Immunoglobulin D myeloma – Problems with diagnosing and staging (own experience and literature review)

Abstract

Immunoglobulin D (IgD) myeloma is a rare disease accounting for about 2% of all myelomas. The distinctive features are the predominant occurrence in males and young patients, short survival time, uncertain appearance of M-component in serum electrophoresis, predominance of lambda light chains, frequent renal impairment, hypercalcemia and amyloidosis. The aim of the present study was to show diagnostic difficulties resulting from a variety of non-specific initial symptoms and laboratory findings as well as to compare the staging system proposed by Durie and Salmon with the new risk grouping by Shimamoto. Case histories of 7 patients were analyzed retrospectively. Five of them were diagnosed as IgD multiple myeloma (IgD MM), 1 as non-secretory IgD myeloma and 1 as solitary bone IgD plasmocytoma that evolved to an IgD MM. All patients were staged according to the Durie and Salmon classification and the new risk grouping by Shimamoto. We report diagnostic problems with IgD myeloma in our patients, with special emphasis on non-specific rheumatoidal and neurological symptoms in 1 case. There was a very good correlation of the Japanese classification with the severity of the disease and the risk of death. In conclusion, the initial symptoms of IgD myeloma can be very misleading. Wide differential diagnosis, including autoimmunological disorders of the connective tissue, is necessary. The new Japanese risk grouping seems to be of greater prognostic significance for IgD myeloma than the Durie and Salmon staging system.

The outcome of IgD myeloma after autologous hematopoietic stem cell transplantation is similar to other Ig subtypes

“…IgD myeloma is believed to have worse prognosis than other Ig subtypes [9][10][11][12][13]. In the study by Blade et al [14], the overall response to therapy was 58%, median overall survival was 21 months, and 5-year survival was 21%.…”

High-dose chemotherapy followed by autologous stem cell transplantation changes prognosis of IgD multiple myeloma

“…IgD M-protein should be sought especially in the setting of l light-chain production and l Bence-Jones proteinuria as IgD MM is associated with these findings. 3,19 In a recent audit by the Czech Myeloma Group, 20 only 38% of laboratories had correctly diagnosed IgD-l MM. A similar experience from the French national external quality assessment was reported by Albarede et al 21 The staging of IgD MM is controversial.…”

Successful Treatment of Immunoglobulin D Myeloma by Bortezomib and Dexamethasone Therapy

“…In addition to its rarity, the poor prognosis might be due to the delayed precise diagnosis. Kuliszkiewicz-Janus et al pointed out the diagnostic difficulties because 40% of IgD myeloma lacks a monoclonal protein spike, and there are a variety of non-specific initial symptoms, such as arthritis and neurological symptoms, due to amyloidosis (1). In the present case, a typical monoclonal protein spike was not recognized by serum protein electrophoresis.…”

“…Few case series are available for IgD myeloma, but it is generally recognized as having aggressive clinical behavior, such as osteolytic lesions, renal impairment, hypercalcemia, anemia, extramedullary plasmacytoma, and amyloidosis (1)(2)(3)(4). Recently, myeloma survival has markedly improved by high-dose chemotherapy with autologous stem cell transplantation (SCT) (5), bortezomib (6), and immunomodulatory drugs, although reports concerning the treatments and responses of IgD myeloma are still limited (2)(3)(4).…”