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Cutaneous Non-MF T-Cell and NK-Cell Lymphoproliferative Disorders

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Molecular Diagnostics in Dermatology and Dermatopathology

Part of the book series: Current Clinical Pathology ((CCPATH))

Abstract

Southern blot analysis (SBA) and polymerase chain reaction (PCR)-based testing for monoclonal T-cell receptor gene rearrangements (TCR-GRs), fluorescence in situ hybridization (FISH) for t(2;5)(p23;q35), in situ hybridization for Epstein-Barr virus-encoded mRNA (EBER-ISH), and SBA for human T-cell lymphotropic virus type I (HTLV-I) provirus integration are the most common molecular techniques used for the diagnosis of primary cutaneous non-mycosis fungoides (MF) T-cell and natural killer (NK)-cell lymphoproliferative disorders.

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References

  1. McCarthy KP, Sloane JP, Kabarowski JH, et al. A simplified method of detection of clonal rearrangements of the T-cell receptor-gamma chain gene. Diagn Mol Pathol. 1992;1:173–9.

    PubMed  CAS  Google Scholar 

  2. van Dongen JJ, Langerak AW, Bruggemann M, et al. Design and standardization of PCR primers and protocols for detection of clonal immunoglobulin and T-cell receptor gene recombinations in suspect lymphoproliferations: report of the BIOMED-2 Concerted Action BMH4-CT98-3936. Leukemia. 2003;17:2257–317.

    Article  PubMed  Google Scholar 

  3. Langerak AW. Undersized, oversized? It is not one-size-fits-all in lymphoid clonality detection. Leuk Res. 2008;32:203–4.

    Article  PubMed  Google Scholar 

  4. Vargas RL, Felgar RE, Rothberg PG. Detection of clonality in lymphoproliferations using PCR of the antigen receptor genes: does size matter? Leuk Res. 2008;32:335–8.

    Article  PubMed  CAS  Google Scholar 

  5. Liao YL, Chang ST, Kuo SY, et al. Angioimmunoblastic T-cell lymphoma of cytotoxic T-cells phenotype containing a large B-cell proliferation with an undersized B-cell clonal product. Appl Immunohistochem Mol Morphol. 2010;18:185–89.

    Google Scholar 

  6. Chuang SS, Chang ST, Chuang WY, et al. NK-cell lineage predicts poor survival in primary intestinal NK-cell and T-cell lymphomas. Am J Surg Pathol. 2009;33:1230–40.

    Article  PubMed  Google Scholar 

  7. Ohshima K, Suzumiya J, Sato K, et al. Nodal T-cell lymphoma in an HTLV-I-endemic area: proviral HTLV-I DNA, histological classification and clinical evaluation. Br J Haematol. 1998;101:703–11.

    Article  PubMed  CAS  Google Scholar 

  8. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–85.

    Article  PubMed  CAS  Google Scholar 

  9. Su IJ, Wu YC, Chen YC, et al. Cutaneous manifestations of postthymic T cell malignancies: description of five clinicopathologic subtypes. J Am Acad Dermatol. 1990;23:653–62.

    Article  PubMed  CAS  Google Scholar 

  10. Nagasawa T, Miwa H, Nakatsuka S, et al. Characteristics of cutaneous lymphomas in Osaka, Japan (1988–1999) based on the European Organization for research and treatment of cancer classification. Am J Dermatopathol. 2000;22:510–4.

    Article  PubMed  CAS  Google Scholar 

  11. Lee MW. Characteristics of cutaneous lymphomas in Korea. Clin Exp Dermatol. 2003;28:639–46.

    Article  PubMed  Google Scholar 

  12. Yasukawa K, Kato N, Kodama K, et al. The spectrum of cutaneous lymphomas in Japan: a study of 62 cases based on the World Health Organization Classification. J Cutan Pathol. 2006;33:487–91.

    Article  PubMed  Google Scholar 

  13. Liao JB, Chuang SS, Chen HC, et al. Clinicopathologic analysis of cutaneous lymphoma in Taiwan: a high frequency of extranodal NK/T-cell lymphoma, nasal type, with an extremely poor prognosis. Arch Pathol Lab Med. 2010;134:996–1002.

    Google Scholar 

  14. Burg G, Kempf W, Cozzio A, et al. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J Cutan Pathol. 2005;32:647–74.

    Article  PubMed  Google Scholar 

  15. Burg G, Jaffe ES, Kempf W, et al. Haematolymphoid tumours. In: Leboit PE, Burg G, Weedon D, Sarasin A, editors. World Health Organization classification of tumours pathology and genetics skin tumours. Lyon: IARC; 2006. p. 165–228.

