Cutaneous T-Cell Lymphoma 

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 9, 2011
 

Background

Cutaneous T-cell lymphomas (CTCLs) are the largest group of cutaneous lymphomas, representing 65% of all cutaneous lymphomas. The World Health Organization (WHO)/European Organization for Research and Treatment of Cancer (EORTC) classification (WHO-EORTC classification) is used to categorize CTCLs.[1, 2, 3] However, a substantial subset of T-cell primary cutaneous lymphomas remains that cannot be classified beyond the unspecified peripheral T-cell category, some of which may have an aggressive course.[4]

Note the clinical images below.

Tumor-stage cutaneous T-cell lymphoma. Tumor-stage cutaneous T-cell lymphoma. Middle-aged woman with mycosis fungoides showing uMiddle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.

Mature T-cell and natural killer (NK) cell neoplasms according to the WHO-EORTC classification are as follows:

  • Mycosis fungoides (MF): - Variants of MF (pagetoid reticulosis [localized disease], follicular, syringotropic, granulomatous variant), subtype of MF (ie, granulomatous slack skin (GSS) syndrome)
  • Sézary syndrome
  • CD30+ T-cell lymphoproliferative disorders of the skin - Lymphomatoid papulosis, primary cutaneous anaplastic large cell lymphoma
  • Subcutaneous panniculitislike T-cell lymphoma
  • Primary cutaneous peripheral T-cell lymphoma (PTL), unspecified - Subtypes of PTL (primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma [provisional], cutaneous gamma/delta-positive T-cell lymphoma (CGD-TCL) [provisional], primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma [provisional])
  • Extranodal NK/T-cell lymphoma, nasal type - Variant (hydroa vacciniformialike lymphoma)
  • Adult T-cell leukemia/lymphoma
  • Angioimmunoblastic T-cell lymphoma

CTCL, most commonly seen as MF, is a relatively common clonal expansion of T helper cells and, more rarely, T suppressor/killer cells or NK cells, that usually appears as a widespread, chronic, cutaneous eruption. MF itself is often an epidermotropic disorder characterized by evolution of patches into plaques and tumors composed of small- to medium-sized skin-homing T cells, some (or, rarely, all) of which have convoluted, cerebriform nuclei.

Pagetoid reticulosis is a variant of MF and is characterized by the presence of localized patches or plaques with an intraepidermal proliferation of neoplastic T cells. The term pagetoid reticulosis should be restricted to the localized type (Woringer-Kolopp type) and should not be used to describe the disseminated type (Ketron-Goodman type). Generalized cases should probably be classified as aggressive epidermotropic CD8+ CTCL, CGD-TCL, or tumor-stage MF.[5]

GSS syndrome is a rare subtype of CTCL characterized by the slow development of folds of lax skin in the major skin folds and, histologically, by an infiltrate of clonal T cells, with exceptionally large multinucleated giant cells sometimes showing inclusion of fragmented elastic fibers. Granulomatous CTCLs are rare, so limited data on the clinicopathological and prognostic features are available.[6] Patients with granulomatous MF and GSS display overlapping histologic features and differ clinically by the development of bulky skin folds in GSS.

Sézary syndrome (SS) has been defined historically by the triad of erythroderma, generalized lymphadenopathy, and the presence of neoplastic T cells (Sézary cells) in skin, lymph nodes, and peripheral blood. Some authorities believe the diagnosis of SS should include one or more of the following[7] :

  • An absolute Sézary cell count of least 1000/µL
  • Demonstration of immunophenotypical abnormalities (an expanded CD4+ T-cell population resulting in a CD4/CD8 ratio of more than 10:1; loss of any or all of the T-cell antigens CD2, CD3, CD4, CD5; or loss of both CD4 and CD5)
  • Demonstration of a T-cell clone in the peripheral blood by molecular or cytogenetic methods

SS is an erythrodermic CTCL. Demonstration of a T-cell clone (preferably of the same T-cell clone in skin and peripheral blood) in combination with one of the aforementioned cytomorphological or immunophenotypical criteria has been suggested as minimal criteria for the diagnosis of SS, in order to exclude patients with benign inflammatory conditions simulating SS.

