Cutaneous T-Cell Lymphoma: Overview and Nursing Perspectives

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Cutaneous T-cell lymphoma (CTCL) is an uncommon and complex malignancy of the immune system with a wide range of clinical presentations primarily involving the skin. An extensive menu of skin-directed and/or systemic treatment options exists. Best practices in management involve multidisciplinary collaboration. Nursing care for patients who have CTCL is a critical component in the successful management of the disease and requires special attention to the patient's physical, emotional, and spiritual needs. Nurses can make a significant impact by being accessible, offering emotional support, demonstrating advocacy, and providing ongoing education for the patient and family.

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Epidemiology

MF and SzS are the most common of the CTCLs with MF being the most prevalent. It is difficult to determine the actual prevalence of the disorder, since it is often indolent and chronic, lasting in many cases for decades. CTCL is most common in the later decades of life, beginning on average at the 5th or 6th decade, but any age group can be affected. The most recent U.S. study on incidence revealed a 2.9 × 10−6 increase per decade since 1973 with a current estimated incidence of 6.4 per million

Etiology

The cause of CTCL remains unknown despite epidemiologic attempts to establish an infectious, environmental, or genetic link [5]. Some cases of multiple family members developing CTCL have been reported; however, there are no established genetic links [6]. Its pathology reflects a cancer of the immune system whereby the skin-homing T cells, preprogrammed to protect the skin, expand clonally, function in an activated state, and achieve clonal dominance [2]. This neoplastic, clonal expansion,

Prognosis

Prognosis is good for patients who present with early-stage CTCL, with longevity approaching that of age-matched control groups and with relatively few reported disease-related deaths. In the advanced stages (IIB–IVB), however, the prognosis is less favorable. Disease-related deaths usually involve the overt failure of the immune system, leading to sepsis or other opportunistic infections [7].

Skin manifestations and symptoms

In the less common variants of MF, unique lesions are observed. Granulomatous slack skin presents as infiltrating lesions, primarily of the axillary/inguinal folds. Over time, the skin in these areas becomes atrophic and loses elasticity, and excessive skin folds are noted. Folliculotropic MF infiltrates hair follicles and produces localized alopecia. Pagetoid reticulosis is characterized by a localized keratotic or psoriasiform plaque that develops slowly and usually is found on a lower

Diagnosis

The diagnosis of CTCL may be delayed by the difficulty in obtaining a diagnostic skin biopsy. It also is delayed when patients ignore asymptomatic, slow-growing, or changing lesions for months to years. It is common for patients to report the existence of the disease for some time before medical attention is sought or before a primary care physician refers the patient to a specialist. Often, multiple biopsies done over time (months to years) are required so that a histopathologic diagnosis can

Treatment

A multidisciplinary approach often is required for patients who have more extensive skin involvement, generally greater than stage IA. For these patients, an interdisciplinary plan of care involving the dermatologist, oncologist, and radiation oncologist often improves outcomes and establishes a network of providers for multiple therapeutic pathways [16].

There are many therapeutic options for CTCL. This variety of options generally means there is no one or two therapies that work for most

Nursing role

Nursing care is a critical element to the successful care of patients who have CTCL and involves administering hands-on care, acting as the patient's advocate, and educating the patient and family members on a multitude of topics. The nurse is instrumental as a liaison with the physician, other health care providers (oncologist, radiation oncologist, dietician, and pharmacist), social services, community agencies, and family members. The author has found that patients are comfortable

Support and patient resources

The CLF was established in 1998 and formerly was known as the “Mycosis Fungoides Foundation.” The CLF is dedicated to supporting people diagnosed as having a cutaneous lymphoma by promoting awareness and education, advancing patient care, and facilitating research. Among its many programs, the CLF partners with industry sponsors and medical centers to promote educational forums around the country through Web casts and day-long symposiums. It also provides two on-line support groups, one geared

Summary

CTCL is an uncommon and complex malignancy of the immune system with a wide range of clinical presentations primarily involving the skin. Involvement of lymph nodes and distant organs is also possible. The disease is chronic by nature and as such affords the dermatologist and dermatology nurse an opportunity to provide care for the patient on a long-term basis. An extensive menu of skin-directed and/or systemic treatment options exists. Best practices in management involve multidisciplinary

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