IgD Multiple Myeloma Research Articles

Characteristics of 15 Subjects Affected by IgD Multiple Myeloma and the Key Role of the Laboratory in Diagnosis: A Retrospective Study Report and Literature Review

Characteristics of 15 Subjects Affected by IgD Multiple Myeloma and the Key Role of the Laboratory in Diagnosis: A Retrospective Study Report and Literature Review

CLO24-090: Demographics and Outcomes of Autologous Stem Cell Transplant Among IgD Multiple Myeloma Patients.

CLO24-090: Demographics and Outcomes of Autologous Stem Cell Transplant Among IgD Multiple Myeloma Patients.

Analysis of efficacy and safety of BCMA chimeric antigen receptor T cells in the treatment of 5 patients with recurrent/refractory IgD multiple myeloma

Analysis of efficacy and safety of BCMA chimeric antigen receptor T cells in the treatment of 5 patients with recurrent/refractory IgD multiple myeloma

Anaplastic morphology in IgD multiple myeloma

Anaplastic morphology in IgD multiple myeloma

IgD Multiple Myeloma Complicated of Acquired Factor XI Deficiency: A Case Report

IgD Multiple Myeloma Complicated of Acquired Factor XI Deficiency: A Case Report

Renal insufficiency predicts worse prognosis in newly diagnosed IgD multiple myeloma patients.

IgD multiple myeloma (MM) is a rare type of MM, accounting for about 1%-2% of all MMs. IgD MM always causes kidney damage and even leads to renal failure, which is the most common complication. This study aimed to explore the risk factors of renal damage and prognosis of IgD MM patients. From March 2018 to November 2021, 85 patients with IgD MM diagnosed for the first time at the First Affiliated Hospital of Zhengzhou University were included in this study. We collected information on clinical features and laboratory examinations. Patients were divided into the renal impairment (RI) (47/85) and non-renal impairment (no-RI) (38/85) groups. Binary logistic regression was used to explore risk factors of renal damage. The Chi-square test was used to analyze the difference in chemotherapy effect between the two groups. We also analyzed whether early dialysis was beneficial to acute renal failure (RF) in IgD MM patients. Finally, Kaplan-Meier was used to compare the survival of the two groups. In IgD MM, 55.3% of patients had renal damage as a complication, of which up to 59.6% presented with acute renal failure as the first manifestation. Serum β2-microglobulin (β2-MG) was an independent risk factor for renal damage in IgD MM (p = 0.002), but cytogenetic analysis suggested that it had no effect on patients' renal damage. There was also no significant difference in the effect of chemotherapy between the two groups (p = 0.255). In patients with acute renal failure, there was no significant difference between dialysis and no dialysis groups in the proportion of patients with improved renal function after treatment. The median overall survival (OS) of the RI group was significantly shorter than that of the no-RI group (p = 0.042). In the RI group, the median OS was 29 months, and in the no-RI group, the median OS was > 40 months. Elevated serum β2-MG is an independent risk factor for renal damage. Compared with the no-RI group, patients in the RI group had poorer prognosis and shorter median OS. For patients with acute renal failure as the first manifestation, the treatment of primary disease is more meaningful than dialysis.

IgD Multiple Myeloma, Does It Still Have a Poor Prognosis?

