Palabras clave
linfoma cutáneo, pediátrico, micosis fungoide.
Cómo citar
Dugo, R., Manente, R., Wittmund, L., Gutiérrez, M., Maglio, S., & Aversa, L. (2019). Linfomas T cutáneos en pacientes pediátricos. Revista Hematología, 21(3), 324–334. Recuperado a partir de https://revistahematologia.com.ar/index.php/Revista/article/view/172
Resumen
Los linfomas cutáneos (LCP) son un grupo de linfoma no Hodgkin de baja frecuencia. Para su diagnóstico es imprescindible el estudio anatomo-patológico que permite su clasificación y estadificación posterior. Los tipos más comunes de LCP en niños son los linfomas de células T maduras cutáneas (LCCT) y las enfermedades linfoproliferativas CD30+. El pronóstico depende del estadío de la enfermedad al diagnóstico, grado de afectación de la piel y presencia o ausencia de enfermedad extracutánea. Las terapias dirigidas a la piel se recomiendan para los pacientes con enfermedad localizada. Se describen cuatro casos clínicos pediátricos y una revisión de la literatura actualizada.
Citas
1. Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, DiazPerez JL, Duncan LM, Grange F, Harris NL, Kempf W, Kerl H, Kurrer M, Knobler R, Pimpinelli N, Sander C, Santucci M, Sterry W et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768-3785.
2. Wilcox, RA. Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk stratification, and management. Am J Hematol. 2016 January; 91(1): 151-165.
3. Pope E,Weitzman S, Ngan B,Walsh S,Morel K,Williams J, Stein S, Garzon M, Knobler E, Lieber C, Turchan. K,Wargon O, Tsuchiya A. Mycosis fungoides in the pediatric population: Report from
an international Childhood Registry of Cutaneous Lymphoma. J Cutan Med Surg. 2010; 14:1-6.
4. Fink-Puches R, Chott A, Ardigo M, Simonitsch I, Ferrara G, Kerl H, Cerroni L. The spectrum of cutaneous lymphomas in patients less than 20 years of age. Pediatr Dermatol. 2004; 21:525-533.
5. Kempf W, Kazakov DV, Belousova IE, Mitteldorf C, Kerl K. Paediatric cutaneous lymphomas: A review and comparison with adult counterparts. J Eur Acad Dermatol Venereol. 2015; 29:1696-
1709.
6. Katalin Ferenczi MD, Hanspaul S. Makkar MD, FRCPC. Cutaneous Lymphoma: Kids are not just little people. Clinics in Dermatology. 2016 Nov - Dec;34(6):749-759.
7. Laws PM, Shear NH, Pope E. Childhood Mycosis Fungoides: Experience of 28 Patients and Response to Phototherapy. Pediatric Dermatology. 2014 Jul-Aug;31(4):459-64.
8. Hodak E, Amitay-Laish I, Feinmesser M, Davidovici B, David M, Zvulunov A, Pavlotsky F, Yaniv I, Avrahami G, Ben-Amitai D. Juvenile mycosis fungoides: Cutaneous T-cell lymphoma with frequent follicular involvement. Journal of the American Academy of Dermatology. 2014; 70:993-1001.
9. Clark RA, Chong B, Mirchandani N, Brinster NK, Yamanaka K, Dowgiert RK, Kupper TS. The vast majority of CLA+ T cells are resident in normal skin. J Immunol. 2006; 176:4431-4439.
10. Watanabe R, Gehad A, Yang C, Scott LL, Teague JE, Schlapbach C, Elco CP, Huang V, Matos TR, Kupper TS, Clark RA. Human skin is protected by four functionally and phenotypically discrete populations of resident and recirculating memory T cells. Science Translational Medicine. 2015 Mar 18;7(279):279ra39.
11. Mackay LK, Rahimpour A, Ma JZ, Collins N, Stock AT, Hafon ML, Vega-Ramos J, Lauzurica P, Mueller SN, Stefanovic T, Tscharke DC, Heath WR, Inouye M, Carbone FR, Gebhardt T. The
developmental pathway for CD103 (+) CD8+ tissue-resident memory T cells of skin. Nat Immunol. 2013; 14:1294-1301.
12. Willemze R. Cutaneous lymphomas with a panniculitic presentation; Seminars in Diagnostic Pathology. 2017 Jan;34(1):36-43.
13. González CL, Medeiros LJ, Braziel RM, Jaffe ES. T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome. Am J Surg Pathol. 1991; 15:17-27.
