An unusual case of IgE-multiple myeloma presenting with systemic amyloidosis 2 years after cervical plasmacytoma...

A 69-year-old man suffered from a fractured cervical vertebra, and magnetic resonance imaging revealed a solitary mass occupying the injured lesion. Surgical resection of the mass was conducted, and the infiltration of plasma cells expressing IgE-lambda monoclonal protein was shown on pathological investigation. Concurrently, a monoclonal band of IgE-lambda was shown in the sera on immunoelectrophoresis, and the proliferation of plasma cells (more than 10%) was also detected in bone marrow aspirates. A rare IgE-multiple myeloma (MM) was diagnosed, and careful monthly follow-up was started. During this observation period, the level of IgE gradually increased and, at 2 years after the initial diagnosis, bilateral leg edema and ascites with marked hepatosplenomegaly appeared. Combination chemotherapy of melphalan and prednisolone (MP) was started, which resulted in the partial, transient resolution of symptoms. Additional treatment with bortezomib and dexamethasone could neither resolve these clinical symptoms nor decrease the serum IgE concentration. At 5 months after the start of chemotherapy, amyloid deposition was suggested based on typical echocardiographic findings, and the diagnosis of amyloidosis was confirmed based on the histopathology of a liver biopsy. In spite of MP plus thalidomide treatment, cardiac failure due to amyloidosis aggravated, and the patient died of multiple organ failure. This is the second reported case in which IgE-MM was complicated by systemic amyloidosis.