IgD Multiple myeloma is considered unusual myeloma, with just 2% of cases of MM being diagnosed as IgD subtype. The infrequency of its presentation and the poor prognosis make the disease challenging to treat, with many patients being selected for clinical trials. A 63-year-old female presented to the ER after falling on ice. After undergoing a CT Head, multiple lytic lesions in the occipital region were found, confirmed by MRI. 2 months later, a bone marrow biopsy found 90% of marrow replaced by CD138 plasma cells, positive for kappa & negative for lambda, consistent with myeloma. On evaluation, she was found to have pan-hypo-gammglobulinemia. However, the skeletal survey did not find any other lytic lesions. Seeing this, IgD levels were measured and found to be >100 times over the upper normal limit with normal beta2-microglobulin levels, confirming monoclonal gammopathy. A FISH study found the patient positive for t(11;14)/CCND1IGH translocation (85% of cells) and negative for TP53. Palliative radiotherapy for the skull base was performed before the patient started chemotherapy. The patient was deemed a suitable candidate, selected for a clinical trial, and placed on RVd [revlimid + velcade + dexamethasone] with delayed bone marrow transplant. As the patient developed a rash and neuropathy from velcade [bortezomib], it was paused and restarted after three months. The patient was doing well on this chemotherapy regimen for two years and was placed on lenalidomide-based maintenance therapy. Two years later, her disease started progressing with an increased frequency of bone pains and opioid dosing. At this juncture, it was decided to change course and start her on pomalidomide/dexamethasone. This did not help the patient, and the neurological side effects of dexamethasone made us change course and initiate a daratumumab, bortezomib, cyclophosphamide and dexamethasone. This proved unfruitful, too, as the patient developed adverse effects of polyneuropathy, pancytopenia requiring transfusion, and worsening GERD. Seeing the disease relapse and intolerance to the previous regimen, we decided to initiate a once-per-week regimen of selinexor, bortezomib, and dexamethasone. The patient has improved on this regimen and is back to her baseline for physical activity, mental status and pain, and an improved appetite. In this case, we wanted to highlight how incidental findings on a CT lead us to diagnose a rare type of multiple myeloma and how, in the era of novel agents, the survival of patients can be prolonged significantly. Considering the rarity of this subtype, international collaborative studies are suggested to further elucidate the underlying mechanisms for developing potent therapeutic approaches. Most of the data reported on how IgD multiple myeloma progresses was written before the arrival of novel agents. More evaluation of survival times and progression of the disease needs to be done as it's possible that IgD MM is not as poor a prognosis as it once was.

IgD multiple myeloma: biology, diagnosis, and treatment

IgD multiple myeloma is uncommon. Patients generally present at a younger age and have shorter progression free and overall survivals (OSs). Its rarity has inhibited development of a specific risk stratification system or informed best treatment protocols. We present interphase fluorescence in situ hybridization results from a group of 29 cases. These showed evidence of a decreased male to female ratio, decreased OS in patients aged 70 and over, better outcomes in those with kappa light chain restriction, and CD56 positive patients had longer survivals than those lacking CD56. We discuss the biology of IgD multiple myeloma, the need for prospective studies, and challenges for improvements in diagnosis and treatment. We suggest an International Register to accelerate development of best practice guidelines for diagnosis, risk stratification, and treatment.

P317 FATAL EVOLUTION IN A YOUNG WOMAN DUE TO AMYLOIDOSIS HEART FAILURE IN A RARE MULTIPLE MYELOMA DISEASE

Abstract Introduction Immunoglobulin (Ig) D (IgD) monoclonal gammopathy is a rare subtype of multiple myeloma (MM) associated with a worse prognosis. Ig light chains amyloidosis is a disorder characterized by extracellular deposition of Ig light chains in various tissues, leading to organ dysfunction. Case A 29–year–old woman was admitted for dyspnea and tachycardia for moderate efforts. Lab revealed hypogammaglobulinemia with a monoclonal IgD K paraprotein. There was an increase in K free chains, with a dramatic increase in K/λ free chains Ratio. Proteinuria was 1360 mg/24h with a free K chains Bence Jones Protein. An increase in values of Troponin–I (137,5 pg/mL) and NT–proBNP (12527 pg/mL) pointed towards heart involvement. Furthermore, the ECG showed low voltages in the peripheral leads. Echocardiogram showed a moderate concentric left ventricular hypertrophy with diffused myocardial speckled pattern, an ejection fraction (EF) of 53% and PAPs of 71 mmHg and pericardial effusion. Chest CT scan showed bilateral pleural effusion. Spine MRI and whole body PET/CT indicated areas of osteolytic lesions. Plasma cells infiltration (85% CD138, MUM1 and K chains positivity) was present at bone marrow biopsy. Amyloid deposition was detected in abdominal fat tissue sample. Total bone scintigraphy excluded a transthyretin heart deposition. Thus, amyloidosis associate to IgD MM was diagnosed. In few days heart failure worsened (hs–cTnI 156,6 pg/mL; NT–proBNP 26583 pg/mL, EF 48%,) and the patient began complaining non–productive cough, dyspnea, and columnar edema of the lower limbs. She was not eligible for bone marrow transplantation, so daratumumab, bortezomib, melphalan and prednisone were administered. Despite serological improvement after a short five days course of therapy, she worsened with a further reduction of EF (35%). Blood gas analysis showed hypoxemia and lactate increase quickly turned into a cardiogenic shock. She died by cardiac arrest, just three weeks after admission. Discussion The present case is remarkable for age and gender of the patient as well as the rapid onset and fast worsening of symptoms, which were related to secondary heart amyloid deposition. Symptoms associated to cardiac amyloidosis are expressions of right heart involvement. The early good results of the therapy did not turn the evolution of disease. The fatal and overwhelming progression of the myocardial involvement led to the patient’s death in less than one month.

