Abstract
Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma (pcSMPTCL) is an extremely rare lymphoma with an overall excellent prognosis. It is a provisional entity in the World Health Organization–European Organization for Research and Treatment of Cancer (WHO/EORTC) guidelines and is considered to have both indolent and aggressive forms. pcSMPTCL typically presents with a persistent, asymptomatic, solitary erythematous nodule or pink plaque on the head, neck, or trunk. Given the generally benign clinical course of this disease, some advocate the use of alternative terms, including T-cell pseudolymphoma, cutaneous nodular proliferation of pleomorphic T lymphocytes of undetermined significance, or clonal T-cell lymphoproliferative disease with indolent behavior [1, 2]. Given the dramatic difference in prognosis between the indolent and aggressive forms, it is possible that this entity, as currently defined, encompasses two distinct diseases. This chapter discusses the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, molecular characteristics, and differential diagnosis of pcSMPTCL. It closes with a clinical case.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Jaffe ES, Nicolae A, Pittaluga S. Peripheral T-cell and NK-cell lymphomas in the WHO classification: pearls and pitfalls. Mod Pathol. 2013;26 Suppl 1:S71–87.
Beltraminelli H, Leinweber B, Kerl H, Cerroni L. Primary cutaneous CD4+ small-/medium-sized pleomorphic T-cell lymphoma: a cutaneous nodular proliferation of pleomorphic T lymphocytes of undetermined significance? A study of 136 cases. Am J Dermatopathol. 2009;31:317–22.
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 tumors of haematopoietic and lymphoid tissues. Lyon: International Agency for Research on Cancer; 2008. p. 302–5.
Ally MS, Prasad Hunasehally RY, Rodriguez-Justo M, Martin B, Verdolini R, Attard N, et al. Evaluation of follicular T-helper cells in primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma and dermatitis. J Cutan Pathol. 2013;40:1006–13.
Grogg KL, Jung S, Erickson LA, McClure RF, Dogan A. Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoma: a clonal T-cell lymphoproliferative disorder with indolent behavior. Mod Pathol. 2008;21:708–15.
RodrĂguez Pinilla SM, Roncador G, RodrĂguez-Peralto JL, Mollejo M, GarcĂa JF, Montes-Moreno S, et al. Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma expresses follicular T-cell markers. Am J Surg Pathol. 2009;33:81–90.
Williams VL, Torres-cabala CA, Duvic M. Primary cutaneous small- to medium-sized CD4+ pleomorphic T-cell lymphoma. Am J Clin Dermatol. 2011;12:389–401.
Garcia-Herrera A, Colomo L, Camós M, Carreras J, Balague O, Martinez A, et al. Primary cutaneous small/medium CD4+ T-cell lymphomas: a heterogeneous group of tumors with different clinicopathologic features and outcome. J Clin Oncol. 2008;26:3364–71.
Cetinozman F, Jansen PM, Willemze R. Expression of programmed death-1 in primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoma, cutaneous pseudo-T-cell lymphoma, and other types of cutaneous T-cell lymphoma. Am J Surg Pathol. 2012;36:109–16.
Baum CL, Link BK, Neppalli VT, Swick BL, Liu V. Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: a series of 10 adult and pediatric patients and review of the literature. J Am Acad Dermatol. 2011;65:739–48.
Isaacson P, Chott A, Nakamura S, Muller-Hermelink HK, Harris NL, Swerdlow SH. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO classification of tumors of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer; 2008. p. 214–7.
Servitje O, Gallardo F, Estrach T, Pujol RM, Blanco A. Clinical and laboratory investigations primary cutaneous marginal zone B-cell lymphoma: a clinical, histopathological, immunophenotypic and molecular genetic study of 22 cases. Br J Dermatol. 2002;147:1147–58.
Geyer JT, Ferry JA, Longtine JA, Flotte TJ, Harris NL, Zukerberg LR. Characteristics of cutaneous marginal zone lymphomas with marked plasmacytic differentiation and a T cell-rich background. Am J Clin Pathol. 2010;133(1):59–69.
Senff NJ, Hoefnagel JJ, Jansen PM, Vermeer MH, van Baarlen J, Blokx WA, et al. Reclassification of 300 primary cutaneous B-cell lymphomas according to the new WHO-EORTC classification for cutaneous lymphomas: comparison with previous classifications and identification of prognostic markers. J Clin Oncol. 2007;25:1581–7.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Goyal, A., Carter, J.B., Duncan, L.M. (2015). Primary Cutaneous CD4+ Small/Medium Pleomorphic 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_14
Download citation
DOI: https://doi.org/10.1007/978-3-319-17217-0_14
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-17216-3
Online ISBN: 978-3-319-17217-0
eBook Packages: MedicineMedicine (R0)