- Email: [email protected]
IgD Multiple Myeloma, Descriptive Report of Eight Cases, Single Centre Experience
Abstracts PO-399 IgD Multiple Myeloma, Descriptive Report of Eight Cases, Single Centre Experience S. Ongoren, I. Erdogan, A. Salihoglu, T. Elverdi, F.F. Yalniz, S. Berk, S. Sadri, D. Keskin, N.O. Yurttas, A.E. Eskazan, M.C. Ar, Z. Baslar, T. Soysal, Y. Aydin Department of Internal Medicine, Division of Hematology, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey
Background: IgD multiple myeloma (IgD MM) is a rare form of MM, accounting for 1-2% of all myeloma cases. It can escape diagnosis unless screened for the presence of IgD. In this report we want to share our experience of IgD MM patients. Materials and Methods: We report eight cases diagnosed with IgD MM between 2003-2015 at a single institution. Retrospective data were collected from patient files. Results: Four patients were male (50%), median age was 60.5 (range 50-73). Seven patients were diagnosed IgD lambda MM (87,5%) while one had IgD kappa MM. Seven patients had symptomatic disease (including back pain, generalized pain, fatigue or nausea) at diagnosis. M spike was detected on serum protein electrophoresis in 4 patients (50%). Three patients (37,5%) had immune paralysis at diagnosis. Six patients (75%) had osteolytic bone lesions. Patient characteristics are summarized on
Table 1. One of the patients had normal serum IgD level at diagnosis but IgD lambda monoclonality was detected by immunoelectrophoresis. Four patients (50%) received VAD regimen as first line treatment, while the rest received bortezomib containing regimen. Three patients (37,5%) had progressive disease, 2 (25%) had VGPR, 2 had partial response, 1 (12,5%) had stable disease after first line treatment. During follow up 2 patients (25%) had undergone autologous stem cell transplantation (ASCT). Median overall survival was 26 months (range: 2-111 months). Five patients (62,5%) are still alive and resuming follow up while three patients died because of progressive disease. Conclusion: IgD MM can be presented with or without M spike so can be misdiagnosed as light chain MM. In order to diagnose this rare type of MM, serum and urine immunoelectrophoresis should be performed cause serum IgD can also be normal or lower than normal range in some cases. Prognosis is variable, this can be because of misdiagnosed disease or skip in diagnostic step. In our experience only 25% of patients had VGPR and ASCT was performed to 2 patients (25%), after that one of them had partial response and the other had progressive disease. Although IgD MM is considered to have an aggressive course and a poor outcome with chemoresistant disease, with the advances in myeloma treatment patients with long survival are reported. More studies are needed to determine if the new drugs and ASCT improve outcome of patients with IgD MM. Keywords: Multiple myeloma, IgD, immunoelectrophoresis.
Table 1 Characteristics of patients Characteristics Patients Age Gender Diagnosis
1
2
50
66
3 73
4
5
6
7
8
60
51
65
60
61
M IgD kappa
F IgD lambda
M IgD lambda
F IgD lambda
M IgD lambda
F IgD lambda
M IgD lambda
F IgD lamda
ISS Durie-Salmon Stage
1 1A
3 2B
3 3A
1 3A
1 3A
3 2A
3 3B
3 3B
M spike Bone lesion
(+) (-)
(-) (-)
(-) (+)
(+) (+)
(-) (+)
(-) (+)
(+) (+)
(+) (+)
(-) 9.3
(+) 8.5
(-) 12.1
(-) 11.6
(-) 11.1
(-) 7.6
(-) 6
17 1.18
322 0.59
193 0.8
161 1.07
120 20.2
172 5.1
63 4
110 4.6
108 4.5
54 3.5
3 2.6
9 3.8
Extramedullary involvement Hemoglobin (g/dl)
(+) 11.3
Platelet (x10⁹⁹/L) Serum creatinine (mg/dl)
183 0.77
245 4.5
eGFR (ml/min) Serum albumin (g/dl)
113 3.7
37 4 30.6
7
2.61
3.28
14.3
>20
Beta-2-microglobulin (mg/L) LDH level
2.32
11.3
N
N
H
N
N
N
N
N
Serum IgG Serum IgA
L L
L L
L L
N N
N N
L L
N L
L L
Serum IgM Serum IgD
L H
L H
L H
L H
L N
L H
L H
L H
First treatment regimen Response
VAD (6) SD
VAD (6) VGPR
Bor (1) Pr
Bor (5) Pr
VAD (3) Pr
Bor (8) VGPR
VAD (2) PR
Bor (6) PR
ASCT OS (months)/Statement (Alive/Dead)
(-) 111 (A)
(-) 44 (D)
(-) 2 (A)
(+) 13 (D)
(-) 7 (A)
(-) 39 (A)
(-) 2 (D)
(+) 47 (A)
Abbreviations: ISS: International Staging System, eGFR: estimated Glomerular Filtration Rate, LDH: serum lactate dehydrogenase, N: Normal, L: Low, H: High, VAD: Vincristine, Adriamycin, Dexamethasone, Bor: Bortezomib, SD: Stabil Disease, VGPR: Very Good Partial Response, Pr: Progression, PR: Partial Response, ASCT: Autologous Stem Cell Transplantation, OS: Overall Survival, A: Alive, D: Dead
