Lymphomatoid Granulomatosis: A Clinicopathologic Study of a Series of Cases in a Single Institution

Lymphomatoid granulomatosis is a rare lymphoproliferative disorder with a proclivity for extranodal involvement, including the lungs, brain, kidney, liver, and skin. It usually affects middle-age adults, with male predominance (M:F ratio >2:1). Its association with EBV (Epstein Barr Virus) infection has been described.

To present our experience of 8 cases of lymphomatoid granulomatosis at Moffitt Cancer Center (MCC) in the last 10 years. Results: We collected the data of 8 cases of lymphomatoid granulomatosis that were diagnosed and treated at MCC from January 2000 to January 2011. The median follow up was 7.5 months. Five out 8 (63%) cases were males. The median age was 65. 3/8 (12.5%) had a performance status (PS) more than 2. 4/8 (50%) of cases had elevated LDH. All patients (100%) had an Ann Arbor Stage IVE. The IPI (International Prognostic Index) was more than high-intermediate in 6/8 (75%) cases. 4/8 (50%) of cases were positive for EBV. 6/8 (75%) have received chemotherapy to date, most commonly R+CHOP. The median time to progression was 8.6 months (1-8 months) following the first treatment and was shorter after the second treatment (4.8 months). 2/8 (25%) are disease free after chemotherapy, all remaining patients have active disease. 2/8 (25%) have died at 7 and 13 months after diagnosis.

Lymphomatoid granulomatosis is a rare lymphoproliferative disease. In our experience it presents as an advanced and high risk disease. Interestingly, EBV was found in only 50% of cases. The overall response to standard chemotherapy is suboptimal and further strategies are needed for optimal management. These might include aggressive chemotherapy and up front stem cell transplantation.

No relevant conflicts of interest to declare.