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive T-cell neoplasm etiologically linked with the human T-cell leukemia virus 1 (HTLV-1). Cutaneous lesions are usually a manifestation of widely disseminated disease, although a slowly progressive form that may only involve the skin may occur.[8]

Subcutaneous panniculitislike T-cell lymphoma (SPTL) is a cytotoxic T-cell lymphoma characterized by the presence of primarily subcutaneous infiltrates of small, medium, or large pleomorphic T cells and many macrophages, predominantly affecting the legs and often complicated by a hemophagocytic syndrome.[9] At least 2 groups of SPTL with different histologies, phenotypes, and prognoses can be distinguished. Cases with an alpha/beta-positive T-cell phenotype are usually CD8+, are restricted to the subcutaneous tissue (with no dermal or epidermal involvement), and tend to run an indolent clinical course.[10, 11] The SPTL designation is only used for patients with an alpha/beta-positive T-cell phenotype, whereas those with a gamma/delta T-cell phenotype are now categorized as having CGD-TCL.[1, 2, 3, 11, 12]

Extranodal NK/T-cell lymphoma, nasal type, is an Epstein-Barr virus (EBV)–positive lymphoma of small, medium, or large cells, usually with an NK-cell, or, more rarely, a cytotoxic T-cell phenotype. The skin is the second most common site of involvement, with the nasal cavity/nasopharynx being the most common and the reason why it was once known as a lethal midline granuloma. Cutaneous involvement may be primary or secondary. Because both primary and secondary involvement are clinically aggressive and require the same type of treatment, distinction between the 2 cutaneous involvements seems unnecessary.[11, 13, 14]

Primary cutaneous PTL, type unspecified, is the designation for CTCLs that do not fit into any of the better-defined subtypes of CTCL.[1, 2, 3] These include the 3 provisional entities described below, because primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, CGD-TCL, and primary cutaneous small-medium CD4+ T-cell lymphoma can be separated out as provisional entities. The remaining diseases that do not fit into any of the better-defined subtypes of CTCL are characterized as follows:

  • Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, a provisional entity, is characterized by a proliferation of epidermotropic CD8+ cytotoxic T cells and aggressive clinical behavior.[5]
  • CGD-TCL is composed of a clonal proliferation of mature, activated gamma/delta T cells with a cytotoxic phenotype.[12, 15, 16] It may be primary or secondary cutaneous lymphoma.
  • Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma is the diagnosis used when a predominance of small- to medium-sized CD4+ pleomorphic T cells are present without a history of patches and plaques typical of MF.[17]

For additional information from other eMedicine articles, see T-Cell Disorders, Mycosis Fungoides, and Lymphoma, Cutaneous T-Cell.

Next

Pathophysiology

The primary pathophysiologic mechanisms for the development of cutaneous T-cell lymphoma (CTCL) (ie, mycosis fungoides [MF]) have not been elucidated. MF may be preceded by a T-cell–mediated chronic inflammatory skin disease, which may occasionally progress to a fatal lymphoma. Karyotypic analysis of cutaneous and blood lymphocytes has shown several genetically aberrant T-cell clones in the same patient.

A genotraumatic T cell is one with a tendency to develop numerous clonal chromosomal aberrations. Normal T lymphocytes show apoptosis during in vitro culturing, whereas genotraumatic ones have the ability to develop clonal chromosomal aberrations to become immortalized. This concept implies genetic instability followed by T-cell proliferation. Successive cell divisions of a genotraumatic T-cell clone may produce multiple and complex chromosomal aberrations. Some may reprogram the genotraumatic cells to apoptosis, whereas one or more may produce the phenotypic alterations of malignancy if not eliminated in vivo.

Thus, one hypothesis is that the development of genotraumatic T lymphocytes is involved in the etiopathogenesis and the progression of MF. It would also predict that each patient would likely have a unique malignant clone, which, in fact, has been found to be the case.

