Hepatosplenic gammadelta-T-cell lymphoma with leukemic course after renal transplantation.
Hepatosplenic gammadelta-T-cell lymphoma (HSTCL) is a rare extranodal T-cell non-Hodgkin's lymphoma (T-NHL) with only 46 well-documented cases in medical literature. Notably, a relatively high number of these case reports (15%) describe the occurrence of HSTCL after solid organ transplantation. We describe the case of a 45-year-old man who developed a leukemic HSTCL 5 years after renal transplantation and continous immunosuppression with cyclosporine A and prednisolone. After a rapid clinical course, the patient died and autopsy was performed. The malignant lymphocytes showed a natural killer-like gammadelta-T-cell phenotype (CD2(+), CD3(+), CD7(+), TCR gammadelta(+), CD56(+), TIA-1(+), CD4(-), CD8(-), and TCR alphabeta(-)) and infiltrated the sinusoids of liver and the red pulp of the spleen. Cytogenetically, an isochromosome 7q, trisomy 8, Y-loss, and a translocation t(1;4) was detectable. This case shows the difficulties of recognizing HSTCL early in the clinical course and underlines that all types of T-NHL, nodal as well as extranodal, have to be considered in the differential diagnosis of posttransplantation lymphoproliferative disorders. Moreover, HSTCL seems to occur as a specific late complication of solid organ transplantation.
Steurer
Michael
M
Division of Haematology and Oncology, University Hospital Innsbruck, Austria.
Stauder
Reinhard
R
Grünewald
Kurt
K
Gunsilius
Eberhard
E
Duba
Hans-Christoph
HC
Gastl
Guenther
G
Dirnhofer
Stephan
S
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002102:Cadaver; D002897:Chromosomes, Human, Pair 7; D017809:Fatal Outcome; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D018404:Isochromosomes; D007621:Karyotyping; D016030:Kidney Transplantation / Q000009:adverse effects*; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis*; D013160:Splenic Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology
D016692:Receptors, Antigen, T-Cell, gamma-delta