Abstract
The association of light-chain (AL) amyloidosis with type B follicular lymphoma is extremely rare, as the clone secreting the amyloidogenic light chain is generally plasmacytic. We here report the case of a 67-year-old patient with no specific pathological history, presenting with a deterioration in general condition, progressively worsening dysphonia and dysphagia, and a large mass in the cavum. Biopsy findings indicated grade 1-2 type-B follicular non-Hodgkin's lymphoma. Cervico-thoraco-abdomino-pelvic computed tomography (CT) scan revealed a nasopharyngeal mass measuring 70 mm x 40 mm and extending over 60 mm. Bone marrow biopsy and pre-therapeutic evaluations were normal. The patient received 4 cycles of rituximab plus CHOP (cyclophosphamide, adriamycine, prednisone and oncovin) with no response, followed by 3 cycles of rituximab plus DHAOX (dexamethasone, high-dose cytarabine and oxalipatin) with persistence of the mass. A subsequent biopsy of the mass revealed the disappearance of B-cell lymphoid infiltration but showed AL kappa chain amyloid deposits. Immunoelectrophoresis of plasma proteins detected the presence of IgM kappa immunoglobulin. Positron emission tomography (PET) imaging identified a hypermetabolic nasopharyngeal process. The patient is currently undergoing treatment with a protocol combining bortezomib, prednisone, and bendamustine.