Cutaneous+T-Cell+Lymphoma

Peak incidence between 7th and 8th generation Unlike other forms of non-Hodgkin lymphoma, CTCL mainly affects the skin.1 Men are twice as likely as women to have the disease1 Twice as common in African Americans than Caucasians 1 || Industrial exposures Genetic factors || Early patched lesions often mistaken for other dermis conditions1 Plaque and tumor phase associated with palpable lesions1 17% of patients with CTCL present with generalized erythroderma1 50% of these have clear-cut Sezary syndrome Average time from onset of lesions to diagnosis is approximately 8-10 years1 || Lab work: CBC, Sezary cell count Biopsy Chest x-ray CT of chest, neck, abdomen, pelvis Integrated whole body CT/PET.2 ||
 * **Epidemiolgy:** || Incidence increases sharply with age1
 * **Etiology:** || Etiology is unknown, there is a link with:1
 * **Signs & Symptoms:** || Lesions most often present in “sun-shaded” areas1
 * **Diagnostic Procedures:** || Full physical examination with evaluation of lymph node bearing areas, liver and spleen
 * **Histology:** || There are two subgroups of Cutaneous T-cell lymphoma; mycosis fungoides (MF) and Sezary syndrome. Both types are malignant T-cell lymphoproliferative disorders that invade the epidermis and follicular epithelium of the skin. " The cellular infiltrate of CTCL consists of malignant T cells mixed with various numbers of normal white blood cells." These malignant T cells are often found circulating in the lymph nodes.2 ||
 * **Lymph node drainage:** || The status of the lymph nodes in the cervical, axillary, and inguinal regions should be evaluated. If a lymph node is palpable, it should be biopsied. A strong effort should be made to confirm the presence of extracutaneous involvement, if suspected. 2 ||
 * **Metastatic spread:** || Extracutaneous disease involving sites beyond the blood and peripheral lymph nodes occurs in advanced cutaneous T-cell lymphoma. 3  Metastasis can occur in virtually any part of the body, but the most common visceral sites of involvement include the lungs, the oral cavity and pharynx, and the central nervous system. 4 ||
 * **Grading:** || <span style="color: #800080; font-family: Arial,Helvetica,sans-serif; font-size: 12px;">CTCL is classified as an indolent, low grade type of Non-Hodgkin’s Lymphoma. ||
 * **Staging:** || **<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">Staging of Cutaneous T-Cell Lymphoma: Tumor-Node-Metastasis Classification ** 3

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">Classification and Description/Definition

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">T: Skin <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">T0 - Lesions clinically and/or pathologically suggestive of CTCL <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">T1 - Limited plaques, papules, or eczematous patches covering <10% of skin surface <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">T2 - Generalized plaques, papules, or erythematous patches covering ≥10% of skin surface <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">T3 - Tumors <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">T4 - Generalized erythroderma

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">N: Lymph nodes

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">N0 - No palpable adenopathy, lymph node pathology negative for CTCL <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">N1 - Palpable lymphadenopathy; lymph node pathology negative for CTCL <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">N2 - No palpable lymphadenopathy, lymph node pathology positive for CTCL <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">N3 - Palpable adenopathy, lymph node pathology positive for CTCL

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">B: Peripheral blood

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">B0 - Atypical circulating cells not present (<5%) <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">B1 - Atypical circulating cells present (>5%)

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">M: Visceral organs

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">M0 - No visceral organ involvement <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">M1 - Visceral involvement (must have pathologic confirmation, and organ involved should be specified)


 * <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">Modified Tumor-Node-Metastasis Staging System for Classification of Cutaneous T-Cell Lymphoma **

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">Stage T:N:M

<span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">IA - 1:0:0 <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">IB - 2:0:0 <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">IIA - 1,2:1:0 <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">IIB - 3:0,1:0 <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">III - 4:0,1:0 <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">IVA - 1–4:2,3:0 <span style="color: #800080; font-family: Arial,Helvetica,sans-serif;">IVB - 1–4:0–3:1 || Long-Term1
 * **Radiation side effects:** || Short -Term1
 * Usually develops mild erythema, dry desquamation, and hyperpigmentation.
 * Swelling of the hands, edema of the ankles, and occasionally large blisters
 * Gynecomastia may also develop
 * Chronic cutaneous
 * Superficial atrophy with wrinkling, telangiectasias, xerosis, and uneven pigmentation.
 * Frank poikiloderma, permanent alopecia, skin fragility, and subcutaneous fibrosis. ||
 * **Prognosis:** || Cutaneous T-cell lymphoma may remain confined to the skin for many years, but the abnormal cells may eventually infiltrate other tissues including blood, lymph nodes, lungs, heart, liver and spleen. Unlike some other lymphomas, the outlook is generally good. Symptoms can usually be controlled with treatment. However treatment is not curative. 5 ||
 * **Treatments:** || * In the United States, the most common radiotherapeutic approach for extensive CTCL is TSEB irradiation.
 * The radiation generally is administered on a 4-day per week schedule, with the total dose depending on curative or palliative intent
 * Doses of 30 to 40 Gy are delivered over an 8- to 10-week interval, with a 1- to 2-week break at 18 to 20 Gy for patients treated with a curative intent; 10 to 20 Gy is administered for palliation.
 * Shielding of the digits and lateral surfaces of the hands or feet may be necessary becaise of overlapping treatment fields in these areas.
 * Shielding of uninvolved skin is recommended in palliative treatment ||
 * **TD 5/5:** || || Ear- Middle || 5000 cGy ||  ||
 * Ear- Vestibular || 6000 cGy ||  ||
 * Large arteries and veins || >8000 cGy || 10cm2 ||
 * Large nodes and lymphatics || 5000 cGy ||  ||
 * Oral cavity || 6000 cGy ||  ||
 * Peripheral nerves || 6000 cGy || 10cm ||
 * Skin || 5500 cGy || 100cm2 ||
 * Eye-Retina || 5500 cGy ||  ||
 * Eye-Cornea || 5000 cGy ||  ||
 * Eye-Lens || 500 cGy ||  ||

Organs affected depend on location of tumor and depth of treatment6
 * **References:** || # <span style="color: #0000ff; font-family: Arial,Helvetica,sans-serif;">Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 3rd edition. Philadelphia, PA: Lippincott Williams & Wilkins. 2011; 135-136.
 * 1) <span style="color: #ff0000; font-family: Arial,Helvetica,sans-serif;">Chao KS, Perez CA, Brady LW. //Radiation Oncology Management Decisions//. 2nd edition. Philadelphia, PA: Lippincott Williams & Wilkins. 2011; 123-128
 * 2) <span style="color: purple; font-family: 'Arial','sans-serif'; font-size: 13px;">Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG. //Abeloff's Clinical Oncology//. 4th ed. Philadelphia, PA: Churchill Livingstone; 2008.
 * 3) <span style="color: purple; font-family: 'Arial','sans-serif'; font-size: 13px;">Hoppe RT, Phillips TL, Mack III M. //Leibel and Phillips Textbook of Radiation Oncology//. 3rd ed. Philadelphia Pa: Elsevier Saunders; 2010
 * 4) DermNet NZ:Cutaneous T-cell lymphoma. availible at [] accsesed 7-2-2012
 * 5) Washington CM. Principles and Practice of Radiation Therapy. 3rd ed. St. Louis, Missouri; Mosby Inc ||
 * 1) Washington CM. Principles and Practice of Radiation Therapy. 3rd ed. St. Louis, Missouri; Mosby Inc ||

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