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Mycosis Fungoides

Mycosis Fungoides (MF) Is The Commonest Cutaneous T Cell Lymphoma. Three Clinical Stages Can Be Identified: Patch, Plaque  , Tumour Stage. The Erythrodermic Stage, Which Is Also Called Sezary Syndrome (Erythroderma, Enlarged Lymph Nodes, And Circulating Sezary Cells) May Develop De-Novo Or From The Earlier Stages.

  1. Histology: One Should Bear In Mind That The Histological Features Is Variable And There Is Some Correlation With The Clinical Features. 4 Types Have Described:
    1. Epidermotrophic Patches And Plaques
    2. Epidermatrophic Erythroderma
    3. Non-Epidermotrophic Erythroderma
    4. Non-Epidermotrophic Tumours
      The Biopsy Specimens May Also Send For:
  2. Immunophenotyping: To Demonstrate T Cell Lineage. Other Markers E.G. IL2, Ki1 Are Helpful In Certain Circumstances.
  3. B. T Cell Recepter Gene Rearrangement Study: By PCR, To Demonstrate Monoclonality.
    1. Treatment
      1. Choice of Treatment Should be Determined By:
      2. Clinical Stages.
      3. Availabilities of Facilities and Expertise.
      4. Patient's Preference.
        1. For all stages of diseases, symptomatic relief with emollient, topical steroid, anti-
        2. Histamine are indispensable.
        3. Choices of treatment according to stages:
        4. Patches and plaques
        5. Puva, repuva is the first line.
        6. Topical nitrogen mustard is an alternative if available.
        7. Total skin electron beam therapy or puva with a interferon are reserved for refractory cases.
    2. Tumours
      Total skin electron beam with local booster radiation to tumours. Extracorporeal photophoresis is an alternative (Not Available Locally).
    3. Erythrodermic Stage And Sezary Syndrome
      1. Photophoresis, if available, is probably the best treatment, and concomitant methotrexate may increase its effectiveness.
      2. Puva may give symptomatic relief but should be administered with care.
      3. Other choice of treatment may include a interferon, systemic chemotherapy, retinoids and bone marrow transplant.
      4. Histological lymph node involvement
      5. Treatment is essential palliative: which may include local radiation to local symtomatic disease, systemic chemotherapy, a interferon, retinoids.
      6. Visceral involvement
      7. Palliative treatment include systemic chemotherapy, interferons, retinoids etc.
    4. Puva: Primarily indicated in patch and plaque skin diseases, this is not effective in tumor diseases and should be used with care in erythrodermic patients. The administration is essentially similar to puva in psoriasis. Sanctuary site lesions will need addition dose or adding of other agents like topical steroid, nitrogen mustard.
    5. Repuva: Adding retinoids (etretinate, isotretinoin) to puva gives earlier response and longer period of remission, although the relapse rate and long term survival is the same as puva alone. It should considered in patients with poor initial response to puva.
Total Skin Electron Beam Therapy: The delivery of high energy electron to a limited depth of skin and preventing systemic toxicity. While it is effective in patch and plaque diseases, its relative poor cutaneous tolerance makes it the treatment reserved to tumor stage or refractory extensive plaque stages. It is relatively contraindicated in erythrodermic disease because of severe side effects.

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