CTCL is a group of T-cell proliferative disorders. Primary cutaneous lymphomas require distinction from histologically similar primary nodal ones because their clinical behavior, prognosis, and therapy are often different. In addition, a difference often exists between primary cutaneous and nodal lymphomas in the presence of specific translocations.

Previous
Next

Epidemiology

Frequency

United States

The incidence of cutaneous T-cell lymphoma (CTCL) is approximately 5 cases per million population per year.

International

Cutaneous T-cell lymphomas (CTCLs) account for 65% of cutaneous lymphomas. CTCLs have a worldwide distribution, with no well-identified increase in prevalence in any region. Some studies have identified an increase in prevalence in industrial populations (eg, among workers who use machine cutting oils). ATLL is endemic in areas with a high prevalence of HTLV-1 infection, such as southwest Japan, the Caribbean islands, South America, and parts of Central Africa. ATLL occurs in 1-5% of seropositive individuals after more than 2 decades of viral persistence.[8] Nasal NK/T-cell lymphoma, which is associated with EBV infection, is more common in Asia, Central America, and South America.

In the dermatology center of Professor Alsaleh in Kuwait, Arab males with MF outnumbered females by a 2:1 ratio.[18] The annual incidence rate in Kuwait was 0.43 case per 100,000 persons; this was found to be significantly higher among Arabs compared with non-Arab Asians.

Race

In the United States, cutaneous T-cell lymphoma (CTCL) is more common among Americans of sub-Saharan African lineage than those of European background, in a ratio of approximately 2:1.

Sex

Cutaneous T-cell lymphoma (CTCL) is more common in men in a ratio of approximately 2:1.

Age

Most patients with cutaneous T-cell lymphoma (CTCL) are middle-aged or elderly. Many patients have had a poorly defined form of dermatitis for many years prior to the onset of CTCL. In a significant proportion of cases, the onset of the disease, or of a dermatitic precursor of the disease, occurred in childhood. CTCL starting in children younger than 10 years is exceedingly rare and does not show a male predominance; one series even reported a strong female predilection. Similar to adult patients, however, most children present in stage IA or IB and have a good-to-excellent prognosis with treatment, although cases progressing to plaque, tumor-stage disease, and death have been reported. Some patients with limited MF are described as having Woringer-Kolopp–type pagetoid reticulosis. These patients are usually middle-aged, with an age distribution in one series ranging from 13-68 years and a mean age of 55 years.

Previous
Proceed to Clinical Presentation
 
 
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.

Specialty Editor Board

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.

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.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at 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.

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.

References

  1. Burg G, Kempf W. Etiology and pathogenesis of cutaneous lymphomas. In: Burg G, Kempf W. Cutaneous Lymphomas. London: Taylor & Francis; 2005.

  2. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. May 15 2005;105(10):3768-85. [Medline].

  3. Burg G, Kempf W, Cozzio A, et al. Cutaneous malignant lymphomas: update 2006. J Dtsch Dermatol Ges. Nov 2006;4(11):914-33. [Medline].

  4. Khamaysi Z, Ben-Arieh Y, Izhak OB, Epelbaum R, Dann EJ, Bergman R. The applicability of the new WHO-EORTC classification of primary cutaneous lymphomas to a single referral center. Am J Dermatopathol. Feb 2008;30(1):37-44. [Medline].

  5. Berti E, Tomasini D, Vermeer MH, Meijer CJ, Alessi E, Willemze R. Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. A distinct clinicopathological entity with an aggressive clinical behavior. Am J Pathol. Aug 1999;155(2):483-92. [Medline].

  6. Kempf W, Ostheeren-Michaelis S, Paulli M, et al. Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC). Arch Dermatol. Dec 2008;144(12):1609-17. [Medline].

  7. Vonderheid EC, Bernengo MG, Burg G, et al. Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. J Am Acad Dermatol. Jan 2002;46(1):95-106. [Medline].