Criteria for Presumptive Diagnosis of IgD Multiple Myeloma

IgD myelomas constitute about 1% of myelomas, have worse prognosis, have higher association with lambda light chains, and are associated with excess free involved light chains. Standard immunofixation protocols stain only for IgG, IgM and IgA heavy chains. Proper attribution of a monoclonal gammopathy as having a delta heavy chain requires additional testing that is not available in most clinical laboratories. Retrospective review of protein electrophoresis and other laboratory results showed characteristic findings in IgD myelomas.

IgD multiple myeloma with central nervous system involvement: A case report and literature review.

Central nervous system (CNS) involvement is a rare manifestation of multiple myeloma (MM) and the optimal management strategies have yet to be determined. The aim of the present study was to describe the case of a 47-year-old male patient with immunoglobulin D-λ MM who presented with multiple extramedullary infiltrations at diagnosis. This patient achieved stringent complete response after 9 cycles of treatment with bortezomib, lenalidomide and dexamethasone, and then received lenalidomide as maintenance therapy. CNS involvement and extramedullary relapse developed 3 months after the last chemotherapy cycle. Despite receiving a second-line treatment protocol, the patient succumbed to the disease within 1 month after recurrence. The characteristics and treatment options for CNS MM are discussed in this case report.

Clinical Characteristics and Survival Analysis of Patients with IgD Multiple Myeloma

To explore the clinical features, prognosis and survival of patients with IgD multiple myeloma (MM). The clinical data of 20 patients with IgD MM was analyzed retrospectively. The prognostic factors and survival analysis was carried out. We summarized their clinical characteristics. The survival analysis was carried out by Kaplan-Meier method, and the prognostic factor were analyzed by using log-rank test for single factor analysis of observation index. Variables of P<0.15 in single factor analysis were enrolled in multifactor cox regression analysis. IgD MM patients accounted for 4.3% of all MM patients in the same period, among which 80% were male, the median age of patients was 57.5(35-77) years old, 90% of the patients belongs to λ light chain type. At the time of diagnosis, 18 patients (90%) were in DS-Ⅲ stages, while 10 patients were in ISS-Ⅲ stage. The first clinical manifestations were fatigue, bone pain, kidney function impairment, anemia (Hb<100 g/L) in 14 cases (70%), 12 cases (60%) with osteolytic bone destruction≥3, combined with renal impairment in 8 cases (40%), and elevated blood calcium in 11 cases (51.4%). In only 5 patients the ratio of albumin to globntin was inverted, hypoalbuminemia accounted for 40%, and globulin increase accounted for only 15%. FISH results showed that the positive rate of 1q21 amplification (50%) was the highest, and it was easy to occur at the same time as other cytogenetic abnormalities. Extramedullary infiltration occurred in 4 cases (20%). The analysis of prognostic factors showed that only the increase of lactate dehydrogenase (LDH) level was an independent poor prognostic factor for IgD MM patients. Extramedullary infiltration and various cytogenetic abnormalities were found in 2 IgD MM patients with primary drug resistance, suggesting that extramedullary infiltration and various cytogenetic abnormalities may be prognostic factors, but the difference was not statistically significant, Which maybe related to the small sample size. All 20 patients were treated with bortezomib-containing regimen, of which 19 patients were evaluated, 17 patients (89.4%) showed effective, including CR+VGPR (52.6%), PR (31.5%), MR (5.3%), 2 patients primary drug resistance. The median PFS and OS was 9.5 and 10.5 months, respectively. IgD MM is a rare and invasive disease. Increased LDH is an independent prognostic factor. Bortizomib-containing regimen can improve the prognosis of IgD MM patients.

