We report on a patient with a rare hepatosplenic γδ T-cell lymphoma (γδ TCL) presenting clinically with B-symptoms, hepatosplenomegaly and pancytopenia. During the initial stage of the disease the sparse malignant cells could not be detected histologically. Furthermore, their identification was obscured by massive macrophage proliferation with haemophagocytosis in the spleen. Diagnosis was established by detection of a clonal T-cell receptor (TcR) rearrangement and, retrospectively, by demonstration of rare cells expressing an aberrant T-cell phenotype. The findings in this patient emphasize that minimal neoplastic T-cell infiltrates can lead to severe clinical symptoms. Initial biopsy findings may be misinterpreted as benign. γδ TCL may elaborate lymphokines that suppress haematopoiesis, leading to pancytopenia and macrophage proliferation.
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Arnold A, Cossman J, Bakhshi A, Jaffe ES, Waldman TA, Korsmeyer SJ (1993) Immunoglobulin-gene rearrangements as unique clonal markers in human lymphoid neoplasms. N Engl J Med 309:1593–1599
Ausubel FM, Brent R, Kingston RE, Moore DD, Seidman JG, Smith JA Struhl K (eds) 1990 Current protocols in molecular biology, vol 1. Green Publishing Associates/Wiley, New York, pp 2.2.1–2.2.3
Bucy RP, Chen-Lo HC, Cihak J, Lösch V, Cooper MD (1988) Avian T cells expressing gamma delta receptors localize in the splenic sinusoid and the intestinal epithelium. J Immunol 141:2200–2205
Cooke CB, Greiner T, Raffeld M, Kingma D, Statler-Stevenson M, Jaffe E (1994) γδ T cell lymphoma: a distinct clinicopathological entity. Mod Pathol 7: 106A
Farcet JP, Gaulard P, Marolleau JP, Le Couedic JP, Henni T, Gourdin MF, Divine M, Haioun C, Zafrani S, Goossens M, Hercend T, Reyos F (1990) Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor γδ. Blood 75:2213–2219
Gaulard P, Zafrani ES, Mavier P, Rocha FD, Farcet JP, Divine M, Haioun C, Pinaudeau Y (1986) Peripheral T-cell lymphoma presenting as predominant liver disease: a report of three cases. Hepatology 6:864–868
Gaulard P, Bourquelot P, Kanavaros P, Haioun C, Le Couedic JP, Divine M, Goossens M, Zafrani ES, Farcet J-P, Reyes F (1990) Expression of the αβ and γδ T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of γδ T-cell lymphomas. Am J Pathol 137:617–628
Haioun C, Gaulard P, Bourquelot P, Roudot-Thoraval F, Divine M, Lavaud A, Bagot M, Vasile N, Farcet J-P, Reyes F (1992) Clinical and biological analysis of peripheral T-cell lymphomas: a single institution study. Leuk Lymphoma 7:449–455
Hsu SM, Raine L, Fauger H (1981) Use of avidin-biotin-peroxidase complex (ABC) in immunoperoxidase techniques: a comparison between ABC and unlabled antibodies (PAP) procedures. J Histochem Cytochem 29:577–580
Janeway CA (1988) Frontiers of the immune system. Nature 333:804–806
Kanavaros P, Farcet JP, Gaulard P, Haioun C, Divine M, Le Couedic JP, Lefranc MP, Reyes F (1991) Recombinative events of the T cell antigen receptor δ gene in peripheral T cell lymphomas. J Clin Invest 87:666–672
Kruskall MS, Weitzmann SA, Stossel TP, Harris N, Robinson SH (1982) Lymphoma with autoimmune neutropenia and hepatic sinusoidal infiltration: a syndrome. Ann Intern Med 97:202–205
Lin MT, Shen MC, Tien HF, Chen YC, Hsu PN, Chang CS, Wang CH (1991) Peripheral T gd lymphoma presenting with idiopathic thrombocytopenic purpura-like picture. Br J Haematol 78:280–282
Mastovich S, Ratcj H, Ware RE, Moore JO, Borowitz MJ (1994) Hepatosplenic T-cell lymphoma: an unusual case of a γδ T-cell lymphoma with a blast-like terminal transformation. Hum Pathol 25:102–108
Minden MD, Toyonaga B, Ha K, Yanagi Y, Chin B, Gelfand E, Mak T (1985) Somatic rearrangement of T-cell antigen receptor gene in human T-cell malignancies. Proc Natl Acad Sci USA 82:1224–1227
Picker LJ, Weiss LM, Medeiros JL, Wood GS, Warnke RA (1987) Immunophenotypic criteria for diagnosis of non-Hodgkin's lymphoma. Am J Pathol 128:181–201
Weiss A, Imboden S, Hardy K, Manger B, Terhorst C, Stobos S (1986) The role of T3 antigen receptor complex in T-cell activation. Annu Rev Immunol 4:593–619
Wilhelm M, Meyer P, Batram C, Tony HP, Dummer R, Nestle F, Burg G, Wilms K (1992) γ/δ Receptor-expressing T-cell clones from a cutaneous T-cell lymphoma suppress hematopoiesis. Ann Hematol 65:111–115
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Dommann-Scherrer, C.C., Zimmermann, D.R., Odermatt, B.F. et al. Occult hepatosplenic T-γδ lymphoma. Vichows Archiv A Pathol Anat 426, 629–634 (1995). https://doi.org/10.1007/BF00192119
- T-cell lymphoma
- γδ T-cell receptor