  8. Setoyama M, Katahira Y, Kanzaki T. Clinicopathologic analysis of 124 cases of adult T-cell leukemia/lymphoma with cutaneous manifestations: the smouldering type with skin manifestations has a poorer prognosis than previously thought. J Dermatol. Dec 1999;26(12):785-90. [Medline].

  9. Weenig RH, Ng CS, Perniciaro C. Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as lipomembranous panniculitis and a review of 72 cases in the literature. Am J Dermatopathol. Jun 2001;23(3):206-15. [Medline].

  10. Hoque SR, Child FJ, Whittaker SJ, et al. Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathological, immunophenotypic and molecular analysis of six patients. Br J Dermatol. Mar 2003;148(3):516-25. [Medline].

  11. Massone C, Chott A, Metze D, et al. Subcutaneous, blastic natural killer (NK), NK/T-cell, and other cytotoxic lymphomas of the skin: a morphologic, immunophenotypic, and molecular study of 50 patients. Am J Surg Pathol. Jun 2004;28(6):719-35. [Medline].

  12. Burg G, Dummer R, Wilhelm M, et al. A subcutaneous delta-positive T-cell lymphoma that produces interferon gamma. N Engl J Med. Oct 10 1991;325(15):1078-81. [Medline].

  13. El Shabrawi-Caelen L, Cerroni L, Kerl H. The clinicopathologic spectrum of cytotoxic lymphomas of the skin. Semin Cutan Med Surg. Jun 2000;19(2):118-23. [Medline].

  14. Miyamoto T, Yoshino T, Takehisa T, Hagari Y, Mihara M. Cutaneous presentation of nasal/nasal type T/NK cell lymphoma: clinicopathological findings of four cases. Br J Dermatol. Sep 1998;139(3):481-7. [Medline].

  15. Arnulf B, Copie-Bergman C, Delfau-Larue MH, et al. Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization. Blood. Mar 1 1998;91(5):1723-31. [Medline].

  16. Jaffe ES, Krenacs L, Raffeld M. Classification of cytotoxic T-cell and natural killer cell lymphomas. Semin Hematol. Jul 2003;40(3):175-84. [Medline].

  17. Bekkenk MW, Vermeer MH, Jansen PM, et al. Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients. Blood. Sep 15 2003;102(6):2213-9. [Medline].

  18. Alsaleh QA, Nanda A, Al-Ajmi H, et al. Clinicoepidemiological features of mycosis fungoides in Kuwait, 1991-2006. Int J Dermatol. Dec 2010;49(12):1393-8. [Medline].

  19. Pope E, Weitzman S, Ngan B, et al. Mycosis fungoides in the pediatric population: report from an international childhood registry of cutaneous lymphoma. J Cutan Med Surg. Jan-Feb 2010;14(1):1-6. [Medline].

  20. Shimauchi T, Sugita K, Nakamura M, Tokura Y. Leukaemic cutaneous T-cell lymphoma-manifesting papuloerythroderma with CD3(-) CD4(+) phenotype. Acta Derm Venereol. 2010;90(1):68-72. [Medline].

  21. Roeschm A, Schleyer V, Landthaler M, Vogt T. Follicular mycosis fungoides: variability of a rare entity. Skinmed. Jan-Feb 2005;4(1):12-7. [Medline].

  22. Muniesa C, Estrach T, Pujol RM, et al. Folliculotropic mycosis fungoides: clinicopathological features and outcome in a series of 20 cases. J Am Acad Dermatol. Mar 2010;62(3):418-26. [Medline].

  23. Pileri A, Facchetti F, Rutten A, et al. Syringotropic mycosis fungoides: a rare variant of the disease with peculiar clinicopathologic features. Am J Surg Pathol. Jan 2011;35(1):100-9. [Medline].

  24. Kohler S. Extranodal NK/T-cell lymphoma, nasal type. In: LeBoit P, Burg G, Weedon D, Lyon SA. WHO Books: Tumors of the Skin. Geneva: World Health Organization IARC. X. 2006:191-2.

