Cutaneous+T-Cell+Lymphoma

Cutaneous T-cell lymphoma (CTCL) is a type of cancer that affects the white blood cells in the body. Their classification is based on lymphocyte type; B-lymphocytes or T-lymphocytes. The incidence of cutaneous T-cell lymphoma increases as we age and it is most commonly manifested in men than women. It also affects the black population more so than whites and hispanics. CTCL affects approximately 30,000 individuals every year in North America. Other factors that will influence the diagnosis of CTCL are exposure to certain chemicals, genetic factors, and a family history. 1 || Etiology of Cutaneous T-cell lymphoma (CTCL) is unknown; however a couple of risk factors such as industrial exposure and genetic factors are thought to be contributing factors. 2 || Typically, the disease will progress slowly through three different stages: the patch/premycotic phase, the infiltrated plaque/mycotic phase, and the tumor/fungoid phase. In the patch phase, lesions are frequently mistaken for other dermatoses. Upon reaching the second phase, the lesions become clinically palpable, often forming irregular shapes. Upon reaching the third stage, cutaneous ulcerations and secondary infections become frequent. 3 || The diagnostic procedures for Cutaneous T-cell Lymphoma can be performed at many locations at the prescription of many medical professionals. A full physical examination addressing any areas of lymph node bearing, predominantly the liver and spleen. Lab work should include CBC and Sezary cell count. A biopsy can provide more detail on areas of concern. A chest x-ray, CT of chest, neck, abdomen, pelvis, or an Integrated whole body CT/PET are all also used for diagnostic purposes in Cutaneous T-cell Lymphoma. 4 ||
 * **Epidemiolgy:** || Pablo
 * **Etiology:** || Becky
 * **Signs & Symptoms:** || Adam
 * **Diagnostic Procedures:** || Megan
 * **Histology:** || Kevin

Cutaneous T-cell lymphoma has two major subgroups: mycosis fungoides (MF) and Sezary syndrome. They usually display CD4 positivity and show a propensity to infiltrate the epidermis (epidermotropism). 5 || Cutaneous T-cell lymphoma is caused with T-lymphocytes become malignant and affect the skin. 6 Generally, only stage IV spreads to areas other than the skin. Not much information is available on the lymph node drainage system, however, when cutaneous T-cell lymphoma does spread somewhere other than the skin, it is spread to the liver, spleen, or bone marrow via the lymph system. || Although every organ system is at risk for cutaneous t-cell lymphoma, there are some specific areas that it could possibly relapse in or spread to. 7 The following are the stages of cutaneous T-cell lymphoma (CTCL). Because the disease has a may have a slow progression, it is somewhat difficult to diagnose. Stage 1 There are red patches and/or raised red patches (plaques) on the skin. This stage is sometimes divided into: Stage 2A Skin symptoms are the same as in stage 1. Some lymph nodes are enlarged, but the lymphoma cells have not spread there. Stage 2B There may be one or more tumours on the skin. Stage 3 More than 80% of the skin is red (erythroderma). Stage 4A There may be any of the skin symptoms described in the previous stages. There are lymphoma cells in the lymph nodes or high numbers of lymphoma cells in the blood, or both. Stage 4B There may be any of the skin symptoms described in the previous stages. Lymphoma cells may or may not have spread to the lymph nodes and/or blood. The lymphoma has spread to other organs in the body such as the liver. 9 || 2 || There are both short term and long term side effects to radiation treatments: 3 Most patients with Cutaneous T-Cell Lymphoma (CTCL) mainly expereince skin symptoms, without serious complications. But, about 10 percent of those who progress to later stages develop serious complications. Early stage CTCL is typically indolent; some patients with early-stage CTCL might not progress to later stages at all, while others might progress rapidly, with the cancer spreading to lymph nodes and/or internal organs. The two main types of CTCL are Mycosis Fungoides and Sezary Syndrome. 10 ||
 * **Lymph node drainage:** || Erin
 * **Metastatic spread:** || Spencer
 * Relapse most commonly into the lymph system. 8
 * Most often spreads into the internal organs such as the liver, spleen, and possibly bone marrow. 8 ||
 * **Grading:** || Pablo
 * Stage 1A - less than 10% of the skin is affected.
 * Stage 1B - 10% or more of the skin surface is affected.
 * **Staging:** || Becky
 * **Radiation side effects:** || Adam
 * Short term
 * Mild erythema
 * Dry desquamation
 * Hyperpigmentation
 * Long term
 * Chronic cutaneous damage
 * Superficial atrophy such as wrinkling, telangiectasias, xerosis, and uneven pigmentation
 * Permanent alopecia, skin fragility, and subcutaneous fibrosis at higher doses (rare) ||
 * **Prognosis:** || Megan
 * **Treatments:** || Kevin

Since total-skin electron irradiation is commonly used to treat cutaneous T-cell lymphoma, there are only a few TD 5/5's that we need to be aware of: 11 Eye lens - 1000cGy (cataracts) Retina - 4500cGy (blindness) || 1. Cutaneous lymphoma foundation. Web site. []. July 1, 2013. 2. Chao KSC, Perez CA, Brady LW. Cutaneous t-cell lymphoma. In: Chao KSC, Perez CA, Brady LW, eds. //Radiation Oncology Management Decisions//. Philadelphia, PA: Lippincott, Williams and Wilkins; 2011: 135-143. 3. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2011. 4. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2002. 123-128. 5. Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 3rd edition. Philadelphia, PA: Lippincott Williams & Wilkins. 2011; 135-142. 6. John Hopkins Medicine website. Cutaneous T-Cell Lymphoma.  http://www.hopkinsmedicine.org/healthlibrary/conditions/skin_cancer/cutaneous_t-cell_lymphoma_85,P01340 /. Accessed July 5, 2013. 7. Lenards, N. Cutaneous T-Cell Lymphoma. [PowerPoint]. La Crosse, WI: UW-L Medical Dosimetry Program; 2013. 8. Lymphoma of the skin. American Cancer Society Web Site. [] . Accessed June 26, 2013. 9. MacMillan Cancer Support. Web site. []. Accessed July 2, 2013. 10. Cutaneous T-Cell Lymphoma (CTCL). Lymphoma Research Foundation web site. []. Accessed July 1, 2013. 11. Leukemia & Lymphoma Society. Cutaneous T-Cell Lymphoma Facts. []. Accessed July 4, 2013. || Back to Week 6
 * Stage IA - possibility of cure with intensive therapy directed at the skin alone using either total-skin electron beam (TSEB) irradiation, topical mechlorethamine chemotherapy, or photonchemotherapy with methoxsalen. 5
 * Stage IIB – TSEB therapy, denileukin diftitox, and oral bexarotene.
 * Stage III or Sezary’s syndrome – extracorporeal photopheresis with the addition of biologic response modifiers as needed
 * Widespread intracutaneous disease in the presence of cutaneous tumors – TSEB irradiation with concomitant multiagent systemic chemotherapy, or total lymph node irradiation if the treatment intent is curative ||
 * **TD 5/5:** || Erin
 * **References:** || Spencer