Clinical characteristics and survival of patients with IgD multiple myeloma.

Clinical characteristics and survival of patients with IgD multiple myeloma.

Immunoglobulin D-Lambda Multiple Myeloma, and a Review of the Literature

IgD multiple myeloma (MM) is a rare plasma cell neoplasm, considered to have a poor prognosis compared to the other isotypes. Many studies reported an advanced stage at the presentation. In contrast to these studies, we report a case of rare IgD-Lambda MM at the early stage. The laboratory data showed no hypercalcemia, without any renal impairment, or monoclonal spike (M-spike or paraprotein) at the Serum protein electrophoresis (SEP) but only a hypogammaglobulinemia.

Light chain deposition disease involving kidney and liver in a patient with IgD myeloma

BackgroundIgD multiple myeloma (MM) is a rare subtype of MM and light chain deposition disease (LCDD) outside the kidney is also a rare and has scarcely been reported. We report herein the details of the first reported case of LCDD involving the kidney and liver co-occurring with IgD myeloma.Case presentationA 66-year-old female with IgD MM presented with rapidly progressive acute renal failure, ascites and pleural effusion. Immunofluorescent study of revealed the characteristic linear deposition of Igκ light chain along the glomerular and tubular basement membrane in kidney. Electron microscopy showed the powdery electron-dense deposits along the tubular and glomerular basement membrane consistent with the diagnosis of LCDD. Laser microdissection followed by mass spectrometry identified only Igκ light chain with more than 95% probability confirm the diagnosis of κ-LCDD but not heavy/light chain deposition disease. Liver biopsy with immunofluorescence study revealed the linear deposition of Igκ chain along the perisinusoidal space indicating the hepatic involvement of κ-LCDD. The patient was successfully treated with combination therapy with bortezomib, cyclophosphamide, dexamethasone, and daratumumab.ConclusionsThis report emphasizes that prompt biopsy of affected organs and initiation of clone directed therapy led to the correct diagnosis and favorable outcome in patient with LCDD who has extrarenal involvement.

A Rare Etiology of Chronic Polyradiculoneuropathy: IgD Multiple Myeloma

Introduction: Immunoglobulin D multiple myeloma (IgD MM) is a rare entity of multiple myeloma (2%), characterised by its clinical severity, renal presentation and poor prognosis. IgD myeloma remains an exceptional cause of chronic polyradiculoneuropathy. Observation: A 54-year-old patient, known to have type 2 diabetes since 2016, was admitted in December 2020 for non-febrile obnubilation associated with tetraparesis. The interrogation noted the occurrence of a motor deficit of the four limbs for 4 months in connection with a sensitivemotor polyradiculoneuropathy of the axon-myelin type of the four limbs. Glasgow’s score was 10. Blood pressure was 130/80 mmHg, pulse 116 beats/min, temperature 37°C, SaO2 92% on room air, low capillary blood glucose 1.08 and oliguria 70 cc/H. The physical examination revealed meningeal syndrome, tetraparesis, anaemia and renal oedema. Investigations revealed severe hypercalcaemia (140 mg/L), purulent meningitis (purulent liquid CSF, 12.38 g/L protein, 0.52 g/L glycorrhaphy, 14/mm3 leucocytes, 2000/mm3 red blood cells) and acute renal failure (74 mg/L creatinine, 5 mL/min/m3 GFR, 2.65 mg/L urea). Symptomatic IgD multiple myeloma was suspected on the basis of bone marrow plasmacytosis, bilateral frontal diploid lacunar osteolysis on CT scan and IgD positivity. Conclusion: Our observation illustrates the possible plurality of symptoms of IgD MM, particularly chronic polyradiculoneuropathy which is a rare neurological sign that should not be ignored in order to avoid diagnostic errors.