  25. Clarijs M, Poot F, Laka A, Pirard C, Bourlond A. Granulomatous slack skin: treatment with extensive surgery and review of the literature. Dermatology. 2003;206(4):393-7. [Medline].

  26. Marzano AV, Berti E, Paulli M, Caputo R. Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol. Jul 2000;136(7):889-96. [Medline].

  27. Torok L, Gurbity TP, Kirschner A, Krenacs L. Panniculitis-like T-cell lymphoma clinically manifested as alopecia. Br J Dermatol. Oct 2002;147(4):785-8. [Medline].

  28. Toro JR, Liewehr DJ, Pabby N, et al. Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood. May 1 2003;101(9):3407-12. [Medline].

  29. Santucci M, Pimpinelli N, Massi D, et al. Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop. Cancer. Feb 1 2003;97(3):610-27. [Medline].

  30. von den Driesch P, Coors EA. Localized cutaneous small to medium-sized pleomorphic T-cell lymphoma: a report of 3 cases stable for years. J Am Acad Dermatol. Apr 2002;46(4):531-5. [Medline].

  31. Barrionuevo C, Anderson VM, Zevallos-Giampietri E, et al. Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru. Appl Immunohistochem Mol Morphol. Mar 2002;10(1):7-14. [Medline].

  32. Chen HH, Hsiao CH, Chiu HC. Hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma. Br J Dermatol. Sep 2002;147(3):587-91. [Medline].

  33. Bi MY, Curry JL, Christiano AM, Hordinsky MK, et al. The spectrum of hair loss in patients with mycosis fungoides and Sezary syndrome. J Am Acad Dermatol. Jan 2011;64(1):53-63. [Medline].

  34. Livingstone I, Ramamurthi S, Drummond S, Kemp E, Roberts F. Corneal perforation due to limbal involvement in Sézary syndrome. Graefes Arch Clin Exp Ophthalmol. Jan 21 2011;[Medline].

  35. Gutte R, Kharkar V, Mahajan S, Chikhalkar S, Khopkar U. Granulomatous mycosis fungoides with hypohidrosis mimicking lepromatous leprosy. Indian J Dermatol Venereol Leprol. Nov-Dec 2010;76(6):686-90. [Medline].

  36. Karube K, Aoki R, Nomura Y, et al. Usefulness of flow cytometry for differential diagnosis of precursor and peripheral T-cell and NK-cell lymphomas: analysis of 490 cases. Pathol Int. Feb 2008;58(2):89-97. [Medline].

  37. Russell-Jones R. Diagnosing erythrodermic cutaneous T-cell lymphoma. Br J Dermatol. Jul 2005;153(1):1-5. [Medline].

  38. Petrella T, Comeau MR, Maynadie M, et al. 'Agranular CD4+ CD56+ hematodermic neoplasm' (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes. Am J Surg Pathol. Jul 2002;26(7):852-62. [Medline].

  39. Smoller BR, Santucci M, Wood GS, Whittaker SJ. Histopathology and genetics of cutaneous T-cell lymphoma. Hematol Oncol Clin North Am. Dec 2003;17(6):1277-311. [Medline].

  40. Haghighi B, Smoller BR, LeBoit PE, Warnke RA, Sander CA, Kohler S. Pagetoid reticulosis (Woringer-Kolopp disease): an immunophenotypic, molecular, and clinicopathologic study. Mod Pathol. May 2000;13(5):502-10. [Medline].

  41. Mao X, Lillington D, Scarisbrick JJ, et al. Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sézary syndrome and mycosis fungoides. Br J Dermatol. Sep 2002;147(3):464-75. [Medline].

  42. Burg G, Zwingers T, Staegemeir E, Santucci M. Interrater and intrarater variabilities in the evaluation of cutaneous lymphoproliferative T-cell infiltrates. EORTC-Cutaneous Lymphoma Project Group. Dermatol Clin. Apr 1994;12(2):311-4. [Medline].

  43. Santucci M, Biggeri A, Feller AC, Massi D, Burg G. Efficacy of histologic criteria for diagnosing early mycosis fungoides: an EORTC cutaneous lymphoma study group investigation. European Organization for Research and Treatment of Cancer. Am J Surg Pathol. Jan 2000;24(1):40-50. [Medline].

