Cutaneous T cell lymphoma
Cutaneous T cell lymphoma
Classification & external resources
| ICD-10 |
C84.0, C84.1 |
| ICD-9 |
202.1, 202.2 |
| ICD-O: |
M9700/3, M9701/3 |
| DiseasesDB |
8595 |
| eMedicine |
med/3486 |
| MeSH |
D016410 |
Cutaneous T-Cell lymphoma (CTCL) is a class of non-Hodgkin's lymphoma, which is a type of cancer of the immune system. Unlike most non-Hodgkin's lymphomas (which are generally B-cell related), CTCL is caused by a mutation of T cells. The malignant T cells in the body are pushed to the surface of the skin in a biological process used to rid the body of offending material, causing various lesions to appear on the skin. These lesions change shape as the disease progresses, typically beginning as what appears to be a rash and eventually forming plaques and tumors before metastatizing to other parts of the body.
Types
Though there are many types of CTCL and presentation can vary from individual to individual, there are two main forms:
There is some dispute over whether SS is an advanced form of MF or a different type of CTCL entirely - in either case, the prognosis for SS patients is substantially poorer than for those of MF.
Treatment
There is no cure for CTCL, but there are a variety of treatment options available and some CTCL patients are able to live somewhat normal lives with this cancer, although symptoms can be debilitating and painful, even in earlier stages.
Treatments include: Topical Corticosteroids, Bexarotene Gel, Carmustine(Nitrogen Mustard), Mechlorethamine, Phototherapy (Broad & Narrow Band UVB or PUVA), Local & Total Skin Electron Beam Radiation, Conventional Radiation Therapy, Oral Corticosteroids, Bexarotene (Targretin®) Capsules, Photopheresis, Interferons, Denileukin Diftitox (Ontak®), Alemtuzumab (Campath-1H), Vorinostat (Zolinza®), Methotrexate, Pentostatin & other purine analogues (Fludarabine, 2- deoxychloroadenosine), Liposomal doxorubicin (Doxil®), Gemcitabine (Gemzar®), Cyclophosphamide, oral, Bone marrow/Stem cell, and Allogenic Transplantation.
See also
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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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