BACKGROUND: Primary Hepatic (PHL) and Primary Splenic (PSL) non-Hodgkin's Lymphoma are rare entities. Small series of PHL and PSL have been reported, suggesting a non-fortuitous association with Hepatitis C Virus (HCV) infection. The prognosis is believed to be dismal, with early recurrence and short survival. PATIENTS: We retrospectively reviewed all PHL and PSL patients diagnosed at our institution between 1990 and 2005. RESULTS: Twenty-five adult patients were identified, six with PHL and 19 with PSL. Twenty-four patients had a B-cell lymphoma, defined as Diffuse Large B-cell lymphoma in 18. The prevalence of HCV infection was 68% among PSL and 66% among PHL. Combination chemotherapy was the mainstay of treatment for PHL and PSL; all but one patient with PSL underwent splenectomy before chemotherapy. Complete remission was achieved in all the cases after frontline therapy; only four patients relapsed but responded to additional chemotherapy courses. Most patients presented with aggressive histological subtypes; 92% were alive at a median follow up of 79 months. HCV infection did not appear to influence the results of therapy. CONCLUSION: Our study confirms the rarity of PHL and PSL, shows a high prevalence of HCV infection, and demonstrates that the outcome of patients with PHL and PSL may be favourable.
BACKGROUND: Primary Hepatic (PHL) and Primary Splenic (PSL) non-Hodgkin's Lymphoma are rare entities. Small series of PHL and PSL have been reported, suggesting a non-fortuitous association with Hepatitis C Virus (HCV) infection. The prognosis is believed to be dismal, with early recurrence and short survival. PATIENTS: We retrospectively reviewed all PHL and PSL patients diagnosed at our institution between 1990 and 2005. RESULTS: Twenty-five adult patients were identified, six with PHL and 19 with PSL. Twenty-four patients had a B-cell lymphoma, defined as Diffuse Large B-cell lymphoma in 18. The prevalence of HCV infection was 68% among PSL and 66% among PHL. Combination chemotherapy was the mainstay of treatment for PHL and PSL; all but one patient with PSL underwent splenectomy before chemotherapy. Complete remission was achieved in all the cases after frontline therapy; only four patients relapsed but responded to additional chemotherapy courses. Most patients presented with aggressive histological subtypes; 92% were alive at a median follow up of 79 months. HCV infection did not appear to influence the results of therapy. CONCLUSION: Our study confirms the rarity of PHL and PSL, shows a high prevalence of HCV infection, and demonstrates that the outcome of patients with PHL and PSL may be favourable.