  44. Santucci M, Smoller B. ISCL study on early diagnosis of mycosis fungoides. (in preparation).

  45. Pimpinelli N, Olsen EA, Santucci M, et al. Defining early mycosis fungoides. J Am Acad Dermatol. Dec 2005;53(6):1053-63. [Medline].

  46. Zelger B, Sepp N, Weyrer K, Grünewald K, Zelger B. Syringotropic cutaneous T-cell lymphoma: a variant of mycosis fungoides?. Br J Dermatol. Jun 1994;130(6):765-9. [Medline].

  47. Gerami P, Guitart J. Basaloid folliculolymphoid hyperplasia: a distinctive finding in follicular mycosis fungoides. J Cutan Pathol. Dec 2007;34 Suppl 1:29-32. [Medline].

  48. Imai S, Burg G, Braun-Falco O. Mycosis fungoides and Sezary's syndrome show distinct histomorphological features. Dermatologica. 1986;173(3):131-5. [Medline].

  49. Kamarashev J, Burg G, Kempf W, Hess Schmid M, Dummer R. Comparative analysis of histological and immunohistological features in mycosis fungoides and Sézary syndrome. J Cutan Pathol. Sep 1998;25(8):407-12. [Medline].

  50. Sibaud V, Beylot-Barry M, Thiebaut R, et al. Bone marrow histopathologic and molecular staging in epidermotropic T-cell lymphomas. Am J Clin Pathol. Mar 2003;119(3):414-23. [Medline].

  51. Takeshita M, Okamura S, Oshiro Y, et al. Clinicopathologic differences between 22 cases of CD56-negative and CD56-positive subcutaneous panniculitis-like lymphoma in Japan. Hum Pathol. Feb 2004;35(2):231-9. [Medline].

  52. Beljaards RC, Meijer CJ, Van der Putte SC, et al. Primary cutaneous T-cell lymphoma: clinicopathological features and prognostic parameters of 35 cases other than mycosis fungoides and CD30-positive large cell lymphoma. J Pathol. Jan 1994;172(1):53-60. [Medline].

  53. Chan JK, Sin VC, Wong KF, et al. Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood. Jun 15 1997;89(12):4501-13. [Medline].

  54. Natkunam Y, Smoller BR, Zehnder JL, Dorfman RF, Warnke RA. Aggressive cutaneous NK and NK-like T-cell lymphomas: clinicopathologic, immunohistochemical, and molecular analyses of 12 cases. Am J Surg Pathol. May 1999;23(5):571-81. [Medline].

  55. de Wolf-Peeters C, Achten R. gammadelta T-cell lymphomas: a homogeneous entity?. Histopathology. Apr 2000;36(4):294-305. [Medline].

  56. Jones D, Vega F, Sarris AH, Medeiros LJ. CD4-CD8-"Double-negative" cutaneous T-cell lymphomas share common histologic features and an aggressive clinical course. Am J Surg Pathol. Feb 2002;26(2):225-31. [Medline].

  57. Olsen EA, Rook AH, Zic J, et al. Sezary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol. Feb 2011;64(2):352-404. [Medline].

  58. Knobler E. Current management strategies for cutaneous T-cell lymphoma. Clin Dermatol. May-Jun 2004;22(3):197-208. [Medline].

  59. Ysebaert L, Truc G, Dalac S, et al. Ultimate results of radiation therapy for T1-T2 mycosis fungoides (including reirradiation). Int J Radiat Oncol Biol Phys. Mar 15 2004;58(4):1128-34. [Medline].

  60. Onozuka T, Yokota K, Kawashima T, et al. An elderly patient with mycosis fungoides successfully treated with chronic low-dose oral etoposide therapy. Clin Exp Dermatol. Jan 2004;29(1):91-2. [Medline].

