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Peripheral Post-Thymic T-Cell Lymphoma

Synonyms: Peripheral T-Cell Lymphoma

Definition

This is an heterogenous group of lymphomas with immunophenotypic and genotypic features of mature (peripheral) T-lymphoid cells. Extranodal presentation is more common; however, primary lymph node presentation is also seen. Some of the subtypes included are angioimmunoblastic like T-cell lymphoma, NK/T cell lymphoma (nasal type), Lennert (lymphoepithelioid) lymphoma and peripheral T-cell lymphoma not otherwise specified (NOS).

Clinical Features

Lennert's Lymphoma

  • Adults
  • Often generalized:
    • stage IV disease at presentation in 74%
  • Sometimes ‘blastic’ transformation into large cell lymphoma14
  • Poor prognosis15,16

Pathogenesis

Histopathology

Lennert's Lymphoma

  • Effacement of architecture by lymphohistiocytic infiltrate
  • Often accompanying:
    • plasma cells
    • eosinophils
    • proliferation of small vessels with plump endothelial cells

Special Stains and Immunohistochemistry

  • All show by definition markers of mature T cell19
  • Most express CD4+/CD8− mature helper phenotype
  • ≈20% express CD4−/CD8+ cytotoxic/suppressor phenotype
  • Rarely CD4−/CD8− or CD4+/CD8+ phenotypes

Paraffin Sections

  • Positive for:
    • CD45RB ≈90% of cases
    • T-cell-associated markers CD45R0, CD43, and CD3 ≈90% of cases
    • CD15 10–15% of cases:
      • a cytoplasmic granular quality generally different from that in Hodgkin's lymphoma20,21

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Peripheral Post-Thymic T-Cell Lymphoma

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Lennert's Lymphoma

  • Hodgkin's lymphoma:
    • misdiagnosis due to:
      • polymorphic infiltrate
      • occasional Reed–Sternberg-like cell
    • key is atypical appearance of small lymphocytes between reactive histiocytes (Fig. 1
      Peripheral T-cell lymphoma with a high content of non-neoplastic histiocytes (Lennert's lymphoma).

      Fig. 1: Peripheral T-cell lymphoma with a high content of non-neoplastic histiocytes (Lennert's lymphoma).

      )
  • Angioimmunoblastic lymphadenopathy

Genetics

References

1 Cabecadas JM, Isaacson PG. Phenotyping of T-cell lymphomas in paraffin sections – which antibodies?. Histopathology. 1991;19:419–424.

2 van Krieken JH, Elwood L, Andrade RE, Jaffe ES, Cossman J, Medeiros LJ. Rearrangement of the T-cell receptor delta chain gene in T-cell lymphomas with a mature phenotype. Am J Pathol. 1991;139:161–168.

3 Ichimura K, Kagami Y, Suzuki R, Kojima M, Yoshino T, Ohshima K, et al. Phenotypic analysis of peripheral T/NK cell lymphoma: study of 408 Japanese cases with special reference to their anatomical sites. Pathol Int. 2003;53:333–344.

4 Burke JS, Butler JJ. Malignant lymphoma with a high content of epithelioid histiocytes (Lennert's lymphoma). Am J Clin Pathol. 1976;66:1–9.

5 Lennert K, Mestdagh J. Lymphogranulomatosen mit konstant hohem Epitheloidzellgehalt. Virchows Arch [A]. 1968;344:1–20.

6 Patsouris E, Noël H, Lennert K. Histological and immunohistological findings in lymphoepithelioid cell lymphoma (Lennert's lymphoma). Am J Surg Pathol. 1988;12:341–350.

7 Pinkus GS, Said JW, Hargreaves H. Malignant lymphoma, T-cell type. A distinct morphologic variant with large multilobulated nuclei, with a report of four cases. Am J Clin Pathol. 1979;72:540–550.

8 Macon WR, Williams ME, Greer JP, Cousar JB. Paracortical nodular T-cell lymphoma. Identification of an unusual variant of peripheral T-cell lymphoma. Am J Surg Pathol. 1995;19:297–303.