15th International Myeloma Workshop, September 23-26, 2015
- e313
Background: IgD multiple myeloma (IgD MM) is a rare form of MM, accounting for 1-2% of all myeloma cases. It can escape diagnosis unless screened for the presence of IgD. In this report we want to share our experience of IgD MM patients. Materials and Methods: We report eight cases diagnosed with IgD MM between 2003-2015 at a single institution. Retrospective data were collected from patient files. Results: Four patients were male (50%), median age was 60.5 (range 50-73). Seven patients were diagnosed IgD lambda MM (87,5%) while one had IgD kappa MM. Seven patients had symptomatic disease (including back pain, generalized pain, fatigue or nausea) at diagnosis. M spike was detected on serum protein electrophoresis in 4 patients (50%). Three patients (37,5%) had immune paralysis at diagnosis. Six patients (75%) had osteolytic bone lesions. Patient characteristics are summarized on
Table 1. One of the patients had normal serum IgD level at diagnosis but IgD lambda monoclonality was detected by immunoelectrophoresis. Four patients (50%) received VAD regimen as first line treatment, while the rest received bortezomib containing regimen. Three patients (37,5%) had progressive disease, 2 (25%) had VGPR, 2 had partial response, 1 (12,5%) had stable disease after first line treatment. During follow up 2 patients (25%) had undergone autologous stem cell transplantation (ASCT). Median overall survival was 26 months (range: 2-111 months). Five patients (62,5%) are still alive and resuming follow up while three patients died because of progressive disease. Conclusion: IgD MM can be presented with or without M spike so can be misdiagnosed as light chain MM. In order to diagnose this rare type of MM, serum and urine immunoelectrophoresis should be performed cause serum IgD can also be normal or lower than normal range in some cases. Prognosis is variable, this can be because of misdiagnosed disease or skip in diagnostic step. In our experience only 25% of patients had VGPR and ASCT was performed to 2 patients (25%), after that one of them had partial response and the other had progressive disease. Although IgD MM is considered to have an aggressive course and a poor outcome with chemoresistant disease, with the advances in myeloma treatment patients with long survival are reported. More studies are needed to determine if the new drugs and ASCT improve outcome of patients with IgD MM. Keywords: Multiple myeloma, IgD, immunoelectrophoresis.
Table 1 Characteristics of patients Characteristics Patients Age Gender Diagnosis
1
2
50
66
3 73
4
5
6
7
8
60
51
65
60
61
M IgD kappa
F IgD lambda
M IgD lambda
F IgD lambda
M IgD lambda
F IgD lambda
M IgD lambda
F IgD lamda
ISS Durie-Salmon Stage
1 1A
3 2B
3 3A
1 3A
1 3A
3 2A
3 3B
3 3B
M spike Bone lesion
(+) (-)
(-) (-)
(-) (+)
(+) (+)
(-) (+)
(-) (+)
(+) (+)
(+) (+)
(-) 9.3
(+) 8.5
(-) 12.1
(-) 11.6
(-) 11.1
(-) 7.6
(-) 6
17 1.18
322 0.59
193 0.8
161 1.07
120 20.2
172 5.1
63 4
110 4.6
108 4.5
54 3.5
3 2.6
9 3.8
Extramedullary involvement Hemoglobin (g/dl)
(+) 11.3
Platelet (x10⁹⁹/L) Serum creatinine (mg/dl)
183 0.77
245 4.5
eGFR (ml/min) Serum albumin (g/dl)
113 3.7
37 4 30.6
7
2.61
3.28
14.3
>20
Beta-2-microglobulin (mg/L) LDH level
2.32
11.3
N
N
H
N
N
N
N
N
Serum IgG Serum IgA
L L
L L
L L
N N
N N
L L
N L
L L
Serum IgM Serum IgD
L H
L H
L H
L H
L N
L H
L H
L H
First treatment regimen Response
VAD (6) SD
VAD (6) VGPR
Bor (1) Pr
Bor (5) Pr
VAD (3) Pr
Bor (8) VGPR
VAD (2) PR
Bor (6) PR
ASCT OS (months)/Statement (Alive/Dead)
(-) 111 (A)
(-) 44 (D)
(-) 2 (A)
(+) 13 (D)
(-) 7 (A)
(-) 39 (A)
(-) 2 (D)
(+) 47 (A)
Abbreviations: ISS: International Staging System, eGFR: estimated Glomerular Filtration Rate, LDH: serum lactate dehydrogenase, N: Normal, L: Low, H: High, VAD: Vincristine, Adriamycin, Dexamethasone, Bor: Bortezomib, SD: Stabil Disease, VGPR: Very Good Partial Response, Pr: Progression, PR: Partial Response, ASCT: Autologous Stem Cell Transplantation, OS: Overall Survival, A: Alive, D: Dead
15th International Myeloma Workshop, September 23-26, 2015
- e313