    Google Scholar 

  16. Bekkenk MW, Geelen FA, van Voorst Vader PC, et al. 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;95:3653–61.

    PubMed  CAS  Google Scholar 

  17. Ralfkiaer E, Willemze R, Pauli M, et al. Primary cutaneous CD30-positive lymphoproliferative disorders. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2008. p. 300–1.

    Google Scholar 

  18. Weiss LM, Wood GS, Trela M, et al. Clonal T-cell populations in lymphomatoid papulosis. Evidence of a lymphoproliferative origin for a clinically benign disease. N Engl J Med. 1986;315:475–9.

    Article  PubMed  CAS  Google Scholar 

  19. Chott A, Vonderheid EC, Olbricht S, et al. The dominant T cell clone is present in multiple regressing skin lesions and associated T cell lymphomas of patients with lymphomatoid papulosis. J Invest Dermatol. 1996;106:696–700.

    Article  PubMed  CAS  Google Scholar 

  20. Steinhoff M, Hummel M, Anagnostopoulos I, et al. Single-cell analysis of CD30+ cells in lymphomatoid papulosis demonstrates a common clonal T-cell origin. Blood. 2002;100:578–84.

    Article  PubMed  CAS  Google Scholar 

  21. Zackheim HS, Jones C, Leboit PE, et al. Lymphomatoid papulosis associated with mycosis fungoides: a study of 21 patients including analyses for clonality. J Am Acad Dermatol. 2003;49:620–3.

    Article  PubMed  Google Scholar 

  22. Gallardo F, Costa C, Bellosillo B, et al. Lymphomatoid papulosis associated with mycosis fungoides: clinicopathological and molecular studies of 12 cases. Acta Derm Venereol. 2004;84:463–8.

    Article  PubMed  Google Scholar 

  23. Liu HL, Hoppe RT, Kohler S, et al. CD30+ cutaneous lymphoproliferative disorders: the Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. J Am Acad Dermatol. 2003;49:1049–58.

    Article  PubMed  Google Scholar 

  24. Willemze R, Beljaards RC. Spectrum of primary cutaneous CD30 (Ki-1)-positive lymphoproliferative disorders. A proposal for classification and guidelines for management and treatment. J Am Acad Dermatol. 1993;28:973–80.

    Article  PubMed  CAS  Google Scholar 

  25. Greisser J, Palmedo G, Sander C, et al. Detection of clonal rearrangement of T-cell receptor genes in the diagnosis of primary cutaneous CD30 lymphoproliferative disorders. J Cutan Pathol. 2006;33:711–5.

    Article  PubMed  Google Scholar 

  26. Kohler S, Jones CD, Warnke RA, et al. PCR-heteroduplex analysis of T-cell receptor gamma gene rearrangement in paraffin-embedded skin biopsies. Am J Dermatopathol. 2000;22:321–7.

    Article  PubMed  CAS  Google Scholar 

  27. Kong YY, Dai B, Kong JC, et al. Neutrophil/eosinophil-rich type of primary cutaneous anaplastic large cell lymphoma: a clinicopathological, immunophenotypic and molecular study of nine cases. Histopathology. 2009;55:189–96.

    Article  PubMed  Google Scholar 

  28. Chang ST, Chuang SS, Wang YH. Polyostotic primary bone lymphoma with progression limited in the skeleton: identical clonal origin of the initial and progressed tumors and a late relapse by VDJ rearrangement analysis. Leuk Lymphoma. 2006;47:1144–8.

    Article  PubMed  Google Scholar 

  29. Chuang SS, Liu H, Huang Y, et al. Primary cerebral diffuse large B-cell lymphoma relapsed solely in the skin with the same clonal origin. Appl Immunohistochem Mol Morphol. 2009;17:77–81.

    Article  PubMed  Google Scholar 

  30. Chen YF, Li YC, Chen LM, et al. Primary cutaneous diffuse large B-cell lymphoma relapsed solely as a huge lung tumor mimicking a primary pulmonary lymphoma. Int J Hematol. 2010;91:112–16.

    Google Scholar 

  31. Chuang SS, Hsieh YC, Ye H, et al. Lymphohistiocytic anaplastic large cell lymphoma involving skin: a diagnostic challenge. Pathol Res Pract. 2009;205:283–7.

    Article  PubMed  Google Scholar 

  32. Gonzalez CL, Medeiros LJ, Braziel RM, et al. T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome. Am J Surg Pathol. 1991;15:17–27.

    Article  PubMed  CAS  Google Scholar 

  33. Toro JR, Liewehr DJ, Pabby N, et al. Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood. 2003;101:3407–12.

    Article  PubMed  CAS  Google Scholar 

  34. Massone C, Chott A, Metze D, et al. Subcutaneous, blastic natural killer (NK), NK/T-cell, and other cytotoxic lymphomas of the skin: a morphologic, immunophenotypic, and molecular study of 50 patients. Am J Surg Pathol. 2004;28:719–35.

    Article  PubMed  Google Scholar 

  35. Willemze R, Jansen PM, Cerroni L, et al. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood. 2008;111:838–45.

    Article  PubMed  CAS  Google Scholar 

  36. Magro CM, Crowson AN, Kovatich AJ, et al. Lupus profundus, indeterminate lymphocytic lobular panniculitis and subcutaneous T-cell lymphoma: a spectrum of subcuticular T-cell lymphoid dyscrasia. J Cutan Pathol. 2001;28:235–47.

    Article  PubMed  CAS  Google Scholar 

  37. Massone C, Kodama K, Salmhofer W, et al. Lupus erythematosus panniculitis (lupus profundus): clinical, histopathological, and molecular analysis of nine cases. J Cutan Pathol. 2005;32:396–404.

    Article  PubMed  Google Scholar 

  38. Chen RL, Hsu YH, Ueda I, et al. Cytophagic histiocytic panniculitis with fatal haemophagocytic lymphohistiocytosis in a paediatric patient with perforin gene mutation. J Clin Pathol. 2007;60:1168–9.

    Article  PubMed  CAS  Google Scholar 

  39. Kong YY, Dai B, Kong JC, et al. Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathologic, immunophenotypic, and molecular study of 22 Asian cases according to WHO-EORTC classification. Am J Surg Pathol. 2008;32:1495–502.

    Article  PubMed  Google Scholar 

  40. Gaulard P, Berti E, Willemze R, et al. Primary cutaneous peripheral T-cell lymphoma, rare subtypes. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2008. p. 302–5.

    Google Scholar 

  41. Toro JR, Beaty M, Sorbara L, et al. Gamma delta T-cell lymphoma of the skin: a clinical, microscopic, and molecular study. Arch Dermatol. 2000;136:1024–32.

    Article  PubMed  CAS  Google Scholar 

  42. Przybylski GK, Wu H, Macon WR, et al. Hepatosplenic and subcutaneous panniculitis-like gamma/delta T cell lymphomas are derived from different Vdelta subsets of gamma/delta T lymphocytes. J Mol Diagn. 2000;2:11–9.

    Article  PubMed  CAS  Google Scholar 

  43. Berti E, Tomasini D, Vermeer MH, et al. Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. A distinct clinicopathological entity with an aggressive clinical behavior. Am J Pathol. 1999;155:483–92.

    Article  PubMed  CAS  Google Scholar 

  44. Santucci M, Pimpinelli N, Massi D, et al. Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop. Cancer. 2003;97:610–27.

    Article  PubMed  Google Scholar 

  45. Kim SK, Kim YC, Kang HY. Primary cutaneous aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma with atypical presentation. J Dermatol. 2006;33:632–4.

    Article  PubMed  Google Scholar 

  46. Fika Z, Karkos PD, Badran K, et al. Primary cutaneous aggressive epidermotropic CD8 positive cytotoxic T-cell lymphoma of the ear. J Laryngol Otol. 2007;121:503–5.

    Article  PubMed  CAS  Google Scholar 

  47. Introcaso CE, Kim EJ, Gardner J, et al. CD8+ epidermotropic cytotoxic T-cell lymphoma with peripheral blood and central nervous system involvement. Arch Dermatol. 2008;144:1027–9.

    Article  PubMed  Google Scholar 

  48. Hagiwara M, Takata K, Shimoyama Y, et al. Primary cutaneous T-cell lymphoma of unspecified type with cytotoxic phenotype: clinicopathological analysis of 27 patients. Cancer Sci. 2009;100:33–41.

    Article  PubMed  CAS  Google Scholar 

  49. Beljaards RC, Meijer CJ, Van der Putte SC, et al. Primary cutaneous T-cell lymphoma: clinicopathological features and prognostic parameters of 35 cases other than mycosis fungoides and CD30-positive large cell lymphoma. J Pathol. 1994;172:53–60.

    Article  PubMed  CAS  Google Scholar 

  50. Garcia-Herrera A, Colomo L, Camos M, 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.

    Article  PubMed  Google Scholar 

  51. Friedmann D, Wechsler J, Delfau MH, et al. Primary cutaneous pleomorphic small T-cell lymphoma. A review of 11 cases. The French Study Group on Cutaneous Lymphomas. Arch Dermatol. 1995;131:1009–15.

