Sézary's disease

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Sézary's disease Classification and external resources
ICD-10	C84.1
ICD-9	202.2
ICD-O:	M9701/3
DiseasesDB	8595
MeSH	D012751

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Overview

Sézary's disease (or "Sézary syndrome") is a type of cutaneous lymphoma characterized by Albert Sézary. ^[1]

"Sézary's cells" are T-cells that have pathological quantities of mucopolysaccharides.

Sézary's disease is sometimes considered a late stage of mycosis fungoides.

General

Sezary syndrome and Mycosis Fungoides are T-cell lymphomas whose primary manifestation is in the skin.

Etiology

HTLV has thought to have been associated with both of the above cutaneous mycoses.

Symptoms

The hallmark symptoms for Sezary Syndrome are (1) Generalized erythroderma (2)Lymphadenopathy, and (3) Atypical T-cells ("Sezary cells") in the peripheral blood.

Treatment

Vorinostat (Zolinza®) is a second-line drug for CTCL. Treatments are often used in combination.

References

External links

• Illustration of Sezary cells
• Illustration of Sezary cells
• Biography of Sezary (in French)
• Sezary Syndrome lymphoma information
• Doctor's doctor
• Cutaneous Lymphoma Foundation
• [http://www.clinicaltrials.gov/ct/show/NCT00106431 Clinical Trial for Sezary Syndrome

• 584056846 at GPnotebook

v • d • e Pathology: hematology, myeloid hematologic disease (primarily D50-D77, 280-289)
RBCs/ hemoglobinopathy	+ Polycythemia - Macrocytosis - Anemia Nutritional Iron deficiency anemia (Plummer-Vinson syndrome), Megaloblastic anemia (Pernicious anemia) Hemolytic Hereditary enzyme: G6PD Deficiency - Pyruvate kinase deficiency - Triosephosphate isomerase deficiency hemoglobin: Thalassemia - Sickle-cell disease/trait membrane: Hereditary spherocytosis - Hereditary elliptocytosis - Hereditary stomatocytosis Acquired Autoimmune (Warm, Cold), HUS, MAHA, PNH, PCH, Myelophthisic Aplastic Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia, Sideroblastic anemia Blood tests Normocytic - Microcytic - Macrocytic - Normochromic - Hypochromic Other Methemoglobinemia
Coagulation/platelets/ coagulopathy/ bleeding diathesis	+ Hypercoagulability primary: Antithrombin III deficiency - Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden - Hyperprothrombinemia acquired: DIC (Congenital afibrinogenemia, Purpura fulminans) - autoimmune (Antiphospholipid) Other Essential thrombocytosis - clotting factor: Hemophilia (A/VIII, B/IX, C/XI) • Von Willebrand disease • Hypoprothrombinemia/II - XIII platelet function: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Gray platelet syndrome - May Hegglin anomaly - Pelger-Huet anomaly Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP Thrombocytopenia (Heparin-induced thrombocytopenia)
Monocytes/ macrophages	+ Histiocytosis WHO-I (Langerhans cell histiocytosis) WHO-II/non-Langerhans-cell (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) WHO-III/malignant (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease) Other Chronic granulomatous disease -cytosis: Monocytosis - -penia: Monocytopenia
Granulocytes	+ -cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia) - -penia: Granulocytopenia/agranulocytosis (Neutropenia, Kostmann syndrome)
See also hematological malignancy and immune disorders

v • d • e WikiDoc Research Resources for Sézary's disease (Click show to right to view)
Articles on Sézary's disease	Most recent articles on Sézary's disease • Most cited articles on Sézary's disease • Review articles on Sézary's disease • Articles on Sézary's disease in N Eng J Med, Lancet, BMJ
Media (Slides, Video, Images, MP3) on Sézary's disease	Powerpoint slides on Sézary's disease • Images of Sézary's disease • Photos of Sézary's disease • Podcasts & MP3s on Sézary's disease • Videos on Sézary's disease
Evidence Based Medicine Regarding Sézary's disease	Cochrane Collaboration on Sézary's disease • Bandolier on Sézary's disease • TRIP on Sézary's disease
Cost Effectiveness of Sézary's disease	Cost Effectiveness of Sézary's disease
Clinical Trials Involving Sézary's disease	Ongoing Trials on Sézary's disease at Clinical Trials.gov • Trial results on Sézary's disease • Clinical Trials on Sézary's disease at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Sézary's disease	US National Guidelines Clearinghouse on Sézary's disease • NICE Guidance on Sézary's disease • NHS PRODIGY Guidance • FDA on Sézary's disease • CDC on Sézary's disease
Textbook Information on Sézary's disease	Books and Textbook Information on Sézary's disease
Pharmacology Resources on Sézary's disease	Dosing of Sézary's disease • Drug interactions with Sézary's disease • Side effects of Sézary's disease • Allergic reactions to Sézary's disease • Overdose information on Sézary's disease • Carcinogenicity information on Sézary's disease • Sézary's disease in pregnancy • Pharmacokinetics of Sézary's disease •
Genetics, Pharmacogenomics, and Proteinomics of Sézary's disease	Genetics of Sézary's disease • Pharmacogenomics of Sézary's disease • Proteomics of Sézary's disease
Newstories on Sézary's disease	Sézary's disease in the news • Be alerted to news on Sézary's disease • News trends on Sézary's disease
Commentary on Sézary's disease	Blogs on Sézary's disease
Patient Resources on Sézary's disease	Patient resources on Sézary's disease • Discussion groups on Sézary's disease • Patient Handouts on Sézary's disease • Directions to Hospitals Treating Sézary's disease • Risk calculators and risk factors for Sézary's disease
Healthcare Provider Resources on Sézary's disease	Symptoms of Sézary's disease • Causes & Risk Factors for Sézary's disease • Diagnostic studies for Sézary's disease • Treatment of Sézary's disease
Continuing Medical Education (CME) Programs on Sézary's disease	CME Programs on Sézary's disease
International Resources on Sézary's disease	Sézary's disease en Espanol • Sézary's disease en Francais
Business Resources on Sézary's disease	Sézary's disease in the Marketplace • Patents on Sézary's disease
Informatics Resources on Sézary's disease	List of terms related to Sézary's disease

de:Sézary-Syndrom

ja:セザリー症候群

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .