Panda, Saumya (2007). "Mycosis fungoides: Current trends in diagnosis and management". Indian Journal of Dermatology. 52 (1): 5. doi:10.4103/0019-5154.31918. ISSN 0019-5154.
- ↑ 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.
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- ↑ 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.
- ↑ Blaizot R, Ouattara E, Fauconneau A, Beylot-Barry M, Pham-Ledard A (August 2018). "Infectious events and associated risk factors in mycosis fungoides/Sézary syndrome: a retrospective cohort study". Br. J. Dermatol. doi:10.1111/bjd.17073. PMID 30098016.
- ↑ 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.
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- ↑ 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.
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- ↑ 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