Abstract
BackgroundImmunoglobulin D multiple myeloma (MM) is rare and has a poorer prognosis than other MM isotypes.Design and methodsSeventeen patients (pts) diagnosed from 1993 to 2009 with IgD MM were selected from six institutions of Multiple Myeloma Latium-Region GIMEMA Working Group.ResultsMedian age was 55 years, 14 patients had bone lesions, eight had renal impairment with estimated glomerular filtration rate (eGFR) < 50 ml/min, one serum calcium ≥ 12 mg/dl, 11 had lambda light chains, five stage III of ISS, six with chromosomal abnormalities. Six pts received conventional chemotherapy (CT): five melphalan + steroids based regimens. Eleven underwent high-doses of chemotherapy with autologous stem cell transplantation (HDT/ASCT), five single and six tandem ASCT: six received bortezomib and/or thalidomide as induction therapy and five VAD. Thalidomide maintenance was used in two pts: one in HDT/ASCT and one in CT group; bortezomib was used in one patient after HDT/ASCT. At a median follow up of 38 (range 19-60) and 50 months (range 17-148) for pts treated with CT and HDT/ASCT, respectively, the overall response rate (ORR) was 83% and 90%. In the group of patients treated with CT, median overall survival (OS) was 34 months (95% CI 15- 54 months), median progression free survival (PFS) was 18 months (95% CI 3-33 months) and median duration of response (DOR) was 7 months (95% CI 5-9 months). Median OS, PFS and DOR were not reached at the time of this analysis in the HDT/ASCT group of patients. Death was observed in 27.3% of pts treated with HDT/ASCT and in 66.7% undergone CT.ConclusionsDespite the retrospective analysis and the small number of pts our study showed that the use of HDT/ASCT seems to improve also the prognosis of IgD MM patients. Treatment options including new drugs, before and after stem cell transplantation, may further improve the outcomes of these patients.
Highlights
Immunoglobulin D multiple myeloma (MM) is rare and has a poorer prognosis than other MM isotypes
Six patients were treated with CT, five with Melphalan plus steroids based regimens and one with Vincristine Adriamycin Dexametasone (VAD) (Vincristine, Adriamycin and Dexametasone) plus Cyclophosphamide Etoposide Dexametasone (CED) (Cyclophosphamide, Etoposide, Dexamethasone); one patient showed a complete response (CR), two very good partial respone (VGPR), two partial response (PR) and one stable disease (SD)
The overall response rate (ORR) was 83%; after a median follow up of 38 months for patient treated with conventional chemotherapy, the median overall survival (OS) was 34 months and the median progression free survival (PFS) was 18 months
Summary
Immunoglobulin D multiple myeloma (MM) is rare and has a poorer prognosis than other MM isotypes. Immunoglobulin (Ig)D multiple myeloma (IgD MM) is a rare subtype of myeloma, accounts for less than 2% of all myelomas [1] and is accompanied with aggressive course, resistance to chemotherapy and poor outcome. It is often associated with relatively high frequencies of renal failure, extra osseous disease, hypercalcemia, amyloidosis and Bence-Jones proteinuria [2,3,4,5]. The survival have shown effectiveness against disease. These developments may have led to changes in the outcomes of IgD MM. Differences between survival curves were tested for statistical significance with the two-sided log-rank test
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