Unusual Course of Splenic Marginal Zone Lymphoma

A 53-year-old woman was diagnosed with splenic marginal zone lymphoma by pathological examination on left submandibular lymph node and bone marrow biopsies and markedly enlarged spleen. Four cycles of Rituximab chemotherapy were given. Seven months after finishing Rituximab chemotherapy, she developed left upper extremity swelling without evidence of deep venous thrombosis. Repeat PET/CT scan demonstrated multiple left axillary lymph nodes extending to left retroclavicular region and a new lymph node posterior to the left scapula. Biopsy of the lymph node demonstrated marginal zone lymhoma pattern with increased numbers of large cells, but not outright diffuse large B-cell lymphoma. Despite resuming rituximab, patient had persistent leukocytosis and severe anemia. Restaging PET/CT showed 3 new left anterior cervical lymph nodes and 1 new right axillary lymph node. Spleen has further enlarged. R-CHOP chemotherapy was started, which improved leukocytosis.After 4 cycles of R-CHOP, PET/CT showed new metabolic activity within right inguinal and abdominal lymph nodes. Patient was given one cycle of Bendamustine. She developed a possible "hematoma" in right medial elbow. However, MRI study revealed a subcutaneous deposit of the lymphoma. Patient needs consistently blood transfusion and she deteriorated quickly. Our patient had an aggressive course of splenic marginal zone lymphoma, not responding to four trials of chemotherapy although SMZL is well-known to be an indolent low grade lymphoma. This case report emphasizes the importance to individualize the treatment in SMZL patients and repeat bone marrow biopsy if the disease recurs.