eMedicine Specialties > Dermatology > Lymphoma and Related Processes

Cutaneous T-Cell Lymphoma: Follow-up

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): W Clark Lambert, MD, PhD, Professor and Head, Dermatopathology, Departments of Pathology and Dermatology, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: Nov 10, 2009

Follow-up

Prognosis

  • The prognosis for patients with cutaneous T-cell lymphomas (CTCLs) or mycosis fungoides (MF) is dependent on stage and, in particular, the type and extent of skin lesions and the presence of extracutaneous disease.2
    • Patients with limited patch/plaque-stage MF seem to have similar life expectancies to age-, sex-, and race-matched control populations. Ten-year disease-specific survival rates were 97-98% for patients with limited patch/plaque disease (covering <10% of the skin surface), 83% for patients with generalized patch/plaque disease (covering >10% of the skin surface), 42% for patients with tumor-stage disease, and approximately 20% for patients with histologically documented lymph node involvement.
    • Patients with effaced lymph nodes, visceral involvement, and transformation to large T-cell lymphoma have an aggressive clinical course and usually die of systemic involvement or infections.
    • Blood eosinophilia at baseline may also serve as a prognostic factor in patients with primary CTCL.64
  • The prognosis associated with FMF is worse than that for classic patch- and plaque-stage MF and corresponds more closely with tumor-stage disease (stage IIB). For this reason, some authors have proposed that FMF be staged with tumor-stage disease (stage IIB). FMF has shown a disease-specific 5-year survival rate of approximately 70-80%.65
  • With SS, the prognosis is often poor, with a median survival of 2-4 years.66 The disease-specific 5-year survival rate has been reported to be 24%, with most patients dying of opportunistic infections due to immunosuppression.
  • In patients with ATLL, the clinical subtype is the main prognostic factor. Survival in persons with acute or lymphomatous variants is poor, ranging from 2 weeks to more than 1 year. Chronic and smoldering forms have a more protracted clinical course and a longer survival, but transformation into an acute phase with an aggressive course may occur.8,67
  • SPTL (with a CD8+, alpha/beta+ T-cell phenotype) tends to have a protracted clinical course with recurrent subcutaneous nodules but without extracutaneous dissemination or the development of a hemophagocytic syndrome.11 The 5-year survival rate of such patients may be greater than 80%.
  • Unilesional pagetoid reticulosis (Woringer-Kolopp disease) progresses slowly. Patients usually have an excellent prognosis.
  • Primary cutaneous PTL, type unspecified, is associated with a poor prognosis, with 5-year survival rates of less than 20%,68  regardless of whether it manifested with solitary/localized lesions or generalized skin lesions.17
  • Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma (provisional entity) is associated with a relatively favorable prognosis, with an estimated 5-year survival rate of approximately 60-80%.17 Patients first seen with solitary or localized skin lesions tend to have an excellent prognosis.
  • Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma often has an aggressive clinical course, and patients have a median survival of 32 months.5
  • CGD-TCL patients tend to have aggressive disease resistant to multiagent chemotherapy and/or radiation, with a median survival in 1 study of only 15 months.23 A trend for decreased survival was noted for patients with subcutaneous fat involvement in comparison with patients who had epidermal or dermal disease only.
  • Nasal type NK/T-cell lymphoma manifesting in the skin is highly aggressive, and patients have a median survival of less than 12 months.45,69 The most important factor predicting a poor outcome is the initial presence of extracutaneous involvement. Patients first seen with only cutaneous involvement had a median survival of 27 months, compared with months for patients presenting with cutaneous and extracutaneous disease.69 CD30+, CD56+ patients seem to have a better prognosis, possibly being examples of cutaneous anaplastic large cell lymphoma with coexpression of CD56.70
  • Granulomatous CTCLs tend to be therapy resistant, with a slowly progressive course apparently worse than that of classic nongranulomatous MF.6

Miscellaneous

Medicolegal Pitfalls

  • The principal concern is following a patient with the patch stage of MF for years without recognizing its transition into a lymphoma.
 


More on Cutaneous T-Cell Lymphoma

Overview: Cutaneous T-Cell Lymphoma
Differential Diagnoses & Workup: Cutaneous T-Cell Lymphoma
Treatment & Medication: Cutaneous T-Cell Lymphoma
Follow-up: Cutaneous T-Cell Lymphoma
Multimedia: Cutaneous T-Cell Lymphoma
References
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References

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Further Reading

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Keywords

CTCL, mycosis fungoides, patch stage mycosis fungoides, large plaque parapsoriasis, plaque stage mycosis fungoides, tumor stage mycosis fungoides, d'emblee mycosis fungoides, primary cutaneous pleomorphic mycosis fungoides, medium-sized T-cell lymphoma, large cell CD30+ T-cell lymphoma, large cell CD30- T-cell lymphomas, solitary mycosis fungoides, unilesional mycosis fungoides, unilesional pagetoid reticulosis, Woringer-Kolopp disease, follicular mycosis fungoides, Sézary syndrome, Sezary syndrome, Sezary's syndrome, erythrodermic mycosis fungoides, granulomatous slack skin, granulomatous CTCL, granulomatous mycosis fungoides, angiocentric CTCL, subcutaneous CTCL, panniculitis-like CTCL, panniculitislike CTCL, lipotropic CTCL, panniculotropic CTCL, subcutaneous mycosis fungoides, suppressor T-cell CTCL, CD8+ CTCL, delta/gamma CTCL, NK cell CTCL, natural killer CTCL, KI-1+ large cell CTCL

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

W Clark Lambert, MD, PhD, Professor and Head, Dermatopathology, Departments of Pathology and Dermatology, UMDNJ-New Jersey Medical School
W Clark Lambert, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Dermatological Association, American Society of Dermatopathology, International Academy of Pathology, Medical Society of New Jersey, Sigma Xi, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Günter Burg, MD, Professor and Chairman Emeritus, Department of Dermatology, University of Zürich School of Medicine; Delegate of The Foundation for Modern Teaching and Learning in Medicine Faculty of Medicine, University of Zürich, Switzerland
Günter Burg, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, International Society for Dermatologic Surgery, North American Clinical Dermatologic Society, and Pacific Dermatologic Association
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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