Diagnosis and treatment of skin T-cell lymphoma undergoing transformation in lymphosarcoma


如何引用文章

全文:

详细

Aim. To define complex of parameters characterizing transformation of skin T-cell tumors in lymphosarcoma; to show specific treatment of patients with this transformation.
Material and methods. Of 57 patients with primary T-cell lymphomas of the skin (mycosis fungoides, Sezary's disease), we studied 12 patients with transformation of the process into lymphosarcoma by clinical, histological, moleculobiological and immunophenotypical parameters.
Results. We found that transformation of T-cell lymphoma into lymphosarcoma occurred in different time from the disease onset (2-12 years). In patients with mycosis fungoides (MF) the transformation was local while in those with Sezary's disease (SD) transformation of the tumor clone was determined by appearance of peripheral blood tumor cells rejuvenation. Morphological alterations were accompanied with immunomorphological parameters of progression. Most significant of them were high expression of the proliferative activity marker Ki-67 (10-70%), enhancement of activation (CD30, CD25), loss of some linear T-cell markers. Treatment of lymphosarcoma arising on the background of lingering MF or SD may combine two types of antitumor treatment - intensive and supporting because of coexistence of different clones of one tumor.
Conclusion. Verification of skin T-cell lymphoma diagnosis and its transformation into lymphosarcoma must be based on the evidence from a number of examinations: histological, immunophenotyping, moleculobiological and clinical. Among criteria of the transformation, markers of lymphoproliferative activation are of great importance.

参考

  1. Bekkenk M. W., Geelen F. A. M. J., van Voorst Vader P. C. Primary and secondary cutaneous CD30+ lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood 2000; 95: 3653-3661.
  2. Burg G., Kempf W., Cozzio A. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J. Cutan. Pathol. 2005; 32: 647-674.
  3. Jaffe E. S. Pathobiology of peripheral T-cell lymphomas. Am. Soc. Hematology Educ Program 2006: 317-322.
  4. Rosen S. T., Querfeld Chr. Primary cutaneous T-cell lymphomas. In: Hematology 2006. 323.
  5. de Bruin P. C., Beljaards R. C., van Heerde P. et al. Differences in clinical behaviour and immunophenotype between primary cutaneous and primary nodal anaplastic large cell lymphoma of T- or null cell phenotype. Histopathology 1993; 23: 127.
  6. Perkins S. L., Kjeldsberg C. R. Immunophenotyping of lymphomas and leukemias in paraffin-embedded tissues. Am. J. Clin. Pathol. 1993; Apr. 99(4): 362-373.
  7. Воробьев А. И. Опухолевая прогрессия в патогенезе лейкозов. Клин. мед. 1970; 4: 52-69.
  8. Bogen S. A., Pellcy D., Charif M. et al. Immunophenotypic identification of Sezary cells in peripheral blood. Am. J. Clin. Pathol. 1996; 106: 739-748.
  9. Massone C., Kodama K., Kerl H., Cerroni L. Histopathologic features of early (patch) lesions of mycosis fungoides: a morphologic study on 745 biopsy specimens from 427 patients. Am. J. Surg. Pathol. 2005; 29: 550-560.
  10. Mao X., Lillington D., Scarisbrick J. J. et al. Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sezary syndrome and mucosis fungoides. Br. J. Dermatol. 2002; 147: 464.
  11. Zhang Q., Raghunath P. N., Vonderheid F. et al. Lack of phosphotyrosine phosphatase SHP-1 expression in malignant T-cell lymphoma cells results from methylation of the SHOP-1 promoter. Am. J. Pathol. 2000; 157: 1137.
  12. Mao X., McElwaine S. Functional copy number changes in Sézary syndrome: toward an integrated molecular cytogenetic map III. Cancer Genet. Cytogenet. 2008; 185(2): 86-94.
  13. Виноградова Ю. Е., Гилязитдинова Е. А., Замулаева И. А. и др. Клинико-морфологические особенности болезни Сезари и грибовидного микоза. Тер. арх. 2005; 77(10): 61-65.
  14. Marti R. M., Estrach T., Reverter J. C., Mascaro J. M. Prognostic clinicopathologic factors in cutaneous T-cell lymphoma. Arch. Dermatol. 1991; 127: 1511-1516.
  15. Kim E. J., Hess S., Richardson S. K. et al. Immunopathogenesis and therapy of cutaneous T cell lymphoma. J. Clin. Invest. 2005; 115(4): 798-812.
  16. Berthelot C., Rivera A., Duvic M. Skin directed therapy for mycosis fungoides: a review. J. Drugs Dermatol. 2008; 7(7): 655-666.

补充文件

附件文件
动作
1. JATS XML
下载

版权所有 © Consilium Medicum, 2009

Creative Commons License
此作品已接受知识共享署名-非商业性使用-相同方式共享 4.0国际许可协议的许可。
 

Address of the Editorial Office:

  • Alabyan Street, 13/1, Moscow, 127055, Russian Federation

Correspondence address:

  • Alabyan Street, 13/1, Moscow, 127055, Russian Federation

Managing Editor:

  • Tel.: +7 (926) 905-41-26
  • E-mail: e.gorbacheva@ter-arkhiv.ru

 

 © 2018-2021 "Consilium Medicum" Publishing house


##common.cookie##