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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.
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
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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 |
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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Sézary's_disease". A list of authors is available in Wikipedia.
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