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

Abstract

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.

A rare and unique case of aggressive IgE-λ plasma cell myeloma in a 28-year-old woman presented initially as an orbital mass

“…A case of prolonged survival, on the other hand, has been documented as well [4]. Other rare clinical presentations included cutaneous manifestation, systemic amyloidosis, and elevated serum CA125 [8][9][10]. Our patient's clinical presentation also included lytic bone lesions and clinical evidence of involvement of the mesentery and lymph nodes, but she also presented with an orbital mass, which differed from what has been previously reported.…”

“…Since the first case of IgE PCM reported in 1967 [2], there have been only 47 cases of IgE PCM described in the English literature [2][3][4][5][6][7][8][9][10]. Aside from the typical clinical presentations of PCM, rare cases of IgE PCM involving the skin, breast, and orbit have also been reported [8,11,12].…”

Liver Failure Caused by Light Chain Deposition Disease Associated with Multiple Myeloma

“…Treatment with melphalan-prednisone and bortezomib is of limited value in most patients with LCCD liver involvement (24,25). Moreover Samanez et al reported a liver failure with rapid fatal outcome that debuted after the first cycle of VAD (26).…”

IgG Lambda Myeloma Presenting as Plasmacytic Ascites: Case Report and Review of Literature

“…IgA was found in 34 % cases (15 cases) including one case of biclonal expression with IgD [16]. Three cases of clonal IgD [14,20], two cases of monoclonal light chain production [21,22], one case of clonal IgE production [23], and one nonsecretory myeloma was reported.…”