BACKGROUND Primary hepatic non‐Hodgkin lymphoma (PHL) is a rare and difficult to diagnose lymphoproliferative disorder of unknown etiology. It is believed that the prognosis in affected patients is dismal, consisting of early recurrence and short survival. METHODS A retrospective cohort review of patients with PHL diagnosed between 1974 and 1995 at a university cancer center was performed. RESULTS Twenty‐four patients with PHL were identified. Typically, the disease occurred in middle‐aged men (median age, 50 years). The primary presenting complaint was right upper quadrant abdominal pain, with hepatomegaly found at physical examination. Serum liver enzymes, lactate dehydrogenase, and β‐2‐microglobulin levels all were elevated, but α‐fetoprotein and carcinoembryonic antigen levels were within normal range. Hypercalcemia was found in 6 of 15 patients who were tested. Six of 10 patients who were tested were positive for the hepatitis C virus (HCV). Liver scans demonstrated either a solitary lesion or multiple lesions. Pathologic examination revealed diffuse large cell lymphoma in 23 patients (96%). Combination chemotherapy was the mainstay of treatment; surgery consisted of diagnostic biopsy. The complete remission rate was 83.3%, and the 5‐year cause specific and failure free survival rates were 87.1% and 70.1%, respectively. HCV infection did not appear to influence the outcome of therapy. CONCLUSIONS The outcome of patients with PHL who are treated with combination chemotherapy may be more favorable than that reported elsewhere. The frequent association of PHL with HCV infection observed in this series warrants further investigation. Cancer 2001;92:2023–9. © 2001 American Cancer Society.

We analyzed the records of 96 previously untreated patients with stage IV follicular low-grade lymphoma (FLGL) uniformly treated with cyclophosphamide, doxorubicin, vincristine, prednisone, and bleomycin (CHOP-Bleo) chemotherapy from 1972 to 1982. The overall complete remission (CR) rate was 77%. At a median follow-up of 138 months, the 10-year cause-specific survival rate was 42% with a median survival of 100 months. Failure-free survival (FFS) was 15% at 10 years with a median FFS of 30 months. Multivariate analysis showed peripheral lymph node size (LN), degree of marrow involvement, and sex, in that order, to be important for FFS, while the number of extranodal sites (#ENS), LN, sex, and degree of marrow involvement were important for cause-specific survival. We devised a tumor burden (TB) model, incorporating #ENS, LN, and degree of marrow involvement. Three groups were identified with statistically significant differences in cause-specific survival and FFS. Those with low TB (one ENS exclusive of extensive marrow and nodal disease less than 5 cm) had a 10-year cause-specific survival of 73% compared with 24% for patients with high TB (greater than or equal to two ENS and nodal disease greater than or equal to 5 cm) (P less than .001) and 40% for those with intermediate TB (either greater than or equal to 2 ENS, or extensive marrow only, or nodal disease greater than 5 cm) (P = .050). Patients with low TB had a 10-year FFS rate of 32%, while the intermediate and high TB groups had 10% and 9% FFS, respectively (P = .003). Because sex was a very strong prognostic variable, we created a risk model for survival and FFS based on TB and sex. Females with low TB had the best prognosis (92% survival and 50% FFS at 10 years) and males with high TB had the worst outlook (median survival and FFS, 43 and 12 months, respectively). Other TB-sex combinations defined two groups with statistically significant differences in survival but comparable FFS. This model should aid in the design and analysis of future trials.

Previously untreated adult patients who presented with advanced diffuse large-cell lymphoma (DLCL) at diagnosis were studied to identify possible prognostic factors. One hundred five patients were seen between 1974 and 1981; 45 patients were stage III and 60 patients were stage IV. All patients received cyclophosphamide, doxorubicin, vincristine, prednisone, and bleomycin (CHOP-Bleo). Stage III patients also received radiation therapy alternated with chemotherapy. Overall survival was 50% at 5 years and 43% at 8 years. Seventy-four patients achieved a complete remission (CR) and 37 are alive and disease-free with a median follow-up of 72 months. There was no difference in clinical outcome between stage III and stage IV. However, a proportional hazards model identified lactic dehydrogenase (LDH) level and tumor burden, among all clinical factors studied, as independent risk factors for survival. These two factors were also important for achievement of remission and relapse-free survival. Three distinct patient risk groups were identified with 5-year survival rates of 87%, 48%, and 20%, respectively. The measure of tumor burden proposed herein, along with LDH level, can be used for developing treatment programs, and for meaningful comparison of different treatment regimens, as well as assessment of prognosis.

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