14. Tan ES, Tang MB, Tan SH: Retrospective 5-year review of 131 patients with mycosis fungoides and Sezary syndrome seen at the National Skin Centre, Singapore. The Australasian Journal of Dermatology. 2006, 47:248-252.
15. Crowley JJ, Nikko A, Varghese A, Hoppe RT, Kim YH. Mycosis fungoides in young patients: clinical characteristics and outcome. Journal of the American Academy of Dermatology. 1998, 38(5 Pt 1):696-701.
16. Wood GS, Tung RM, Haeffner AC et al. Detection of clonal T-cell receptor gamma gene rearrangements in early mycosis fungoides/Sézary syndrome by polymerase chain reaction and denaturing gradient gel electrophoresis (PCR/DGGE). J Invest Dermatol. 1994; 103:34-41.
17. Muche JM, Lukowsky A, Asadullah K, Gellrich S,Sterry W. Demonstration of frequent occurrence of clonal T cells in the peripheral blood of patients with primary cutaneous T-cell lymphoma. Blood. 1997; 90:1636-1642.
18. Nanda A, AlSaleh QA, Al-Ajmi H, Al-Sabah H, Elkashlan M, Al-Shemmari S, Demierre MF. Mycosis fungoides in Arab children and adolescents: a report of 36 patients from Kuwait. Pediatric Dermatology. 2010, 27:607-613.
19. Yazganoglu KD, Topkarci Z, Buyukbabani N, Baykal C. Childhood mycosis fungoides: a report of 20 cases from Turkey. Journal of the European Academy of Dermatology and Venereology:
JEADV. 2013, 27:295-300.
20. Kim ST, Sim HJ, Jeon YS, Lee JW, Roh HJ, Choi SY, Kim YJ, Suh KS. Clinicopathological features and T-cell receptor gene rearrangement findings of mycosis fungoides in patients younger than age 20 years. The Journal of Dermatology; 2009, 36:392-402.
21. Boccara O, Blanche S, de Prost Y, Brousse N, Bodemer C, Fraitag S. Cutaneous hematologic disorders in children. Pediatric Blood & Cancer. 2012, 58:226-232.
22. Pope E, Weitzman S, Ngan B, Walsh S, Morel K, Williams J, Stein S, Garzon M, Knobler E, Lieber C et al. Mycosis fungoides in the pediatric population: report from an international Childhood Registry of Cutaneous Lymphoma. Journal of Cutaneous Medicine and Surgery. 2010 JanFeb;14(1):1-6.
23. Fink-Puches R, Chott A, Ardigo M, Simonitsch I, Ferrara G, Kerl H, Cerroni L. The spectrum of cutaneous lymphomas in patients less than 20 years
of age. Pediatric Dermatology. 2004, 21:525-533.
24. Ben-Amitai D, Michael D, Feinmesser M, Hodak E. Juvenile mycosis fungoides diagnosed before 18 years of age. Acta Dermato-Venereologica. 2003, 83:451-456.
25. Tan E, Tay YK, Giam YC. Profile and outcome of childhood mycosis fungoides in Singapore. Pediatric Dermatology. 2000, 17:352-356.
26. Quaglino P, Zaccagna A, Verrone A, Dardano F, Bernengo MG: Mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the
literature and study of the clinical course. Dermatology. 1999, 199:8-14.
27. Agar NS, Wedgeworth E, Crichton S, Mitchell TJ, Cox M, Ferreira S, Robson A, Calonje E, Stefanato CM, Wain EM et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organization for Research and Treatment of Cancer staging proposal. Journal of Clinical Oncology.
2010, 28(31):4730-4739.
28. Ai WZ, Keegan TH, Press DJ, Yang J, Pincus LB, Kim YH, Chang ET. Outcomes After Diagnosis of Mycosis Fungoides and Sézary Syndrome Before 30 Years of Age: A Population Based Study. JAMA Dermatology; 2014 Jul;150(7):709-15.
29. Kadin ME. Pathobiology of CD30+ cutaneous T-cell lymphomas. Journal of Cutaneous Pathology. 2006, 33 Suppl 1:10-17.
30. Tomaszewski MM, Moad JC, Lupton GP. Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma in childhood. Journal of the American Academy of Dermatology. 1999, 40(5 Pt 2):857-861.
31. Kumar S, Pittaluga S, Raffeld M, Guerrera M, Seibel NL, Jaffe ES: Primary cutaneous CD30-positive anaplastic large cell lymphoma in childhood: report of 4 cases and review of the literature. Pediatric and Developmental Pathology. 2005, 8:52-60.
