Kalpana Kalpana (Editor)

Cutaneous T cell lymphoma

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Covid-19
ICD-10  C84.0, C84.1
ICD-O  M9700/3, M9701/3
eMedicine  med/3486
ICD-9-CM  202.1, 202.2
DiseasesDB  8595
Cutaneous T cell lymphoma
Specialty  Hematology and oncology

Cutaneous T cell lymphoma (CTCL) is a class of non-Hodgkin lymphoma, which is a type of cancer of the immune system. Unlike most non-Hodgkin lymphomas (which are generally B cell related), CTCL is caused by a mutation of T cells. The malignant T cells in the body initially migrate to the skin, causing various lesions to appear. These lesions change shape as the disease progresses, typically beginning as what appears to be a rash which can be very itchy and eventually forming plaques and tumors before metastasizing to other parts of the body.

Contents

Classification

Cutaneous T-cell lymphomas may be divided into the following types:

  • Mycosis fungoides
  • Pagetoid reticulosis
  • Sézary syndrome
  • Granulomatous slack skin
  • Lymphomatoid papulosis
  • Pityriasis lichenoides chronica
  • Pityriasis lichenoides et varioliformis acuta
  • CD30+ cutaneous T-cell lymphoma
  • Secondary cutaneous CD30+ large cell lymphoma
  • Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
  • Pleomorphic T-cell lymphoma
  • Lennert lymphoma
  • Subcutaneous T-cell lymphoma
  • Angiocentric lymphoma
  • Blastic NK-cell lymphoma
  • A WHO-EORTC classification has been developed.

    Treatment

    There is no cure for CTCL, but there are a variety of treatment options available and some CTCL patients are able to live normal lives with this cancer, although symptoms can be debilitating and painful, even in earlier stages.

    FDA approved treatments are:

  • (1999) Denileukin diftitox (Ontak)
  • (2000) Bexarotene (Targretin) a retinoid
  • (2006) Vorinostat (Zolinza) a hydroxymate histone deacetylase (HDAC) inhibitor
  • (2009) Romidepsin (Istodax) a cyclic peptide histone deacetylase (HDAC) inhibitor
  • Other (off label) treatments include:

  • Topical and oral corticosteroids
  • Bexarotene (Targretin) gel and capsules
  • Carmustine (BCNU, a nitrosourea)
  • Mechlorethamine (Nitrogen Mustard)
  • Phototherapy (Broad & Narrow Band UVB or PUVA)
  • Local and Total Skin Electron Therapy (TSET)
  • Conventional Radiation Therapy
  • Photopheresis
  • Interferons
  • Alemtuzumab (Campath-1H)
  • Methotrexate
  • Pentostatin and other purine analogues (Fludarabine, 2-deoxychloroadenosine)
  • Liposomal doxorubicin (Doxil)
  • Gemcitabine (Gemzar)
  • Cyclophosphamide
  • Bone marrow / stem cells
  • Allogenic transplantation
  • Forodesine (Inhibits Purine Nucleoside phosphorylase)
  • Other drugs are under investigation (for example: panobinostat, Resimmune, resiquimod and synthetic hypericin).

    In 2010, the U.S. Food and Drug Administration granted orphan drug designation for a topical treatment for pruritus in cutaneous T-cell lymphoma to a pharmaceutical company called Elorac.

    Epidemiology

    Of all cancers involving the same class of blood cell, 2% of cases are cutaneous T cell lymphomas.

    There is some evidence of a relationship with human T-lymphotropic virus.

    References

    Cutaneous T cell lymphoma Wikipedia


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