セザリー症候群
- 関
- also Lym phoma, cutaneous Tcell
WordNet
- a pattern of symptoms indicative of some disease
- a complex of concurrent things; "every word has a syndrome of meanings"
PrepTutorEJDIC
- (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/11/10 11:14:44」(JST)
[Wiki en表示]
| Sézary disease |
| Classification and external resources |
Sézary syndrome This 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).
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| ICD-10 |
C84.1 |
| ICD-9 |
202.2 |
| ICD-O: |
M9701/3 |
| DiseasesDB |
8595 |
| eMedicine |
med/1541 derm/566 med/3486 |
| MeSH |
D012751 |
Sézary's disease (often called Sézary syndrome) is a type of cutaneous lymphoma that was first described by Albert Sézary.[1] The affected cells are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.[2][3] There are currently no known causes of Sézary's disease.[4]
Contents
- 1 Signs and symptoms
- 2 Diagnosis
- 3 Treatment
- 4 Epidemiology
- 5 See also
- 6 References
- 7 External links
Signs and symptoms[edit]
Sézary cells: pleomorphic abnormal T cells with convoluted nucleus (Peripheral blood - MGG stain)
Sézary syndrome and mycosis fungoides are T-cell lymphomas whose primary manifestation is in the skin.[5] The disease's origin is a peripheral CD4+ T-lymphocyte,[2] although rarer CD8+/CD4- cases have been observed.[2] Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease.[2] The dominant symptoms of the disease are:
- Generalized erythroderma[2]
- Lymphadenopathy[2]
- Atypical T-cells ("Sézary cells") in the peripheral blood[2]
- Hepatosplenomegaly[6]
Diagnosis[edit]
Patients who have Sézary's disease often present with skin lesions that do not heal with normal medication.[7] A blood test generally reveals any change in the levels of lymphocytes in the blood, which is often associated with a cutaneous T-cell lymphoma.[7] Finally, a biopsy of a skin lesion can be performed to rule out any other causes.[7]
Treatment[edit]
Vorinostat (Zolinza) is a second-line drug for cutaneous T-cell lymphoma.[citation needed] Treatments are often used in combination with phototherapy and chemotherapy.[2] No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.[5]
Epidemiology[edit]
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma.[2] In the western population there are around 0.3 cases of Sezary syndrome per 100,000 people.[2] Sézary disease is more common in males with a ratio of 2:1,[2] and the mean age of diagnosis is between 55 and 60 years of age.[2][6]
See also[edit]
- Cutaneous T-cell lymphoma
- List of cutaneous conditions
References[edit]
- ^ synd/3594 at Who Named It?
- ^ a b c d e f g h i j k l Cuneo A, Castoldi. "Mycosis fungoidses/Sezary's syndrome". Retrieved 2008-02-15.
- ^ Thangavelu, Maya. "Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome". Retrieved 2008-02-15.
- ^ "Causes and Symptoms". Retrieved 2008-02-15.
- ^ a b Cerroni, Lorenzo; Kevin Gatter, Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 39. ISBN 978-1-4051-1376-2.
- ^ a b Lorincz, A. I. "Sezary syndrome". Retrieved 2008-02-15.
- ^ a b c "Diagnosis". Retrieved 2008-02-15.
External links[edit]
- Illustration of Sezary cells
- Biography of Sezary (in French)
- Sezary Syndrome lymphoma information
- Doctor's doctor
- Cutaneous Lymphoma Foundation
- Clinical Trial for Sezary Syndrome
- Skin Research Center lab Hopital St Louis, Paris (France) Dir. Dr. A. Bensussan
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Hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)
Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590–9739, 9800–9839)
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B cell
(lymphoma,
leukemia)
(most CD19
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By development/
marker
|
|
TdT+
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- ALL (Precursor B acute lymphoblastic leukemia/lymphoma)
|
|
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CD5+
|
mantle zone (Mantle cell)
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|
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CD22+
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- Prolymphocytic
- CD11c+ (Hairy cell leukemia)
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CD79a+
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- germinal center/follicular B cell (Follicular
- Burkitt's
- GCB DLBCL
- Primary cutaneous follicular lymphoma)
marginal zone/marginal-zone B cell (Splenic marginal zone
- MALT
- Nodal marginal zone
- Primary cutaneous marginal zone lymphoma)
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RS (CD15+, CD30+)
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- Classic Hodgkin's lymphoma (Nodular sclerosis)
- CD20+ (Nodular lymphocyte predominant Hodgkin's lymphoma)
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PCDs/PP
(CD38+/CD138+)
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- see immunoproliferative immunoglobulin disorders
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|
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By infection
|
- KSHV (Primary effusion)
- EBV (Lymphomatoid granulomatosis
- Post-transplant lymphoproliferative disorder)
- HIV (AIDS-related lymphoma)
- Helicobacter pylori (MALT lymphoma)
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|
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Cutaneous
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- Diffuse large B-cell lymphoma
- Intravascular large B-cell lymphoma
- Primary cutaneous marginal zone lymphoma
- Primary cutaneous immunocytoma
- Plasmacytoma
- Plasmacytosis
- Primary cutaneous follicular lymphoma
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|
|
|
T/NK
