hepatosplenic gammadelta T-cell lymphoma

Bone marrow (HNE) Bone marrow (CD68) Spleen (HNE) Spleen (HNE) Spleen (HNE) Spleen (HNE) Spleen (HNE) Spleen (HNE) Spleen (CD20) Spleen (CD3) Spleen (CD3) Spleen (CD68)

Definition: Hepatosplenic gammadelta T-cell lymphoma is a lymphoma in which tumoral cells bearing the gammadelta T-cell receptor (TCR) infiltrate the sinusoids of the liver and the sinuses of the splenic red pulp and bone marrow, without lymph node involvement. It is characterized by a recurrent cytogenetic finding, isochromosome 7q (i7q10).

It has been associated in some cases with immunosuppression, mainly after solid organ transplants.

The T-cell receptor (TCR) expressed on the surface of most T-lymphocytes is of alpha beta type, and only a minority bear the gamma delta-TCR. Similarly, postthymic T-cell lymphomas rarely express gamma delta-TCR.

Synopsis

- bone marrow localization: sinusoidal infiltrate of atypical small to medium-sized lymphoid cells (11554170)

Cytogenetics

- isochromosome 7q (i7q10) (17509984, 11807981)

  • possible increased number of i(7)(q10) chromosome during evolution (11807981)

- t(7;14)(q34;q13) (7q34 and 14q13) (11554170)

Associations

- hepatitis B virus infection (9597812)
- azathioprine (12688327)

References

- Vega F, Medeiros LJ, Gaulard P. Hepatosplenic and other gammadelta T-cell lymphomas. Am J Clin Pathol. 2007 Jun;127(6):869-80. PMID: 17509984

- Navarro JT, Ribera JM, Mate JL, Granada I, Juncà J, Batlle M, Millá F, Feliu E. Hepatosplenic T-gammadelta lymphoma in a patient with Crohn’s disease treated with azathioprine. Leuk Lymphoma. 2003 Mar;44(3):531-3. PMID: 12688327

- Belhadj K, Reyes F, Farcet JP, Tilly H, Bastard C, Angonin R, Deconinck E, Charlotte F, Leblond V, Labouyrie E, Lederlin P, Emile JF, Delmas-Marsalet B, Arnulf B, Zafrani ES, Gaulard P. Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. Blood. 2003 Dec 15;102(13):4261-9. PMID: 12907441

- Gaulard P, Belhadj K, Reyes F.Gammadelta T-cell lymphomas.Semin Hematol. 2003 Jul;40(3):233-43. PMID: 12876672

- Wlodarska I, Martin-Garcia N, Achten R, De Wolf-Peeters C, Pauwels P, Tulliez M, de Mascarel A, Brière J, Patey M, Hagemeijer A, Gaulard P. Fluorescence in situ hybridization study of chromosome 7 aberrations in hepatosplenic T-cell lymphoma: isochromosome 7q as a common abnormality accumulating in forms with features of cytologic progression.Genes Chromosomes Cancer. 2002 Mar;33(3):243-51. PMID: 11807981

- Wong KF, Chan JK, Matutes E, McCarthy K, Ng CS, Chan CH, Ma SK. Hepatosplenic gamma delta T-cell lymphoma. A distinctive aggressive lymphoma type. Am J Surg Pathol. 1995 Jun;19(6):718-26. PMID: 7755158

[ Return to site page ]