IgE Multiple Myeloma

Abstract

IgE multiple myeloma is a rare disease characterized by a high frequency of Bence-Jones proteinuria and plasma cell leukaemia when compared to other isotypes of monoclonal proteins. Only 35 cases have been reported. We describe a 70-year-old woman with a stage III IgE kappa multiple myeloma presenting with a sacral plasmacytoma. Immunological and biochemical studies showed IgE kappa producing tumoral plasma cells. Serum total IgE was high without clinical symptoms suggesting an hyperIgE syndrome or mast cell activation. The patient underwent surgical removal of the sacral tumor and monthly melphalan-prednisone treatment together with intravenous pamidronate infusions. Magnetic Resonance Imaging (MRI) of the dorsolumbar spine revealed an epidural process leading to T6-T9 radiotherapy. Bone densitometry showed a decreased bone mineral content supporting the management of myeloma-related osteoporosis with bisphosphonate infusions. A good partial response with plateau-phase and increase of bone mineral content was achieved after 1 year of treatment and still persists after a 28 months follow-up.

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

“…Unique clinical features such as a higher frequency of plasma cells in the peripheral blood, a high incidence of osteosclerotic (osteoblastic) change, and hepatosplenomegaly have been documented [10]. Possibly because of diagnostic delay, IgE-MM cases show more aggressive clinical courses, with a mean survival of 16 months, compared with an average of 30 months in other immunoglobulin types [11].…”

Plasma cell leukemia producing monoclonal immunoglobulin E

“…Notably, 4 of 6 primary IgE PCL patients had neither osteolytic skeletal lesions nor elevation of serum calcium concentrations. In fact, the lower incidence of lytic bone lesions was reported in patients with IgE PCD than that in patients with PCD from other immunoglobulins than IgE (50 vs. 80%) [16,22].…”

“…An aggressive clinical course and no history of prior smoldering PCD suggested that our patient had developed the primary PCL rather than the secondary one. Macro et al [16] demonstrated a higher incidence of PCL in IgE PCDs than that in other classes of PCDs (approximately 18% vs. 1.5-2%), although the former presents various clinical pictures as well. Including our patient described, we found 8 cases of IgE-producing PCL in the literature (6 primary and 2 possible secondary PCLs [1,5,[17][18][19][20][21]) (Table 1).…”

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

“…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].…”