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T cell
(lymphoma,
leukemia)
(most CD3
|
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By development/
marker
|
- TdT+: ALL (Precursor T acute lymphoblastic leukemia/lymphoma)
- prolymphocyte (Prolymphocytic)
- CD30+ (Anaplastic large-cell lymphoma
- Lymphomatoid papulosis type A)
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|
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Cutaneous
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MF+variants
|
- indolent: Mycosis fungoides
- Pagetoid reticulosis
- Granulomatous slack skin
aggressive: Sézary's disease
- Adult T-cell leukemia/lymphoma
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|
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Non-MF
|
- CD30-: Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
- Pleomorphic T-cell lymphoma
- Lymphomatoid papulosis type B
CD30+: CD30+ cutaneous T-cell lymphoma
- Secondary cutaneous CD30+ large cell lymphoma
- Lymphomatoid papulosis type A
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Other peripheral
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- Hepatosplenic
- Angioimmunoblastic
- Enteropathy-associated T-cell lymphoma
- Peripheral T-cell lymphoma-Not-Otherwise-Specified (Lennert lymphoma)
- Subcutaneous T-cell lymphoma
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|
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By infection
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- HTLV-1 (Adult T-cell leukemia/lymphoma)
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NK cell/
(most CD56)
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- Aggressive NK-cell leukemia
- Blastic NK cell lymphoma
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T or NK
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- EBV (Extranodal NK-T-cell lymphoma/Angiocentric lymphoma)
- Large granular lymphocytic leukemia
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Lymphoid+myeloid
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- Acute biphenotypic leukaemia
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Lymphocytosis
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- Lymphoproliferative disorders (X-linked lymphoproliferative disease
- Autoimmune lymphoproliferative syndrome)
- Leukemoid reaction
- Diffuse infiltrative lymphocytosis syndrome
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|
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| Cutaneous lymphoid hyperplasia |
- Cutaneous lymphoid hyperplasia
- with bandlike and perivascular patterns
- with nodular pattern
- Jessner lymphocytic infiltrate of the skin
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cell/phys/auag/auab/comp, igrc
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UpToDate Contents
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English Journal
- Acitretin for the treatment of cutaneous T-cell lymphoma.
- Cheeley J, Sahn RE, Delong LK, Parker SR.SourceDepartment of Dermatology, Emory University, Atlanta, Georgia.
- Journal of the American Academy of Dermatology.J Am Acad Dermatol.2013 Feb;68(2):247-54. doi: 10.1016/j.jaad.2012.07.013. Epub 2012 Aug 20.
- BACKGROUND: Bexarotene is the only Food and Drug Administration-approved retinoid for the treatment of cutaneous T-cell lymphoma (CTCL) and is associated with a relatively high frequency of adverse effects. Acitretin has anecdotally been reported to be effective for CTCL.OBJECTIVE: We sought to dete
- PMID 22917895
- Incidence and survival patterns of cutaneous T-cell lymphomas in the United States.
- Imam MH, Shenoy PJ, Flowers CR, Phillips A, Lechowicz MJ.SourceDepartment of Internal Medicine, Emory University , Atlanta, GA , USA.
- Leukemia & lymphoma.Leuk Lymphoma.2013 Jan 7. [Epub ahead of print]
- Using the United States Surveillance, Epidemiology and End Results (SEER) 17 dataset, we examined incidence and survival patterns for patients with cutaneous T-cell lymphomas (CTCLs) diagnosed following institution of the World Health Organization-European Organisation for Research and Treatment of
- PMID 23004352
- T-Cell Non-Hodgkin's Lymphomas Reported to the FDA AERS With Tumor Necrosis Factor-Alpha (TNF-α) Inhibitors: Results of the REFURBISH Study.
- Deepak P, Sifuentes H, Sherid M, Stobaugh D, Sadozai Y, Ehrenpreis ED.SourceDepartment of Gastroenterology, NorthShore University Health System, Evanston, Illinois, USA.
- The American journal of gastroenterology.Am J Gastroenterol.2013 Jan;108(1):99-105. doi: 10.1038/ajg.2012.334. Epub 2012 Oct 2.
- OBJECTIVES: The risk of non-Hodgkin's lymphoma (NHL) with tumor necrosis factor alpha (TNF-α) inhibitors is unclear, whether related to concomitant thiopurines usage or due to the underlying inflammatory disease. We sought to review all cases of T-cell NHL reported to the Food and Drug Administrati
- PMID 23032984
Japanese Journal
- 菌状息肉症とSezary症候群のバイオマーカーを読み解く (特集 皮膚T細胞リンパ腫研究)
- Allogeneic hematopoietic stem cell transplantation following reduced-intensity conditioning for mycosis fungoides and Sezary syndrome
- 症例報告 同種骨髄移植を施行したSezary症候群の1例
- 臨床皮膚科 = Japanese journal of clinical dermatology 69(11), 853-858, 2015-10
- NAID 40020602596
Related Links
- セザリー症候群（セザリーしょうこうぐん、英: Sézary syndrome）は、皮膚の悪性 リンパ腫の一種であり、全身が赤くなり痒みが出現し、発熱とリンパ節腫脹も伴う疾患。 フランスの皮膚科医アルベール・セザリー (1880-1956) が報告した。 菌状息肉症と 同じく、 ...
- Sézary's disease (often called Sézary syndrome) is a type of cutaneous lymphoma that was first described by Albert Sézary. The affected cells are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is ...
Related Pictures
★リンクテーブル★
[★]
- 英
- Sezary syndrome, Sezary syndrome, SS
- 同
- Sezary症候群 Sezary症候群、セザリー-ブヴラン症候群 Sezary-Bouvrain syndrome
- 関
- [[]]
[★]
