Immunoglobulin D myeloma: clinical features and outcome in the era of novel agents.
OBJECTIVE
Immunoglobulin D (IgD) multiple myeloma is an uncommon variant of the disease probably associated with poorer prognosis. However, data on IgD myeloma patients treated in the novel agent era are lacking.


METHODS
To assess the frequency and the specific characteristics and evaluate the outcome of patients with IgD myeloma, we analyzed the database of the Greek Myeloma Study Group.


RESULTS
Between January 2000 and December 2012, among the 1239 patients with symptomatic myeloma, 31 (2.5%) were diagnosed with IgD myeloma. The median age of patients with IgD myeloma was 65 yr (range 26-80 yr) versus 68 yr (range 23-96 yr) of all others, and 84% had lambda light chain (vs. 38% of the patients with other subtypes). Patients with IgD myeloma presented more often with features of high-risk disease, that is, with advanced ISS, high LDH, significant renal dysfunction, and large amounts of Bence Jones proteinuria. Response to primary therapy was similar to other patients, although there was a trend for better quality of responses in patients with IgD myeloma. The median survival of these patients was 51.5 months versus 50.7 months for patients of other subtypes. In a multivariate model to adjust for differences in prognostic features, IgD myeloma was not associated with a different prognosis.


CONCLUSIONS
The incidence of IgD myeloma is 2.5%. Although patients with IgD myeloma present more often with high-risk features, their outcome in the era of novel agents is similar to that of patients with other myeloma subtypes.
Department of Clinical Therapeutics, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Flora
F
Zagouri
Efstathios
E
Kastritis
Argiris S
AS
Symeonidis
Nikolaos
N
Giannakoulas
Eirini
E
Katodritou
Sosana
S
Delimpasi
Panagiotis
P
Repousis
Evangelos
E
Terpos
Meletios A
MA
Dimopoulos
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D016208:Databases, Factual; D005260:Female; D006115:Greece / Q000453:epidemiology; D006801:Humans; D007072:Immunoglobulin D / Q000097:blood*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D011379:Prognosis; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D007072:Immunoglobulin D
Bence Jones proteinuria; IgD myeloma; lambda light chain; novel agents; prognosis