Sezary syndrome: Difference between revisions
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=== Symptoms === | === Symptoms === | ||
*Symptoms of Sezary syndrome may include the following: | *Symptoms of Sezary syndrome may include the following: | ||
:* | :*Most common symtom of Sezary syndrome is pruritus | ||
:*[symptom 2] | :*[symptom 2] | ||
:*[symptom 3] | :*[symptom 3] |
Revision as of 19:23, 14 November 2018
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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.[1]
Classification
If the staging system involves specific and characteristic findings and features:
The staging of sezazry syndrome is based on the TNMB:[2]
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:[2][3]
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.[4]
- Cutaneous T cell lymphoma arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response.[5]
- 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.
- Sezary syndrome and mycosis fungoides are T-cell lymphomas that primary manifest as multiple cutaneous lesions.
- 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.
- A few patients of Sezary syndrome are associated with human T lymphotropic viruses types 1 and 2 (HTLV1 and HTLV2) In Japan, Caribbean islands, and the Middle East.[6][7]
- 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.[8]
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.[9]
- Cutaneous T cell lymphoma is usually initially seen by dermatologists with patients presenting with skin lesions such as erythematous patches or plaque.[10]
- Patients with Sezary syndrome often have a history of several years of eczematous or dermatitis skin lesions before the diagnosis is finally established.[11]
- The skin lesions then progress from the patch stage to the plaque stage to cutaneous tumors
Signs
Common signs of Sezary syndrome include:[12]
- 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)[13]
- The severity of erythema body surface area (BSA) involved may wax and wane.
Skin lesions
The other skin lesion symptoms of Sezary syndrome are
- Patches and plaques to erythroderma
- Keratosis pilaris
- Alopecia
- Ectropion
- Keratoderma
- Hypertrophic nails
- Erosions
- Lichenificatio
Other Signs
Some patients with Sezary syndrome have[14][15]
- Lymphadenopathy
- Viscer
Differentiating Sezary syndrome from other Diseases
- Sezary syndrome must be differentiated from other diseases that cause:
- Mycosis fangoides
- Sezaruy syndrome vis 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 [16]
- Eczema
- Psoriasis
- Mycosis fangoides
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.[17][18]
Age
- The median age at diagnosis of Sézary syndrome is 60 years of age(SS).[19]
- Sezary syndrome is more commonly observed among elderly patients.[20]
Gender
- Males are more commonly affected with Sezary syndrome than females(2:1).[21]
Race
- Sezary syndrome usually affects individuals of the whites race.[22]
- Sezary syndrome is rare disease that tends to affect Whites [22] but in this study African american has more percentage[23]
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
- Early clinical features include mimic psoriasis, chronic eczema, atopic dermatitis, leprosy or lichenoid pityriasis.
- 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
- Symptoms of Sezary syndrome may include the following:
- Most common symtom of Sezary syndrome is pruritus
- [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 .[24]
- 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:[25]
- 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).[26]
- 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. [27]
Stage | PUVA | Topical chemotherapy | Systemic chemotherapy | Radiotherapy | Biological therapy | Retinoid therapy | Photopheresis |
---|---|---|---|---|---|---|---|
Stage I |
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Stage II |
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Stage III |
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Stage IV |
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Recurrent cutaneous T cell lymphoma |
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--------- | --------- |
Treatment | Description | |
---|---|---|
Phototherapy or Ultraviolet light therapy | ||
PUVA (psoralen and ultraviolet A light therapy) |
| |
Ultraviolet B (UVB) light |
| |
Chemotherapy | ||
Topical chemotherapy |
| |
Systemic chemotherapy |
| |
Radiation therapy | ||
Local external beam radiation therapy |
| |
Total skin electron beam (TSEB) therapy |
| |
Biological therapy | ||
Interferon alfa |
| |
Denileukin diftitox |
| |
Retinoid therapy | ||
Retinoids |
| |
Photopheresis | ||
Photopheresis |
|
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].
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.
- ↑ 2.0 2.1 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ "Extracorporeal photophoresis: an evidence-based analysis". Ont Health Technol Assess Ser. 6 (6): 1–82. 2006. PMC 3379535. PMID 23074497.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 22.0 22.1 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.
- ↑ 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
- ↑ 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.
- ↑ 27.0 27.1 27.2 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