Descriptive Analysis of Isatuximab Use Following Prior Daratumumab in Patients with Relapsed/Refractory Multiple Myeloma

Background: Anti-CD38 monoclonal antibodies (mAb) like daratumumab (dara) have become integral in managing relapsed/refractory (RR) and newly diagnosed (ND) multiple myeloma (MM). Isatuximab (isa), a newer CD38 mAb, induces direct rather than indirect apoptosis of MM cells. However, little is known about whether the use of one prior CD38 mAb will alter the efficacy of another in subsequent lines of therapy. Methods: All patients (pts) with MM treated at MD Anderson with isa after receiving dara in prior lines of therapy were identified. We conducted a retrospective analysis with data points including patient and disease characteristics, responses to dara, response to isa, the presence of high risk features, and the presence of t(11,14). Results: 9 pts were identified, ages 56-72. 5 pts (55%) were male. 5 pts (55%) were alive at the time of data cutoff. 5 pts were Hispanic, 3 White, and 1 Black. 8 pts (89%) had high risk features as represented by the presence of del17p, t(4,14), t(14,16), t(14,20), p53 mutations, gain 1q, extramedullary disease (EMD), CNS disease, early relapse (within 1 year) after autologous transplant, or an increased (&amp;gt;5%) peripheral blood plasma cells (PBPC). 2 (22%) had t(11,14). 4 (44%) had IgG MM. 2 (22%) with light chain disease, 2 (22%) with IgA MM, and 1 (11%) with IgD MM. Dara was initially used in lines 2-7. Dara combinations with pomalidomide (pom), bortezomib (bor), thalidomide (thal), lenalidomide (len), or carfilzomib (car); and pom combinations that also included elotuzumab (elo) or Cytoxan (cytox) are noted in table 1. Dara was discontinued (dc'd) in 8 pts due to progressive disease (PD) and in 1 pt due to toxicity. 8 pts (89%) experienced a best overall response (ORR) of partial response (PR) to dara; 1 pt had stable disease (SD). All pts received prior len and 8 pts received prior pom at some time during the treatment of MM. All pts received isa in combination with pom/dexamethasone (dex). Best ORR to isa/pom/dex: 5 pt (55%) had PR, 2 pt with minimal response (MR), 1 SD, 1 PD. Median treatment duration of isa/pom/dex was 5 weeks (2-14 weeks) at data cutoff. 3 pts dc'd isa/pom/dex due to infections, and 2 due to later progression. 2 pts remain on therapy. 1 pt chose to dc all MM therapy for quality of life purposes despite PR with isa/pom/dex. 1 pt died from cardiac disease unrelated to MM or treatment. Conclusions: Our current study of heavily pretreated pts with RRMM demonstrates that despite prior anti-CD38 therapy with dara, most patients (77%) experienced a response of MR or better with treatment with another anti-CD38 therapy isa. To our knowledge, this is the first report of outcomes to isa in patients with prior dara therapy. Further long term follow up will be needed to determine the length of response. Additional studies are planned to further evaluate this patient population. Table 1 Disclosures Thomas: Pharmacyclics: Other: Advisory Boards; BMS: Research Funding; Ascentage: Membership on an entity's Board of Directors or advisory committees, Research Funding; X4 Pharma: Research Funding; Xencor: Research Funding; Genentech: Research Funding. Iyer:Rhizen: Research Funding; CRISPR: Research Funding; Spectrum: Research Funding; Merck: Research Funding; Curio Biosciences: Honoraria; Target Oncology: Honoraria; Afffimed: Research Funding; Daiichi Sankyo: Consultancy; Legend Biotech: Consultancy; Trillium: Research Funding; Seattle Genetics, Inc.: Research Funding. Patel:Celgene: Consultancy, Research Funding; Cellectis: Research Funding; Nektar: Consultancy, Research Funding; Oncopeptides: Consultancy; Poseida: Research Funding; Precision Biosciences: Research Funding; Takeda: Consultancy, Research Funding; Janssen: Consultancy, Research Funding; Bristol Myers Squibb: Consultancy, Research Funding. Manasanch:Adaptive Biotechnologies: Honoraria; GSK: Honoraria; Sanofi: Honoraria; BMS: Honoraria; Takeda: Honoraria; Quest Diagnostics: Research Funding; Merck: Research Funding; JW Pharma: Research Funding; Novartis: Research Funding; Sanofi: Research Funding. Kaufman:Janssen: Research Funding; Bristol Myers Squibb: Research Funding; Karyopharm: Honoraria. Lee:Genentech: Consultancy; GlaxoSmithKline: Consultancy, Research Funding; Takeda: Consultancy, Research Funding; Amgen: Consultancy, Research Funding; Celgene: Consultancy, Research Funding; Janssen: Consultancy, Research Funding; Sanofi: Consultancy; Daiichi Sankyo: Research Funding; Regeneron: Research Funding; Genentech: Consultancy. Orlowski:Sanofi-Aventis, Servier, Takeda Pharmaceuticals North America, Inc.: Honoraria, Membership on an entity's Board of Directors or advisory committees; Amgen, Inc., AstraZeneca, BMS, Celgene, EcoR1 Capital LLC, Forma Therapeutics, Genzyme, GSK Biologicals, Ionis Pharmaceuticals, Inc., Janssen Biotech, Juno Therapeutics, Kite Pharma, Legend Biotech USA, Molecular Partners, Regeneron Pharmaceuticals, Inc.,: Honoraria, Membership on an entity's Board of Directors or advisory committees; STATinMED Research: Consultancy; Founder of Asylia Therapeutics, Inc., with associated patents and an equity interest, though this technology does not bear on the current submission.: Current equity holder in private company, Patents &amp; Royalties; Laboratory research funding from BioTheryX, and clinical research funding from CARsgen Therapeutics, Celgene, Exelixis, Janssen Biotech, Sanofi-Aventis, Takeda Pharmaceuticals North America, Inc.: Research Funding.

