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Primary Cutaneous CD8+ Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma

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Atlas of Cutaneous Lymphomas

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Abstract

Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (CD8+ AECTCL) is a rare, aggressive cutaneous T-cell lymphoma with a poor prognosis. Although CD8+ AECTCL typically initially presents on the skin, visceral dissemination is common. Given the rarity of this condition, it is considered a provisional entity in the fourth edition of the World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification guidelines. In this chapter we discuss the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, molecular characteristics, and differential diagnosis of CD8+ AECTCL. The chapter closes with a clinical case.

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References

  1. Gaulard P, Berti E, Willemze R, Jaffe ES. Primary cutaneous peripheral T-cell lymphomas, rare subtypes. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. p. 302–5.

    Google Scholar 

  2. 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. 1999;155:483–92.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  3. Gormley RH, Hess SD, Anand D, Junkins-Hopkins J, Rook AH, Kim EJ. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. J Am Acad Dermatol. 2010;62:300–7.

    Article  PubMed  Google Scholar 

  4. Nofal A, Abdel-Mawla MY, Assaf M, Salah E. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: proposed diagnostic criteria and therapeutic evaluation. J Am Acad Dermatol. 2012;67:748–59.

    Article  PubMed  Google Scholar 

  5. Liu V, Cutler C, Young AZ. Case 38–2007: a 44-year-old woman with generalized, painful, ulcerated skin lesions. N Engl J Med. 2007;357:2496–505.

    Article  CAS  PubMed  Google Scholar 

  6. Diwan H, Ivan D. CD8-positive mycosis fungoides and primary cutaneous aggressive epidermotropic CD8-positive cytotoxic T-cell lymphoma. J Cutan Pathol. 2009;36:390–2.

    Article  PubMed  Google Scholar 

  7. Marzano AV, Ghislanzoni M, Gianelli U, Caputo R, Alessi E, Berti E. Fatal CD8+ epidermotropic cytotoxic primary cutaneous T-cell lymphoma with multiorgan involvement. Dermatology. 2005;211:281–5.

    Article  PubMed  Google Scholar 

  8. Santucci M, Pimpinelli N, Massi D, Kadin ME, Meijer CJLM, Müller-Hermelink HK, et al. Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop. Cancer. 2003;97:610–27.

    Article  PubMed  Google Scholar 

  9. Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol Venerol. 2009;23:1008–17.

    Article  CAS  Google Scholar 

  10. Saggini A, Gulia A, Argenyi Z, Fink-Puches R, Lissia A, Magaña M, et al. A variant of lymphomatoid papulosis simulating primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma. Description of 9 cases. Am J Surg Pathol. 2010;34:1168–75.

    Article  PubMed  Google Scholar 

  11. Kempf W, Kazakov DV, Ru A, Mentzel T, Paredes BE, Palmedo G, et al. Angioinvasive lymphomatoid papulosis. Am J Surg Pathol. 2013;37:1–13.

    Article  PubMed  Google Scholar 

  12. Chan J, Quintanilla-Martinez L, Ferry J, Peh S-C. Extranodal NK/T-cell lymphoma, nasal type. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. p. 285–8.

    Google Scholar 

  13. Roschewski M, Wilson WH. Lymphomatoid granulomatosis. Cancer J. 2012;18:469–74.

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Harvard-MIT Division of Health Sciences and Technology, Harvard Medical School, Boston, MA, USA

    Amrita Goyal M.D.

  2. Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Joi B. Carter M.D. & Daniela Kroshinsky M.D.

  3. Comprehensive Cutaneous Lymphoma Program, Department of Medicine, Massachusetts General Hospital Cancer Center, Boston, MA, USA

    Jeffrey A. Barnes M.D., Ph.D.

  4. Hematopathology Service, Department of Pathology Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Aliyah R. Sohani M.D.

  5. Dermatopathology Service, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Lyn McDivitt Duncan M.D.

Authors
  1. Amrita Goyal M.D.
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  2. Joi B. Carter M.D.
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  3. Daniela Kroshinsky M.D.
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  4. Jeffrey A. Barnes M.D., Ph.D.
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  5. Aliyah R. Sohani M.D.
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  6. Lyn McDivitt Duncan M.D.
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Corresponding author

Correspondence to Lyn McDivitt Duncan M.D. .

Editor information

Editors and Affiliations

  1. Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA

    Joi B. Carter

  2. Division of Health Sciences and Technology, Harvard Medical School, Boston, Massachusetts, USA

    Amrita Goyal

  3. Dermatopathology Service, Massachusetts General Hospital, Boston, Massachusetts, USA

    Lyn McDivitt Duncan

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© 2015 Springer International Publishing Switzerland

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Goyal, A., Carter, J.B., Kroshinsky, D., Barnes, J.A., Sohani, A.R., Duncan, L.M. (2015). Primary Cutaneous CD8+ Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma. In: Carter, J., Goyal, A., McDivitt Duncan, L. (eds) Atlas of Cutaneous Lymphomas. Springer, Cham. https://doi.org/10.1007/978-3-319-17217-0_12

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  • DOI: https://doi.org/10.1007/978-3-319-17217-0_12

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-17216-3

  • Online ISBN: 978-3-319-17217-0

  • eBook Packages: MedicineMedicine (R0)

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