Hepatosplenic T-Cell Lymphoma: A Population-Based Study Assessing Incidence and Association With Immune-Mediated Disease.
Hepatosplenic T-cell lymphoma (HSTCL) is a rare malignancy of unknown incidence that has been associated with immune-mediated disease. This study explored the incidence and patient characteristics of HSTCL in a population of 15.5 million over a 13-year period using a comprehensive national pathology database in The Netherlands (Pathologisch-Anatomisch Landelijk Geautomatiseerd Archief) with 100% capture. Twelve cases of HSTCL were identified during this period. The overall incidence of HSTCL in the Dutch population over this period was estimated at 0.06 per million inhabitant-years. All but 2 of the patients were adults at the time of diagnosis (median age, 34.5 years), and most patients died within a year of diagnosis. Three patients had a history of immune-mediated disease, 1 of whom was receiving azathioprine at the time of HSTCL diagnosis. Azathioprine as well as anti-tumor necrosis factor-α agents have been reported as possibly being associated with HSTCL. None of the 12 HSTCL patients had been treated with an anti-tumor necrosis factor-α agent.
Dr Montgomery is the executive medical director at Incyte Corporation in Wilmington, Delaware. At the time of the study, he was a director at Janssen Biotech in Horsham, Pennsylvania. Ms van Santen is a surgical pathologist at the Academic Medical Center of the University of Amsterdam in Amsterdam, The Netherlands. Dr Biemond is an internist-hematologist in the Department of Haematology at the Academic Medical Center of the University of Amsterdam. Dr Diamond is the vice president of medical affairs at BTG International, Inc, in West Conshohocken, Pennsylvania. At the time of the study, he was the lead medical director at Janssen Biotech. Dr Pals is a professor of pathology at the Academic Medical Center of the University of Amsterdam.
Michael
M
Montgomery
Dr Montgomery is the executive medical director at Incyte Corporation in Wilmington, Delaware. At the time of the study, he was a director at Janssen Biotech in Horsham, Pennsylvania. Ms van Santen is a surgical pathologist at the Academic Medical Center of the University of Amsterdam in Amsterdam, The Netherlands. Dr Biemond is an internist-hematologist in the Department of Haematology at the Academic Medical Center of the University of Amsterdam. Dr Diamond is the vice president of medical affairs at BTG International, Inc, in West Conshohocken, Pennsylvania. At the time of the study, he was the lead medical director at Janssen Biotech. Dr Pals is a professor of pathology at the Academic Medical Center of the University of Amsterdam.
Marije M
MM
van Santen
Dr Montgomery is the executive medical director at Incyte Corporation in Wilmington, Delaware. At the time of the study, he was a director at Janssen Biotech in Horsham, Pennsylvania. Ms van Santen is a surgical pathologist at the Academic Medical Center of the University of Amsterdam in Amsterdam, The Netherlands. Dr Biemond is an internist-hematologist in the Department of Haematology at the Academic Medical Center of the University of Amsterdam. Dr Diamond is the vice president of medical affairs at BTG International, Inc, in West Conshohocken, Pennsylvania. At the time of the study, he was the lead medical director at Janssen Biotech. Dr Pals is a professor of pathology at the Academic Medical Center of the University of Amsterdam.
Bart J
BJ
Biemond
Dr Montgomery is the executive medical director at Incyte Corporation in Wilmington, Delaware. At the time of the study, he was a director at Janssen Biotech in Horsham, Pennsylvania. Ms van Santen is a surgical pathologist at the Academic Medical Center of the University of Amsterdam in Amsterdam, The Netherlands. Dr Biemond is an internist-hematologist in the Department of Haematology at the Academic Medical Center of the University of Amsterdam. Dr Diamond is the vice president of medical affairs at BTG International, Inc, in West Conshohocken, Pennsylvania. At the time of the study, he was the lead medical director at Janssen Biotech. Dr Pals is a professor of pathology at the Academic Medical Center of the University of Amsterdam.
Robert H
RH
Diamond
Dr Montgomery is the executive medical director at Incyte Corporation in Wilmington, Delaware. At the time of the study, he was a director at Janssen Biotech in Horsham, Pennsylvania. Ms van Santen is a surgical pathologist at the Academic Medical Center of the University of Amsterdam in Amsterdam, The Netherlands. Dr Biemond is an internist-hematologist in the Department of Haematology at the Academic Medical Center of the University of Amsterdam. Dr Diamond is the vice president of medical affairs at BTG International, Inc, in West Conshohocken, Pennsylvania. At the time of the study, he was the lead medical director at Janssen Biotech. Dr Pals is a professor of pathology at the Academic Medical Center of the University of Amsterdam.
Steven T
ST
Pals
Lymphoma; hepatosplenic; immune-mediated disease; immunodeficiency; immunosuppression