Mikosis Fungoides Folikulotropik Disertai Ko-Ekspresi Pan B-Cell Markers Dengan Manifestasi Klinis Berupa Facies Leonina
DOI:
https://doi.org/10.33820/mdvi.v45i4.37Abstract
Mikosis fungoides folikulotropik (MFF) merupakan varian dari mikosis fungoides (MF) dengan perjalanan klinis yang lebih agresif dan prognosis lebih buruk dibandingkan MF klasik. Facies leonina (FL) merupakan manifestasi klinis yang sangat jarang ditemukan pada MFF. CD20 dan CD79a merupakan pan B-cell markers yang sangat jarang ditemukan pada kasus cutaneous T-cell lymphoma (CTCL), termasuk di dalamnya MFF. Dilaporkan satu kasus MFF pada seorang laki-laki berusia 51 tahun dengan manifestasi klinis FL dan madarosis disertai papula folikel menyerupai lesi keratosis pilaris pada dada, punggung, kedua lengan, dan paha. Diagnosis MFF pada pasien ini ditegakkan berdasarkan pemeriksaan histopatologis sediaan biopsi kulit wajah yang menunjukkan gambaran folikulotropisme. Pada pemeriksaan imunohistokimia ditemukan fenotipe CD3 yang dominan, CD4, CD8, CD30, dan Ki-67 lebih dari 40% sel tumor. Penanda sel-B juga ditemukan positif pada beberapa sel yaitu CD20, CD79a, dan bcl-2. Setelah dua bulan diterapi dengan kortikosteroid topikal tidak didapatkan pendataran pada lesi FL. Pasien direncanakan pemberian regimen kemoterapi siklofosfamid, doxorubicin, vincristine, dan prednison. Mikosis fungoides folikulotropik dapat bermanifestasi klinis FL. Fenotipe CD20 dan CD79a pada kasus ini bukan merupakan cutaneous B-cell lymphoma karena fenotipe CD3, CD4, dan CD8 yang ditemukan lebih dominan. Folikulotropisme menyebabkan MFF kurang responsif terhadap skin-targeted therapies sehingga membutuhkan terapi yang lebih agresif.Kata kunci: facies leonina, mikosis fungoides folikulotropik, pan-B cell markers
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References
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3. Gerami P, Rosen S, Kuzel T, Boone SL, Guitart J. Folliculotropic mycosis fungoides an aggressive variant of cutaneous T-cell lymphoma. Arch Dermatol. 2008;144(6):738-46.
4. Beyer M, Sterry W. Cutaneous lymphoma. Dalam: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, dkk., penyunting. Fitzpatrick dermatology in general medicine. Edisi ke8. New York: McGraw-Hill; 2012.h.1745-66.
5. Willemze R. Cutaneous T-cell lymphoma. Dalam: Bolognia JL, Jorizzo JL,Rapini RP, penyunting. Dermatology. Edisi ke-2. Philadelphia: Mosby; 2008.h.1867-86.
6. Vergote, Wolter P, Hauben E, Busschots AM. Folliculotropic mycosis fungoides: A clinicopathological study of aseries of 10 cases. J Dermatol Res Ther.2016,2:1-5.
7. Keehn CA, Belongie IP, Shistik G, Fenske NA, Glass LF. The diagnosis, staging, and treatment options for mycosis fungoides. Cancer Control. 2007;14(2):104-11.
8. Ishibashi M, Ohshima K, Chen KR. Folliculotropic mycosis fungoides with eosinophilia and CD30+ large-cell transformation: a case with a fatal outcome presenting with multifocal lesions and leonine facies. Clin Exp Dermatol. 2009;35:133–6.
9. Jindal N, Jindal P, Kumar J, VK Jain. Animals eponyms in dermatology. Indian J Dermatol. 2014;59(6):631.
10. Brown DN, Wieser I, Wang C, Dabaja BS, Duvic M. Leonine facies (LF) and mycosis fungoides (MF): A single-center study and systematic review of the literature. J Am Acad Dermatol. 2015;73:976-86.
11. Strutton G. Cutaneous infiltrates- lymphomatous and leukemic. Dalam:Weedon D, penyunting. Weedon’s skin pathology. Edisi ke-3. China: Elsevier; 2010. h.971-87.
12. Hodak E, Amitay-Laish I, Atzmony L, Prag-Naveh H, Yanichkin N, Kershenovich R, dkk. New insights into folliculotropic mycosis fungoides (FMF): A single-center experience. J Am Acad Dermatol. 2016:75(2):347-55
13. El-Darouti MA. Cutaneous lymphoma and lympocytic infiltrate. Dalam: El Darouti MA, penyunting. Challenging Cases in Dermatology. Edisi ke-1. London: Springer-Verlag; 2013.h.229-316.
14. Olsen E, Vonderland E, Pimpinelli N, Willemze R, Kim Y, Knobler R, dkk. Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110:1713-22.
15. Trautinger F, Knobler R, Willemze R, Peris K, Stadler R, Laroche L, dkk. EORTC consensus recommendation for the treatment of mycosis fungoides/sezary syndrome. Eur J Cancer. 2006;42:1014-30.
16. Doorn V, Scheffer E, Willemze R. Follicular mycosis fun
goides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients. Arch Dermatol. 2002;138(2):191-8.
17. James WD, Berger TG, Elston DM. Cutaneous lymphoid hyperplasia, cutaneous T-cell lymphoma, other malignant lymphomas, and allied diseases. Dalam: James WD, Berger TG, Elston DM, penyunting. Andrew’s diseases of the skin, clinical dermatology. Edisi ke-11. Philadelphia: WB Saunders Company; 2006.h.720-40.
18. Scarisbrick JJ, Prince HM, Vermeer MH, Quaglino P, Horwitz S, Porcu P, dkk. Cutaneous lymphoma international consortium (CLIC) study outcome in advanced stage of mycosis fungoides & Sézary syndrome: Effect of specific prognostic markers on survival. J Clin Oncol.2015;33(32):3766-73. 19. Twomey J. Proliferative disorders of the T-cell series. Dalam: Good RA, Day SB, penyunting. Comprehensive immunology. The Immunopathology of lymphoreticular neoplasms. Edisi ke-1. New York and London: Plenum Publishing Corporation; 1978;h. 493-523.
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Published
2019-04-27
How to Cite
Sutedja, E. K., Febrina, D., Rohana, E., Roslina, N., Agusni, J. H., & Fadjari, T. H. (2019). Mikosis Fungoides Folikulotropik Disertai Ko-Ekspresi Pan B-Cell Markers Dengan Manifestasi Klinis Berupa Facies Leonina. Media Dermato-Venereologica Indonesiana, 45(4). https://doi.org/10.33820/mdvi.v45i4.37
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