Presentation Patterns, Diagnostic Markers, Management Strategies, and Outcomes of IgD Multiple Myeloma: A Systematic Review of Literature.

Immunoglobulin (Ig) D multiple myeloma (MM) is a rare subtype of MM comprising 2% of all the cases. Malignant plasma cell invasion leads to signs and symptoms similar to other subtypes of MM. The synthesis rate of IgD is lower in IgD MM patients, making it very difficult to diagnose compared to other subtypes. As there is no available diagnostic test with 100% accuracy, the diagnosis of IgD MM is based on multiple factors. Recent advances in the treatment have resulted in a better overall survival for IgD MM patients. The aim of this systematic review was to summarize the data on presentation patterns, diagnosis modalities, management strategies, and outcomes in patients with IgD MM.

Presentation Pattern, Diagnostic Markers, Management and Outcome of IgD Multiple Myeloma: A Systematic Review

Background:IgD multiple myeloma (MM) is a rare subtype contributing 2% of all multiple myeloma cases. Despite difficulty and delay in diagnosis, recent advances in the treatment of multiple myeloma in general also improved the outcomes for IgD myeloma subtype. The aim of this study is to summarize the data on presentation patterns, diagnosis, management approaches and outcomes for patients (pts) with IgD myeloma.Methods:A comprehensive literature search for articles published after December 2013 was performed using four databases: PubMed, Embase, Cochrane, and Clinicaltrials.gov. With initial search we identified 209 articles and after screening by two independent reviewers we included only 8 studies in the final analysis.Results:A total of 166 pts with IgD MM were included from 8 selected retrospective case series. Lambda (λ) was the predominant light chain sub-type in 136 patients (81.9%). Initial manifestations of IgD MM were Bence jones proteinuria (BJP>2 g/day-64.5%), renal dysfunction (63%), bone pain (55.9%), weakness, fatigue (34.2%) and extra medullary involvement (28.3%). Renal function was assessed by estimated glomerular filtration rate (eGFR<60 ml/min/1.73m2) in 54.3 % of patients and by serum creatinine level (Cr >2mg/dl) in 46.1% patients. Cytogenetic karyotype analysis in 56 pts using fluorescence in-situ hybridization (FISH) identified abnormal cytogenetics in 41 (73.2%) patients, cases were further classified as high risk (85.7%) and standard risk (14.3%). The patient characteristics and disease manifestations are mentioned in Table 1.Serum protein electrophoresis (SPEP) showed positive monoclonal spike (M) spike in 84% of the patients. The median M spike value for IgD MM was 9.42 g/l in a cohort of 17pts, (Djidjik et al.) which was lower as compared to IgG (median: 35 g/L) and IgA (median: 32 g/L) in this study. Abnormal serum free light chain ratio (sFLCR) was observed in 83% pts. Quantitative serum IgD levels were elevated only in 28% of the cases. Bone marrow (BM) plasmacytosis showing > 40% abnormal plasma cells was detected in 95.6% of the patients. N glycans are newly discovered biomarkers used for detecting abnormal protein glycosylation in MM patients. NG1(6)A2F and NG1(3)A2F were the two most significant N glycan markers for IgD MM patients with sensitivity of 95% & 95.2%, respectively and specificity of 95% & 78.6%, respectively. The prognostic