9 Ohshima K, Suzumiya J, Kikuchi M. The World Health Organization classification of malignant lymphoma: incidence and clinical prognosis in HTLV-1-endemic area of Fukuoka. Pathol Int. 2002;52:1–12.

10 Su I-J, Wang CH, Cheng A-L, Chen Y-C, Hsieh H-C, Chen C-J, et al. Characterization of the spectrum of post-thymic T-cell malignancies in Taiwan. A clinicopathologic study of HTLV-1-positive and HTLV-1-negative cases. Cancer. 1988;61:2060–2070.

11 Kazakov DV, Kempf W, Michaelis S, Schmid U, Cogliatti S, Dummer R, et al. T-zone lymphoma with cutaneous involvement: a case report and review of the literature. Br J Dermatol. 2002;146:1096–1100.

12 Rudiger T, Ichinohasama R, Ott MM, Muller-Deubert S, Miura I, Ott G, et al. Peripheral T-cell lymphoma with distinct perifollicular growth pattern: a distinct subtype of T-cell lymphoma. Am J Surg Pathol. 2000;24:117–122.

13 Mukai HY, Hasegawa Y, Kojima H, Okoshi Y, Takei N, Yamashita Y, et al. Nodal CD8 positive cytotoxic T-cell lymphoma: a distinct clinicopathological entity. Mod Pathol. 2002;15:1131–1139.

14 Klein MA, Jaffe R, Neiman RS. “Lennert's lymphoma” with transformation to malignant lymphoma, histiocytic type (immunoblastic sarcoma). Am J Clin Pathol. 1977;68:601–605.

15 Kim H, Jacobs C, Warnke RA, Dorfman RF. Malignant lymphoma with a high content of epithelioid histiocytes. A distinct clinicopathologic entity and a form of so-called “Lennert's lymphoma.”. Cancer. 1978;41:620–635.

16 Spier CM, Lippman SM, Miller TP, Grogan TM. Lennert's lymphoma. A clinicopathologic study with emphasis on phenotype and its relationship to survival. Cancer. 1988;61:517–524.

17 Tajima K, Kuroishi T. Estimation of rate of incidence of ATL among ATLV (HTLV-I) carriers in Kyushu, Japan. Jpn J Clin Oncol. 1985;15:423–430.

18 Jones JF, Shurin S, Abramowsky C, Tubbs RR, Sciotto CG, Wahl R, et al. T-cell lymphomas containing Epstein–Barr viral DNA in patients with chronic Epstein–Barr virus infections. N Engl J Med. 1988;318:733–741.

19 Borowitz MJ, Reichert TA, Brynes RK, Cousar JB, Whitcomb CC, Collins RD, et al. The phenotypic diversity of peripheral T-cell lymphomas. The Southeastern Cancer Study Group experience. Hum Pathol. 1986;17:567–574.

20 Sheibani K, Battifora H, Burke JS, Rappaport H. Leu-M1 antigen in human neoplasms. An immunohistologic study of 400 cases. Am J Surg Pathol. 1986;10:227–236.

21 Wieczorek R, Burke JS, Knowles DM. Leu-M1 antigen expression in T-cell neoplasia. Am J Pathol. 1985;121:374–380.

22 Picker LJ, Brenner MB, Weiss LM, Smith SD, Warnke RA. Discordant expression of CD3 and T-cell receptor β-chain antigens in T-lineage lymphomas. Am J Pathol. 1987;129:434–440.

23 Sheibani K, Wu A, Ben-Ezra J, Stroup R, Rappaport H, Winberg C. Rearrangement of kappa-chain and T-cell receptor b-chain genes in malignant lymphomas of “T-cell” phenotype. Am J Pathol. 1987;129:201–207.

24 Kadin ME. Genetic and molecular genetic studies in the diagnosis of T-cell malignancies. Hum Pathol. 2003;34:322–324.

25 Waldmann TA, Davis MM, Bongiovanni KD, Korsmeyer SJ. Rearrangements of genes for the antigen receptor on T-cells as markers of lineage and clonality in human lymphoid neoplasms. N Engl J Med. 1985;313:776–783.

Last updated: 9 Apr 2006

Peripheral Post-Thymic T-Cell Lymphoma

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