peripheral T-cell lymphoma

Digital case

 HPC:325 : Intestinal T-cell lymphoma

Microscopy

Thick-walled vessels are more prominent in peripheral T-cell lymphomas than in B-cell non-Hodgkin lymphomas, and the even mixture of atypical small, intermediate, and large lymphoid elements is anotherclue to the lineage of this type of lymphoma.

Scattered eosinophils and plasma cells may also be seen.

Irregularity of the nuclear contour may be particularly prominent, and, in further contrast to lymphomas of B-cell lineage, these tumors in many cases have cells with a clear cytoplasm.

Immunochemistry

Detailed phenotypic studies have shown that peripheral T-cell lymphomas are uniformly TdT−, CD45+ with most having a CD3+, CD45Ro+, CD4+, CD8− helper T-cell phenotype.

Other T-cell antigens (e.g., CD2, CD5, CD7) are variably expressed, and the loss of these markers is characteristic of peripheral T-cell lymphoma.

 CD30+/- (14657710)
 CD15+/- (14657710)

Differential diagnosis

Virally mediated or drug hypersensitivity immuno-blastic reactions may mimic peripheral T-cell lymphoma, as can Hodgkin lymphoma, B-cell lymphomas rich in T-cells (“T-cell-rich B-cell lymphomas”), and Fas mutation-related (ALPS).

Pediatric PTCL

Peripheral T-cell lymphomas, including angioimmunoblastic T-cell lymphoma, represent only a small fraction of lymphomas in children, and heterogeneity is their histologic hallmark.

At low power, the lymph node architecture is either diffusely effaced or shows interfollicular expansion.

Localization

 nodal peripheral T-cell lymphoma

Variants

 CD56+/CD3+ peripheral T-cell lymphoma (15794680)
 peripheral T-cell lymphoma with nodular growth pattern (12100523)
 peripheral T-cell lymphoma with follicular T-cell markers (18779728)
 peripheral T-cell lymphoma, NOS

Associations

 clonal B-cell populations
 secondary Epstein-Barr virus (EBV) driven B-cell expansions
 EBV-associated B-cell lymphoma
 associated Epstein-Barr virus negative clonal plasma cell proliferations and lymphomas (17721185)

• clonal/monotypic plasma cell proliferation
• clonal/monotypic large B-cell proliferation
• B-cell lymphoma with plasmacytic/plasmablastic differentiation

T-cell receptor-Vβ repertoire

T-cell receptor-Vβ repertoire analysis by flow cytometry in biopsies is a basis for peripheral T-cell lymphoma diagnosis and precise T-cell clone identification and characterization. (22627740)

Cytogenetics

 t(14;19)(q11;q13) in peripheral T-cell lymphoma (PTCL)

• involvement of the TRA@/TRD@ locus at 14q11 and PVRL2 at 19q13 (17696193)

CGH (17044049)

 gains

• Chr.17 gain (17q11-q25 gains)
• Chr.8 gain (involving the MYC locus at 8q24)
• 22q gain

 losses

• 13q losses
• Chr.9 losses (9p21-q33 losses)

See also

 T-cell lymphomas

• peripheral T-cell lymphoma, NOS

References

 Accurate detection of the tumor clone in peripheral T-cell lymphoma biopsies by flow cytometric analysis of TCR-Vβ repertoire. Salameire D, Solly F, Fabre B, Lefebvre C, Chauvet M, Gressin R, Corront B, Ciapa A, Pernollet M, Plumas J, Macintyre E, Callanan MB, Leroux D, Jacob MC. Mod Pathol. 2012 May 25. PMID: 22627740

 Balague O, Martinez A, Colomo L, Rosello E, Garcia A, Martinez-Bernal M, Palacin A, Fu K, Weisenburger D, Colomer D, Burke JS, Warnke RA, Campo E. Epstein-Barr virus negative clonal plasma cell proliferations and lymphomas in peripheral T-cell lymphomas: a phenomenon with distinctive clinicopathologic features. Am J Surg Pathol. 2007 Sep;31(9):1310-22. PMID: 17721185

 Thorns C, Bastian B, Pinkel D, Roydasgupta R, Fridlyand J, Merz H, Krokowski M, Bernd HW, Feller AC. Chromosomal aberrations in angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma unspecified: A matrix-based CGH approach. Genes Chromosomes Cancer. 2007 Jan;46(1):37-44. PMID: 17044049