• Title/Summary/Keyword: Myeloma

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A Case of Amyloidosis Presenting as Lymphadenopathy at the Porta Hepatis (간문 주위 림프절병증으로 발현된 아밀로이드증 1예)

  • Lee, Ja In;Kim, Joon Sung;Kim, Byung Wook
    • The Korean journal of helicobacter and upper gastrointestinal research
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    • v.18 no.3
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    • pp.209-212
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    • 2018
  • We report a rare case of systemic amyloidosis with gastrointestinal and lymph node involvement. A 64-year-old woman was admitted to our hospital with dyspepsia and weight loss. Initial esophagogastroduodenoscopy (EGD) revealed nonspecific findings, and abdominal computed tomography showed necrotizing lymphadenopathy at the porta hepatis. Laparoscopic lymph node biopsy was performed under suspicion of tuberculous lymphadenopathy, but a definite diagnosis was not established. Follow-up EGD performed 6 months later revealed multiple telangiectasia-like lesions at the gastric body, and endoscopic biopsy revealed amyloid deposition. Through additional blood and urine protein electrophoresis, the patient was finally diagnosed with systemic amyloidosis associated with multiple myeloma. She was treated with dexamethasone, thalidomide, and bortezomib; however, she died 3 months after diagnosis because of pneumonia and multiple organ failure.

Robust Reference Intervals for Serum Kappa and Lambda Free Light Chains from a Multi Centre Study Population from Hyderabad, India: Myeloma Diagnostic Implications

  • Mohammed, Noorjahan;Chandran, Priscilla Abraham;Kandregula, Madhavi;Mattaparthi, Ratna Deepika;Gundeti, Sadasivudu;Volturi, Jyotsna;Darapuneni, Radhika;Raju, Sree Bhushan;Dattatreya, Palanki Satya
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.5
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    • pp.2605-2610
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    • 2016
  • The International Myeloma Working Group considers the serum free light chain (SFLC) assay to be an adjunct to traditional tests. Apart from the FLC ratio, the absolute values of individual free light chains also are gaining importance as they appear to be more relevant in certain clinical settings. Automated assays are available for their determination. As laboratories put new test systems into use catering to different disease populations, they are required by accreditation and certification bodies to verify or establish performance specifications, including reference intervals (RIs) representative of their population. Our aim was to establish local RIs for SFLC in a multicentre representative healthy population using a robust method. There was no significant relationship between SFLC levels and age, gender and creatinine levels. The 95% RI for ${\kappa}SFLC$ was 4.81 to 33.86mg/L, for ${\lambda}$ SFLC was 5.19 to 23.67mg/L and for ${\kappa}/{\lambda}SFLC$ was 0.36 to 2.33, significantly higher than the values given by the manufacturer. The ${\kappa}/{\lambda}$ SFLC ratio at 2.23, covering 100% of the data, showed 72% sensitivity (95% CI=39.0 - 94.0), 100% specificity (95% CI=71.5 - 100.0), 100% PPV (95% CI=21.5 - 100.0), 95% NPV (95% CI=75.4 - 99.9), and 79% accuracy (95% CI=56.0 - 93.0). In the patient group, kit RI for ${\kappa}/{\lambda}$ SFLC ratio classified 45.5% (n=5) as positive vs 9.1% (n=1) positive by the study RI, while the kit RI for kappa FLC classified 90.9% (n=10) as positive vs 54.5% (n=6), indicating increased probability of false positive test results with the kit RI when applied to our patient population. Appropriate and specific reference intervals and criteria values result in fewer false-positive and false-negative results which means fewer wrong or missed diagnoses.

A Systematic Review of MRI, Scintigraphy, FDG-PET and PET/CT for Diagnosis of Multiple Myeloma Related Bone Disease - Which is Best?

  • Weng, Wan-Wen;Dong, Meng-Jie;Zhang, Jun;Yang, Jun;Xu, Qin;Zhu, Yang-Jun;Liu, Ning-Hu
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.22
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    • pp.9879-9884
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    • 2014
  • Aim: The purpose of the current study was to conduct a systematic review of the published literature to evaluate the diagnostic accuracy of FDG-PET, PTE/CT, MRI and scintigraphy for multiple myeloma related bone disease. Methods: Through a search of PubMed, EMBASE, and the Cochrane Library, two reviewers independently assessed the methodological quality of each study. We estimated pooled sensitivity, specificity, positive and negative likelihood ratios (PLR and NLR), and two sample Z-tests were conducted to evaluate for differences in sensitivity, specificity, area under the curve (AUC), and the $Q^*$ index between any two diagnostic modalities. Results: A total of 17 studies were reviewed. The MRI had a pooled sensitivity of 0.88, specificity of 0.68, AUC of 0.897, and $Q^*$ index of 0.828, whereas for MIBI, the corresponding values were 0.98, 0.90, 0.991, and 0.962, respectively, and for bone scan, they were 066, 0.83, 0.805, and 0.740, respectively. The corresponding values of MIBI were 0.98, 0.90, 0.991, and 0.962, respectively. For PET and PET/CT, the values were 0.91, 0.69, 0.927 and 0.861, respectively. Statistically significant differences were not found in the sensitivity, specificity, AUC, and $Q^*$ index between MRI, scintigraphy, FDG-PET and PET/CT. Conclusions: On the condition that X ray is taken as a reference in our study, we suggested that FDG-PET, PTE/CT, MRI and scintigraphy are all associated with high detection rate of bone disease in patients with MM. Thus, in clinical practice, it is recommended that we could choose these tests according to the condition of the patient.