significance of several other biomarkers studied in IgD MM is mentioned in Table 2.Treatment details were available for 149 patients and overall response rate (ORR) was seen in 125 (83.9%) patients while 24 (15.4%) patients had progressive or stable disease. The median overall survival (mOS) was between 9 - 62 months. Novel agents (NA) such as bortezomib, thalidomide and lenalidomide were given to 111 (74.4%) pts with the mOS between 15 - 38.6 months. Conventional agents (CA) such as melphalan, vinblastine, vincristine, epirubicin, and ifosfamide were given to 38 (25.5%) patients with mOS between 12.5 - 17 months. Data showed bortezomib based regimens resulted in a higher ORR (ORR=94%, CR=52%) compared to non bortezomib based regimens (ORR=77.8%, CR=27.7%) respectively. Stem cell transplant (SCT) was used in 37 (24.8%) patients with chemotherapy (NA=26, CA=11) which showed good response rate (ORR>90%,CR >60%). The comparison of response rate with different drugs is mentioned in the Table 3.Conclusion:N-glycan assay can overcome the limitations of SPEP and quantitative serum IgD assay in the diagnosis of IgD MM. This difficulty is posed by the lower concentrations of M spike ( SPEP) or lower-normal serum IgD value on quantitative serum assays, which can delay its diagnosis. Novel and conventional agents have good responses with ORR>90%, but mOS of conventional agents was lower when compared to novel agents. Bortezomib based regimens showed better responses when compared to non-bortezomib regimens. The best response (ORR=95%) was obtained with use of SCT in combination with novel agents. Serum free light chain levels, N glycan assay and serum IgD quantification were found to be the best prognostic markers for IgD MM. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Kliničke i laboratorijske odlike pojedinih imunoloških tipova multiplog mijeloma

Introduction: Multiple myeloma (MM) is a malignant blood disease, characterized by clonal proliferation of malignant plasma cells in the bone marrow and clinical manifestation of a disease that is heterogeneous and it's stratified by an internationally recognized Durie-Salmon classification (DS). Aim: The aim of this study was to determine whether the immune type of multiple myeloma affects the clinical and laboratory characteristics of the disease. Material and methods: The study included 45 newly diagnosed patients with MM (25 men and 20 women), the average age of 62.21 ± 8.62 years. A comparative analysis was performed between the class of M - protein and the mean value of the parameters contained in the DS classification. Results: The most common immune type was IgG MM. Considering gender, age, serum albumin values, total proteins, LDH, hemoglobin, beta 2 - MG, radiographic findings (number of osteolysis) and DS stages, there was no statistically significant difference in relation to individual immunological types MM. The IgD type was associated with a higher incidence of hypercalcemia than IgG and IgA type (p < 0.01) and with a higher incidence of renal insufficiency, compared to IgA MM (p < 0.01) and IgG MM (p < 0.05). Conclusion: Our findings exclude the association of Ig type of multiple myeloma with individual biochemical parameters, except for the increased incidence of hypercalcemia and renal insufficiency in IgD MM, which corresponds to literature data.