    Article  PubMed  CAS  Google Scholar 

  52. Grogg KL, Jung S, Erickson LA, et al. 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.

    Article  PubMed  CAS  Google Scholar 

  53. Rodriguez Pinilla SM, Roncador G, Rodriguez-Peralto JL, 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.

    Article  PubMed  Google Scholar 

  54. Cerroni L. Lymphoproliferative lesions of the skin. J Clin Pathol. 2006;59:813–26.

    Article  PubMed  CAS  Google Scholar 

  55. Lin MT, Chang HM, Huang CJ, et al. Massive expansion of EBV+ monoclonal T cells with CD5 down regulation in EBV-associated haemophagocytic lymphohistiocytosis. J Clin Pathol. 2007;60:101–3.

    Article  PubMed  CAS  Google Scholar 

  56. Ohshima K, Jaffe ES, Kikuchi M. Adult T-cell leukemia/lymphoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2008. p. 281–4.

    Google Scholar 

  57. Shimoyama M. Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984–87). Br J Haematol. 1991;79:428–37.

    Article  PubMed  CAS  Google Scholar 

  58. Yamaguchi T, Ohshima K, Karube K, et al. Clinicopathological features of cutaneous lesions of adult T-cell leukaemia/lymphoma. Br J Dermatol. 2005;152:76–81.

    Article  PubMed  CAS  Google Scholar 

  59. Takahashi K, Tanaka T, Fujita M, et al. Cutaneous-type adult T-cell leukemia/lymphoma. A unique clinical feature with monoclonal T-cell proliferation detected by Southern blot analysis. Arch Dermatol. 1988;124:399–404.

    Article  PubMed  CAS  Google Scholar 

  60. Yagi H, Takigawa M, Hashizume H. Cutaneous type of adult T cell leukemia/lymphoma: a new entity among cutaneous lymphomas. J Dermatol. 2003;30:641–3.

    PubMed  Google Scholar 

  61. Amano M, Kurokawa M, Ogata K, et al. New entity, definition and diagnostic criteria of cutaneous adult T-cell leukemia/lymphoma: human T-lymphotropic virus type 1 proviral DNA load can distinguish between cutaneous and smoldering types. J Dermatol. 2008;35:270–5.

    Article  PubMed  CAS  Google Scholar 

  62. Tsukasaki K, Hermine O, Bazarbachi A, et al. Definition, prognostic factors, treatment, and response criteria of adult T-cell leukemia-lymphoma: a proposal from an international consensus meeting. J Clin Oncol. 2009;27:453–9.

    Article  PubMed  Google Scholar 

  63. Shimamoto Y, Suga K, Shibata K, et al. Clinical importance of extraordinary integration patterns of human T-cell lymphotropic virus type I proviral DNA in adult T-cell leukemia/lymphoma. Blood. 1994;84:853–8.

    PubMed  CAS  Google Scholar 

  64. Tsukasaki K, Tsushima H, Yamamura M, et al. Integration patterns of HTLV-I provirus in relation to the clinical course of ATL: frequent clonal change at crisis from indolent disease. Blood. 1997;89:948–56.

    PubMed  CAS  Google Scholar 

  65. Karube K, Suzumiya J, Okamoto M, et al. Adult T-cell lymphoma/leukemia with angioimmunoblastic T-cell lymphoma-like features: report of 11 cases. Am J Surg Pathol. 2007;31:216–23.

    Article  PubMed  Google Scholar 

  66. Chuang SS, Ichinohasama R, Chu JS, Ohshima K. Differential diagnosis of angioimmunoblastic T-cell lymphoma with seropositivity for anti-HTLV antibody from adult T-cell leukemia/lymphoma. Int J Hematol 2010;91:687–91.

    Google Scholar 

  67. Hsieh YC, Chang ST, Huang WT, et al. Adult T-cell leukemia/lymphoma comprising non-floral leukemic cells in Taiwan, a country non-endemic for human T-cell leukemia virus type I. Leuk Lymphoma. 2009;50:1540–2.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Pathology, Chi-Mei Medical Center, Tainan and Taipei Medical University, 901, Chuang Hwa Road, Yung Kang City, Tainan, 710, Taiwan

    Shih-Sung Chuang

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  1. Shih-Sung Chuang
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Correspondence to Shih-Sung Chuang .

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  1. Health Center, Dept. Dermatology, University of Connecticut, Farmington Ave. 263, Farmington, 06030, Connecticut, USA

    Michael J. Murphy

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Chuang, SS. (2011). Cutaneous Non-MF T-Cell and NK-Cell Lymphoproliferative Disorders. In: Murphy, M. (eds) Molecular Diagnostics in Dermatology and Dermatopathology. Current Clinical Pathology. Humana Press. https://doi.org/10.1007/978-1-60761-171-4_11

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  • DOI: https://doi.org/10.1007/978-1-60761-171-4_11

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