32. Oschlies I, Lisfeld J, Lamant L, Nakazawa A, d’Amore ES, Hansson U, Hebeda K, SimonitschKlupp I, Maldyk J, Mullauer L et al. ALK-positive anaplastic large cell lymphoma limited to the skin: clinical, histopathological and molecular analysis of 6 pediatric cases. A report from the ALCL99 study. Haematologica. 2013, 98:50-56.
33. Kempf W, Pfaltz K, Vermeer MH, Cozzio A, Ortiz-Romero PL, Bagot M, Olsen E, Kim YH, Dummer R, Pimpinelli N 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.
2. Wilcox, RA. Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk stratification, and management. Am J Hematol. 2016 January; 91(1): 151-165.
3. Pope E,Weitzman S, Ngan B,Walsh S,Morel K,Williams J, Stein S, Garzon M, Knobler E, Lieber C, Turchan. K,Wargon O, Tsuchiya A. Mycosis fungoides in the pediatric population: Report from
an international Childhood Registry of Cutaneous Lymphoma. J Cutan Med Surg. 2010; 14:1-6.
4. Fink-Puches R, Chott A, Ardigo M, Simonitsch I, Ferrara G, Kerl H, Cerroni L. The spectrum of cutaneous lymphomas in patients less than 20 years of age. Pediatr Dermatol. 2004; 21:525-533.
5. Kempf W, Kazakov DV, Belousova IE, Mitteldorf C, Kerl K. Paediatric cutaneous lymphomas: A review and comparison with adult counterparts. J Eur Acad Dermatol Venereol. 2015; 29:1696-
1709.
6. Katalin Ferenczi MD, Hanspaul S. Makkar MD, FRCPC. Cutaneous Lymphoma: Kids are not just little people. Clinics in Dermatology. 2016 Nov - Dec;34(6):749-759.
7. Laws PM, Shear NH, Pope E. Childhood Mycosis Fungoides: Experience of 28 Patients and Response to Phototherapy. Pediatric Dermatology. 2014 Jul-Aug;31(4):459-64.
8. Hodak E, Amitay-Laish I, Feinmesser M, Davidovici B, David M, Zvulunov A, Pavlotsky F, Yaniv I, Avrahami G, Ben-Amitai D. Juvenile mycosis fungoides: Cutaneous T-cell lymphoma with frequent follicular involvement. Journal of the American Academy of Dermatology. 2014; 70:993-1001.
9. Clark RA, Chong B, Mirchandani N, Brinster NK, Yamanaka K, Dowgiert RK, Kupper TS. The vast majority of CLA+ T cells are resident in normal skin. J Immunol. 2006; 176:4431-4439.
10. Watanabe R, Gehad A, Yang C, Scott LL, Teague JE, Schlapbach C, Elco CP, Huang V, Matos TR, Kupper TS, Clark RA. Human skin is protected by four functionally and phenotypically discrete populations of resident and recirculating memory T cells. Science Translational Medicine. 2015 Mar 18;7(279):279ra39.
11. Mackay LK, Rahimpour A, Ma JZ, Collins N, Stock AT, Hafon ML, Vega-Ramos J, Lauzurica P, Mueller SN, Stefanovic T, Tscharke DC, Heath WR, Inouye M, Carbone FR, Gebhardt T. The
developmental pathway for CD103 (+) CD8+ tissue-resident memory T cells of skin. Nat Immunol. 2013; 14:1294-1301.
12. Willemze R. Cutaneous lymphomas with a panniculitic presentation; Seminars in Diagnostic Pathology. 2017 Jan;34(1):36-43.
13. González CL, Medeiros LJ, Braziel RM, Jaffe ES. T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome. Am J Surg Pathol. 1991; 15:17-27.
14. Tan ES, Tang MB, Tan SH: Retrospective 5-year review of 131 patients with mycosis fungoides and Sezary syndrome seen at the National Skin Centre, Singapore. The Australasian Journal of Dermatology. 2006, 47:248-252.
15. Crowley JJ, Nikko A, Varghese A, Hoppe RT, Kim YH. Mycosis fungoides in young patients: clinical characteristics and outcome. Journal of the American Academy of Dermatology. 1998, 38(5 Pt 1):696-701.
16. Wood GS, Tung RM, Haeffner AC et al. Detection of clonal T-cell receptor gamma gene rearrangements in early mycosis fungoides/Sézary syndrome by polymerase chain reaction and denaturing gradient gel electrophoresis (PCR/DGGE). J Invest Dermatol. 1994; 103:34-41.
