eISSN: 2084-9893
ISSN: 0033-2526
Dermatology Review/Przegląd Dermatologiczny
Current issue Archive About the journal Special Issues Abstracting and indexing Subscription Contact Instructions for authors
SCImago Journal & Country Rank
vol. 104
Special paper

Primary cutaneous lymphomas – diagnostic and therapeutic guidelines of the Polish Dermatological Society

Małgorzata Sokołowska-Wojdyło, Joanna Maj, Ewa Robak, Waldemar Placek, Anna Wojas-Pelc, Alina Jankowska-Konsur, Karolina Olek-Hrab, Robert Gniadecki, Lidia Rudnicka

Dermatol Rev/Przegl Dermatol 2017, 104, 243–268
Online publish date: 2017/07/05
View full text
Get citation
JabRef, Mendeley
Papers, Reference Manager, RefWorks, Zotero
Primary cutaneous lymphomas are a group of rare lymphoproliferative diseases with incompletely understood pathogenesis. They comprise cutaneous T-cell lymphomas, cutaneous B-cell lymphomas and T/NK-cell lymphomas. The aim of the guidelines is to provide physicians with a tool to facilitate the diagnostic and therapeutic process in patients with this group of diseases. The updated recommendations are consistent with the guidelines for haematologists and oncologists, which will enable the unification of diagnostics and therapy conducted by physicians of all specialties. However, it is important to note that therapeutic decisions must be made individually on the basis of the general condition of the patient, history of internal diseases and medicines used, with careful consideration of the potential efficacy and safety profile of the proposed treatment, which may differ from patient to patient.