  61. Dummer R, Urosevic M, Kempf W, Kazakov D, Burg G. Imiquimod induces complete clearance of a PUVA-resistant plaque in mycosis fungoides. Dermatology. 2003;207(1):116-8. [Medline].

  62. Apisarnthanarax N, Talpur R, Ward S, Ni X, Kim HW, Duvic M. Tazarotene 0.1% gel for refractory mycosis fungoides lesions: an open-label pilot study. J Am Acad Dermatol. Apr 2004;50(4):600-7. [Medline].

  63. Singh F, Lebwohl MG. Cutaneous T-cell lymphoma treatment using bexarotene and PUVA: a case series. J Am Acad Dermatol. Oct 2004;51(4):570-3. [Medline].

  64. Jones GW, Hoppe RT, Glatstein E. Electron beam treatment for cutaneous T-cell lymphoma. Hematol Oncol Clin North Am. Oct 1995;9(5):1057-76. [Medline].

  65. Leverkus M, Rose C, Bröcker EB, Goebeler M. Follicular cutaneous T-cell lymphoma: beneficial effect of isotretinoin for persisting cysts and comedones. Br J Dermatol. Jan 2005;152(1):193-4. [Medline].

  66. Jacob R, Scala M, Fung MA. A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy. J Am Acad Dermatol. Jan 2009;60(1):152-4. [Medline].

  67. Russell-Jones R. Extracorporeal photopheresis in cutaneous T-cell lymphoma. Inconsistent data underline the need for randomized studies. Br J Dermatol. Jan 2000;142(1):16-21. [Medline].

  68. Lundin J, Hagberg H, Repp R, et al. Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood. Jun 1 2003;101(11):4267-72. [Medline].

  69. Fukushima T, Horio K, Matsuo E, et al. Successful cord blood transplantation for mycosis fungoides. Int J Hematol. Dec 2008;88(5):596-8. [Medline].

  70. Tsuji H, Wada T, Murakami M, et al. Two cases of mycosis fungoides treated by reduced-intensity cord blood transplantation. J Dermatol. Dec 2010;37(12):1040-5. [Medline].

  71. Lambert WC, Cohen PJ, Schwartz RA. Surgical management of mycosis fungoides. J Med. 1997;28(3-4):211-22. [Medline].

  72. Foss F. Overview of cutaneous T-cell lymphoma: prognostic factors and novel therapeutic approaches. Leuk Lymphoma. 2003;44 Suppl 3:S55-61. [Medline].

  73. Kaye FJ, Bunn PA Jr, Steinberg SM, et al. A randomized trial comparing combination electron-beam radiation and chemotherapy with topical therapy in the initial treatment of mycosis fungoides. N Engl J Med. Dec 28 1989;321(26):1784-90. [Medline].

  74. Meyer N, Paul C, Misery L. Pruritus in cutaneous T-cell lymphomas: frequent, often severe and difficult to treat. Acta Derm Venereol. 2010;90(1):12-7. [Medline].

  75. Agar NS, Wedgeworth E, Crichton S, et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal. J Clin Oncol. Nov 1 2010;28(31):4730-9. [Medline].

  76. Tancrede-Bohin E, Ionescu MA, de La Salmoniere P, et al. Prognostic value of blood eosinophilia in primary cutaneous T-cell lymphomas. Arch Dermatol. Sep 2004;140(9):1057-61. [Medline].

  77. van Doorn R, Scheffer E, Willemze R. Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients. Arch Dermatol. Feb 2002;138(2):191-8. [Medline].

  78. Scarisbrick JJ, Whittaker S, Evans AV, et al. Prognostic significance of tumor burden in the blood of patients with erythrodermic primary cutaneous T-cell lymphoma. Blood. Feb 1 2001;97(3):624-30. [Medline].

  79. Shimoyama M. Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). Br J Haematol. Nov 1991;79(3):428-37. [Medline].

  80. Fink-Puches R, Zenahlik P, Back B, Smolle J, Kerl H, Cerroni L. Primary cutaneous lymphomas: applicability of current classification schemes (European Organization for Research and Treatment of Cancer, World Health Organization) based on clinicopathologic features observed in a large group of patients. Blood. Feb 1 2002;99(3):800-5. [Medline].

