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Sézary's disease

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

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.

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1 General
2 Etiology
3 Symptoms
4 Treatment
5 References

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

^ synd/3594 at Who Named It

584056846 at GPnotebook

v • d • e Hematological malignancy histology (ICD-O 9590-9989)
Lymphomas (9590-9759)	Hodgkin's lymphoma vs. Non-Hodgkin lymphoma - Diffuse lymphoma vs. Follicular lymphoma B-cell lymphoma (Small cell, Primary effusion, Diffuse large, ,Burkitt's, Splenic marginal zone, MALT) T-cell lymphoma (Cutaneous , Mycosis fungoides/Sézary's disease, Angioimmunoblastic, Anaplastic large cell, Hepatosplenic) plasma cell (Plasmacytoma, Multiple myeloma) mast cell tumor (Mast-cell sarcoma, Malignant mastocytosis, Malignant histiocytosis, Langerhans cell histiocytosis)
Immunoproliferative disorders (9760-9799)	Waldenström macroglobulinemia - Lymphomatoid granulomatosis
Lymphoid leukemias (9800-9839)	ALL - CLL - T-cell leukemia (Adult, Large granular lymphocyte, Prolymphocytic, Acute lymphoblastic) - B-cell leukemia (Prolymphocytic)
Myeloid leukemias (9840-9939, 9963)	AML (M2, APL/M3, AMoL/M5, Erythroleukemia/M6) - CML (CMoL, CNL, Philadelphia chromosome) - Granulocytic sarcoma
Other leukemias (9940-9949)	Hairy cell leukemia - Aggressive NK-cell leukemia
Myeloproliferative disease (9950-9961)	Polycythemia vera - Essential thrombocytosis - Myelofibrosis
Other (9964-9989)	Hypereosinophilic syndrome - Post-transplant lymphoproliferative disorder - Myelodysplastic syndrome