guidelines, lymphomatoid papulosis, diagnostics, treatment, mycosis fungoides

Whitmore A.S., Holly E.A., Lee I.M., Abel EA, Adams RM, Nickoloff BJ, et al.: Mycosis fungoides in relation to environmental exposure and immune response. J Natl Cancer Inst 1989, 81, 1560-1567.
Li G., Vowels B.R., Benoit B.M., Rook A.H., Lessin S.R.: Failure to detect human T-lymphotropic virus type I (HTLV-I) proviral DNA in cell lines and tissues from patients with cutaneous T-cell lymphoma. J Invest Dermatol 1996, 7, 308-313.
Agar N.S., Wedgeworth E., Crichton S., Mitchell T.J., Cox M., Ferreira 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 2010, 28, 4730-4739.
Jahan-Tigh R.R., Huen A.O., Lee G.L., Pozadzides J.V., Liu P., Duvic M.: Hydrochlorothiazide and cutaneous T-cell lymphoma: prospective analysis and case series. Cancer 2013, 119, 825-831.
Sokołowska-Wojdyło M.: Pierwotne chłoniaki skóry. [In:] Onkologia kliniczna. M. Krzakowski, P. Potemski, K. Warzocha,
P. Wysocki (eds.). Via Medica, Gdańsk, 2015, 1382-1402.
Sokołowska-Wojdyło M.: Pierwotne chłoniaki skóry. [In:] Zalecenia postępowania diagnostyczno-terapeutycznego w nowotworach złośliwych. Vol. 2. Nowotwory układu chłonnego. K. Warzocha, M. Prochorec-Sobieszek, E. Lech-Marańda (eds.). VM Media, Gdańsk, 2013, 948-968.
Sokołowska-Wojdyło M.: Zasady postępowania w chłoniakach pierwotnie skórnych. [In:] Praktyka hematologiczna. W.W. Jędrzejczak, T. Robak, M. Podolak-Dawidziak (eds.). Termedia, Poznań, 2015, 271-284.
Sokołowska-Wojdyło M., Lech-Marańda E., Placek W., Meder J., Zaucha J.M., Walewski J.: Leczenie pierwotnych chłoniaków skóry. Rekomendacje Sekcji Chłoniaków Skóry Polskiej Grupy Badawczej Chłoniaków (PLRG). Onkol Prakt Klin 2010; 6: 29-47.
Wong H.K., Mishra A., Hake T., Porcu P.: Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome). Br J Haematol 2011, 155, 150-166.
Sokołowska-Wojdyło M., Olek-Hrab K., Ruckemann-Dziurdzińska K.: Primary cutaneous lymphomas: diagnosis and treatment. Postep Dermatol Alergol 2015, 32, 368-383.
Bagot M.: Cutaneous T-cell lymphoma (CTCL) – classification, staging, and treatment options. Dermatol Clin 2008, 26 (Suppl 1), 3-12.
Swerdlow S., Campo E., Harris N., Jaffe E.S., Pileri S.A., Stein H., et al. (ed.).: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARC Press, Lyon 2008.
Reich A., Maj J., Schlue J., Kreipe H.H., Mazur G.: Primary cutaneous peripheral T-cell non-Hodgkin lymphoma, not otherwise specified, with cytotoxic features. Int J Dermatol 2010, 49, 967-969.
Stevens S.R., Ke M.S., Parry E.J., Mark J., Cooper K.D.: Quantifying skin disease burden in mycosis fungoides- type cutaneous T-cell lymphomas: the severity-weighted assessment tool (SWAT). Arch Dermatol 2002, 138, 42-48.
Wilcox R.A.: Cutaneous T-cell lymphoma: 2014 update on diagnosis, risk-stratification, andmanagement. Am J Hematol 2014, 89, 837-851.
Kempf W., Sander C.A.: Classification of cutaneous lymphomas – an update. Histopathology 2010, 56, 57-70.
Maj J., Kołodziej T., Woźniak Z.: Subcutaneous T-cell lymphoma. A clinical and histopathological study of an additional case. Acta Dermatovenerol Croat 2004, 12, 274-277.
Willemze R., Jansen P.M., Cerroni L., Berti E., Santucci M., Assaf C., et al.: Subcutaneous panniculitis like T-cell lymphoma: definition, classification and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood 2008, 111, 838-845.
Maryniak R.K., Jankowska-Konsur A.: Histoclinical and immunohistochemical diagnosis of cutaneous lymphomas. Pol J Pathol 2011, 62 (4 Suppl 3), 1-23.
Pimpinelli N., Olsen E.A., Santucci M., Vonderheid E., Haeffner A.C., Stevens S., et al.: International Society for Cutaneous Lymphoma. Defining early mycosis fungoides. J Am Acad Dermatol 2005, 53, 1053-1063.
van Doorn R., Scheffer E., Willemze R.: Follicular mycosis fungoides: a distinct disease entity with or without associated follicular mucinosis. Arch Dermatol 2002, 138, 191-198.
Gerami P., Rosen S., Kuzel T., Boone S.L., Guitart J.: Folliculotropic mycosis fungoides: an aggressive variant of cutaneous T-cell lymphoma. Arch Dermatol 2008, 144, 738-746.
Trautinger F., Eder J., Assaf C., Bagot M., Cozzio A., Dummer R., et al.: European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2017. Eur J Cancer 2017, 77, 57-74.
Plettenberg H., Stege H., Megahed M., Ruzicka T., Hosokawa Y., Tsuji T., et al.: Ultraviolet A1 (340-400 nm) phototherapy for cutaneous T-cell lymphoma. J Am Acad Dermatol 1999, 41, 47-50.
Zane C., Leali C., Airo P., De Panfilis G., Pinton P.C.: “High-dose” UVA1 therapy of widespread plaquetype, nodular, and erythrodermic mycosis fungoides. J Am Acad Dermatol 2001, 44, 629-633.
Musson R.E., Hensbergen P.J., Westphal A.H., Temmink W.P., Deelder A.M., van Pelt J., et al.: UVA1 radiation inhibits calcineurin through oxidative damage mediated by photosensitization. Free Radic Biol Med 2011, 50, 1392-1399.
Kamstrup M.R., Lindahl L.M., Gniadecki R., Iversen L., Skov L., Petersen P.M., et al.: Low-dose total skin electron beam therapy as a debulking agent for cutaneous T-cell lymphoma: an open-label prospective phase II study. Br J Dermatol 2012, 166, 399-404.
Navi D., Riaz N., Levin Y.S., Sullivan N.C., Kim Y.H., Hoppe R.T.: The Stanford University experience with conventionaldose, total skin electron-beam therapy in the treatment of generalized patchor plaque (T2) and tumor (T3) mycosis fungoides. Arch Dermatol 2011, 147, 561-567.
Chan D.V., Aneja S., Honda K., Carlson S., Yao M., Katcher J., et al.: Radiation therapy in the management of unilesional primary cutaneous T-cell lymphomas. Br J Dermatol 2012, 166, 1134-1137.
Mann B.S., Johnson J.R., He K., Sridhara R., Abraham S., Booth B.P., et al.: Vorinostat for treatment of cutaneous manifestations of advanced primary cutaneous T-cell lymphoma. Clin Cancer Res 2007, 13, 2318-2322.
Quereux G., Marques S., Nguyen J.M., Bedane C., D‘incan M., Dereure O., et al.: Prospective multicenter study of pegylated liposomal doxorubicin treatment in patients with advanced or refractory mycosis fungoides or Sézary syndrome. Arch Dermatol 2008, 144, 727-733.
Duarte R.F., Schmitz N., Servitje O., Sureda A.: Haematopoietic stem cell transplantation for patients with primary cutaneous T-cell lymphoma. Bone Marrow Transplant 2008, 41, 597-604.
Shiratori S., Fujimoto K., Nishimura M., Hatanaka K.C., Kosugi-Kanaya M., Okada K., et al.: Allogeneic hematopoietic stem cell transplantation following reduced-intensity conditioning for mycosis fungoides and Sezary syndrome. Hematol Oncol 2016, 34, 9-16.
Zinzani P.L., Baliva G., Magagnoli M., Bendandi M., Modugno G., Gherlinzoni F., et al.: Gemcitabine treatment in pretreated cutaneous T-cell lymphoma: experience in 44 patients. J Clin Oncol 2000, 18, 2603-2606.
Prince H.M., Whittaker S., Hoppe R.T.: How I treat mycosis fungoides and Sézary syndrome. Blood 2009, 114, 4337-4353.
Kempf W., Pfaltz K., Vermeer M.H., Cozzio A., Ortiz-Romero P.L., Bagot M., et al.: EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood 2011, 118, 4024-4035.
Maj J., Jankowska-Konsur A.: CD30+ T-cell Lymphoproliferative disorders of the skin. [In:] New research on cutaneous lymphomas. Nova Science Publishers, New York 2007, 87-103.
Younes A., Bartlett N.L., Leonard J.P., Kennedy D.A., Lynch C.M., Sievers E.L., et al.: Brentuximab vedotin (SGN-35) for relapsed CD30-positive lymphomas. N Engl J Med 2010, 363, 1812-1821.
Murga C.Z., Smith L.B.: Primary cutaneous acral CD8+ T cell lymphoma Copyright: 2002-2017, PathologyOutlines.com, Inc. Revised: 28 March 2017, last major update August 2016.
Senff N.J., Noordijk E.M., Kim Y.H., Bagot M., Berti E., Cerroni L., et al.: European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood 2008, 112, 1600-1609.
Quick links
© 2018 Termedia Sp. z o.o. All rights reserved.
Developed by Bentus.
PayU - płatności internetowe