17. Muche JM, Lukowsky A, Asadullah K, Gellrich S,Sterry W. Demonstration of frequent occurrence of clonal T cells in the peripheral blood of patients with primary cutaneous T-cell lymphoma. Blood. 1997; 90:1636-1642.
18. Nanda A, AlSaleh QA, Al-Ajmi H, Al-Sabah H, Elkashlan M, Al-Shemmari S, Demierre MF. Mycosis fungoides in Arab children and adolescents: a report of 36 patients from Kuwait. Pediatric Dermatology. 2010, 27:607-613.
19. Yazganoglu KD, Topkarci Z, Buyukbabani N, Baykal C. Childhood mycosis fungoides: a report of 20 cases from Turkey. Journal of the European Academy of Dermatology and Venereology:
JEADV. 2013, 27:295-300.
20. Kim ST, Sim HJ, Jeon YS, Lee JW, Roh HJ, Choi SY, Kim YJ, Suh KS. Clinicopathological features and T-cell receptor gene rearrangement findings of mycosis fungoides in patients younger than age 20 years. The Journal of Dermatology; 2009, 36:392-402.
21. Boccara O, Blanche S, de Prost Y, Brousse N, Bodemer C, Fraitag S. Cutaneous hematologic disorders in children. Pediatric Blood & Cancer. 2012, 58:226-232.
22. Pope E, Weitzman S, Ngan B, Walsh S, Morel K, Williams J, Stein S, Garzon M, Knobler E, Lieber C et al. Mycosis fungoides in the pediatric population: report from an international Childhood Registry of Cutaneous Lymphoma. Journal of Cutaneous Medicine and Surgery. 2010 JanFeb;14(1):1-6.
23. Fink-Puches R, Chott A, Ardigo M, Simonitsch I, Ferrara G, Kerl H, Cerroni L. The spectrum of cutaneous lymphomas in patients less than 20 years
of age. Pediatric Dermatology. 2004, 21:525-533.
24. Ben-Amitai D, Michael D, Feinmesser M, Hodak E. Juvenile mycosis fungoides diagnosed before 18 years of age. Acta Dermato-Venereologica. 2003, 83:451-456.
25. Tan E, Tay YK, Giam YC. Profile and outcome of childhood mycosis fungoides in Singapore. Pediatric Dermatology. 2000, 17:352-356.
26. Quaglino P, Zaccagna A, Verrone A, Dardano F, Bernengo MG: Mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the
literature and study of the clinical course. Dermatology. 1999, 199:8-14.
27. Agar NS, Wedgeworth E, Crichton S, Mitchell TJ, Cox M, Ferreira S, Robson A, Calonje E, Stefanato CM, Wain EM et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organization for Research and Treatment of Cancer staging proposal. Journal of Clinical Oncology.
2010, 28(31):4730-4739.
28. Ai WZ, Keegan TH, Press DJ, Yang J, Pincus LB, Kim YH, Chang ET. Outcomes After Diagnosis of Mycosis Fungoides and Sézary Syndrome Before 30 Years of Age: A Population Based Study. JAMA Dermatology; 2014 Jul;150(7):709-15.
29. Kadin ME. Pathobiology of CD30+ cutaneous T-cell lymphomas. Journal of Cutaneous Pathology. 2006, 33 Suppl 1:10-17.
30. Tomaszewski MM, Moad JC, Lupton GP. Primary cutaneous Ki-1(CD30) positive anaplastic large cell lymphoma in childhood. Journal of the American Academy of Dermatology. 1999, 40(5 Pt 2):857-861.
31. Kumar S, Pittaluga S, Raffeld M, Guerrera M, Seibel NL, Jaffe ES: Primary cutaneous CD30-positive anaplastic large cell lymphoma in childhood: report of 4 cases and review of the literature. Pediatric and Developmental Pathology. 2005, 8:52-60.
32. Oschlies I, Lisfeld J, Lamant L, Nakazawa A, d’Amore ES, Hansson U, Hebeda K, SimonitschKlupp I, Maldyk J, Mullauer L et al. ALK-positive anaplastic large cell lymphoma limited to the skin: clinical, histopathological and molecular analysis of 6 pediatric cases. A report from the ALCL99 study. Haematologica. 2013, 98:50-56.
33. Kempf W, Pfaltz K, Vermeer MH, Cozzio A, Ortiz-Romero PL, Bagot M, Olsen E, Kim YH, Dummer R, Pimpinelli N 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.
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