  81. Bekkenk MW, Jansen PM, Meijer CJ, Willemze R. CD56+ hematological neoplasms presenting in the skin: a retrospective analysis of 23 new cases and 130 cases from the literature. Ann Oncol. Jul 2004;15(7):1097-108. [Medline].

  82. Mraz-Gernhard S, Natkunam Y, Hoppe RT, LeBoit P, Kohler S, Kim YH. Natural killer/natural killer-like T-cell lymphoma, CD56+, presenting in the skin: an increasingly recognized entity with an aggressive course. J Clin Oncol. Apr 15 2001;19(8):2179-88. [Medline].

  83. Burg G, Kempf W. Therapy of cutaneous lymphomas. In: Burg G, Kempf W. Cutaneous Lymphomas. London: Taylor & Francis; 2005:475-528.

  84. Burg G, Kempf W, Cozzio A, et al. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J Cutan Pathol. Nov 2005;32(10):647-74. [Medline].

 
Previous
Next
 
Tumor-stage cutaneous T-cell lymphoma.
Patch-stage cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.
Plaque-stage parapsoriasis.
Patch-stage mycosis fungoides progressing to plaque stage with cutaneous cigarette-paper appearance evident.
Erythematous partially confluent plaques in advancing mycosis fungoides.
Close-up view of advancing plaque-stage mycosis fungoides with partially confluent erythematous plaques.
Early patch-stage cutaneous T-cell lymphoma.
Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.
Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.
Nodular mycosis fungoides.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Mycosis fungoides with large atypical T lymphocytes in both epidermis and dermis.
Mycosis fungoides with epidermal nest of large atypical T cells displaying bizarre, convoluted nuclei surrounded by a clear space (Pautrier microabscess).
Table 1. Staging of CTCL
StageSkin LesionsLymphadenopathyErythrodermaHistological Lymphoma
Patches



< 10%



Plaques



³10%



TumorsLymph NodesViscera
IA+------
IB+ or -+-----
IIA+ or -+ or -+ or -+---
IIB+ or -+ or -++ or ----
III+ or -+ or -+ or -+ or -+--
IVA+ or -+ or -+ or -+ or -+ or -+-
IVB+ or -+ or -+ or -+ or -+ or -+ or -+
Table 2. TNMB Staging of CTCL
Stage ClassStageDefinition
T



(Tumor)



T1Patches/plaques involving < 10% of body surface
T2Patches/plaques involving ³10% of body surface
T3Tumor(s) present on skin
T4Erythroderma
N



(Nodes)



N0No enlarged lymph node present
N1Enlarged lymph nodes, histologically uninvolved
N2No enlarged lymph node; one or more nodes histologically involved*
N3Enlarged lymph nodes, histologically involved
M



(Metastasis to viscera)



M0No visceral lesion present
M1Visceral involvement
B



(Blood involvement)



B0Circulating atypical lymphocytes (Sézary cells) £5% of lymphocytes
B1Circulating atypical lymphocytes ³5% of lymphocytes (Sézary syndrome)
*Uncommon finding, usually not considered/investigated.
Table 3. Comparison of Staging Systems for CTCL
Clinical StageTNM (B) Stage
IAT1 N0 M0
IIBT2 N0 M0
IIAT1 N1 M0T2 N1 M0
IIBT3 N0 M0T3 N1 M0
IIIT4 N0 M0T4 N1 M0
IVAT1 N2 M0T2 N2 M0T3 N2 M0T4 N2 M0
T1 N3 M0T2 N3 M0T3 N3 M0T4 N3 M0
IVBT1 N0 M1T2 N0 M1T3 N0 M1T4 N0 M1
T1 N1 M1T2 N1 M1T3 N1 M1T4 N1 M1
T1 N2 M1T2 N2 M1T3 N2 M1T4 N2 M1
T1 N3 M1T2 N3 M1T3 N3 M1T4 N3 M1
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.