The Immunotyping Distribution of Serum Monoclonal Paraprotein and Environmental Impact on Multiple Myeloma (MM) and Monoclonal Gammopathy of Uncertain Significance (MGUS) in Taiwan: A Medical Center-Based Experience

  • Chang, Chih-Chun;Su, Ming-Jang;Lee, Shu-Jene;Tsai, Yu-Hui;Kuo, Lin-Yin;Lin, I-Hsin;Huang, Hui-Ling;Yen, Tzung-Hai;Chu, Fang-Yeh
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.1
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    • pp.395-399
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    • 2016
  • Background: Whether ambient exposure to environmental pollutants leads to hematopoietic malignancies such as multiple myeloma (MM) remains to be ascertained. Therefore, we aimed to investigate the immunotyping distribution of serum monoclonal paraprotein and the environmental influence on MM and monoclonal gammopathy of uncertain significance (MGUS) in the Taiwanese population. Materials and Methods: Serum protein electrophoresis with immunosubtraction by the capillary zone electrophoresis method was performed as primary screening for MM and MGUS. Clinical, pathological, and residence data of patients were also obtained. Results: From August, 2013 to June, 2015, a total of 327 patients underwent serum protein electrophoresis with immunosubtraction. Among these, 281 demonstrated no remarkable findings or non-malignant oligoclonal gammopathy, 23 were detected to have MGUS, 18 were identified as MM, and a further 5 were found as other malignancies. The most frequent immunotyping distribution of serum monoclonal paraprotein was IgG kappa (54.3%, n=25), followed by IgA lambda (15.2%, n=7) and IgG lambda (10.9%, n=5) in subjects with gammopathy. Additionally, it was shown that the elderly (OR: 4.61, 95% CI: 1.88-11.30, P<0.01) and males (OR: 2.04, 95% CI: 1.04-4.02, P=0.04) had significantly higher risk of developing MM and MGUS. There was no obvious impact of environmental factors on the health risk of MM and MGUS evolution (OR: 0.77, 95% CI: 0.40-1.50, P=0.49). Conclusions: The most frequent immunotyping distribution of serum monoclonal paraprotein included IgG kappa, IgA lambda and IgG lambda in MM and MGUS in the Taiwanese population. The elderly and male subjects are at significantly higher risk of MM and MGUS development, but there was no obvious impact of environmental factors on risk.

Highly Expressed Integrin-α8 Induces Epithelial to Mesenchymal Transition-Like Features in Multiple Myeloma with Early Relapse

  • Ryu, Jiyeon;Koh, Youngil;Park, Hyejoo;Kim, Dae Yoon;Kim, Dong Chan;Byun, Ja Min;Lee, Hyun Jung;Yoon, Sung-Soo
    • Molecules and Cells
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    • v.39 no.12
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    • pp.898-908
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    • 2016
  • Despite recent groundbreaking advances in multiple myeloma (MM) treatment, most MM patients ultimately experience relapse, and the relapse biology is not entirely understood. To define altered gene expression in MM relapse, gene expression profiles were examined and compared among 16 MM patients grouped by 12 months progression-free survival (PFS) after autologous stem cell transplantation. To maximize the difference between prognostic groups, patients at each end of the PFS spectrum (the four with the shortest PFS and four with the longest PFS) were chosen for additional analyses. We discovered that integrin-${\alpha}8$ (ITGA8) is highly expressed in MM patients with early relapse. The integrin family is well known to be involved in MM progression; however, the role of integrin-${\alpha}8$ is largely unknown. We functionally overexpressed integrin-${\alpha}8$ in MM cell lines, and surprisingly, stemness features including $HIF1{\alpha}$, VEGF, OCT4, and Nanog, as well as epithelial mesenchymal transition (EMT)-related phenotypes, including N-cadherin, Slug, Snail and CXCR4, were induced. These, consequently, enhanced migration and invasion abilities, which are crucial to MM pathogenesis. Moreover, the gain of integrin-${\alpha}8$ expression mediated drug resistance against melphalan and bortezomib, which are the main therapeutic agents in MM. The cBioPortal genomic database revealed that ITGA8 have significant tendency to co-occur with PDGFRA and PDGFRB and their mRNA expression were up-regulated in ITGA8 overexpressed MM cells. In summary, integrin-${\alpha}8$, which was up-regulated in MM of early relapse, mediates EMT-like phenotype, enhancing migration and invasion; therefore, it could serve as a potential marker of MM relapse and be a new therapeutic target.

Chemotherapy adherence is a favorable prognostic factor for elderly patients with multiple myeloma who are treated with a frontline bortezomib-containing regimen

  • Cho, Hee-Jeong;Seo, Sang-Kyung;Baek, Dong Won;Park, Sung-Woo;Lee, Yoo-Jin;Sohn, Sang-Kyun;Lee, Ho-Sup;Lee, Won Sik;Lee, Ji Hyun;Kim, Sung Hyun;Moon, Joon-Ho
    • Journal of Yeungnam Medical Science
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    • v.35 no.1
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    • pp.76-83
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    • 2018
  • Background: Elderly patients with multiple myeloma (MM) are vulnerable to adverse events (AEs). This study evaluated adherence to chemotherapy and treatment outcomes in elderly patients treated with a frontline bortezomib (BTZ), melphalan, and prednisone (VMP) regimen and regimens without BTZ. Methods: One-hundred and forty elderly patients who were diagnosed with MM from March 2007 to March 2015 were included in this retrospective study. To evaluate regimen adherence, patients who were treated with more than 4 cycles were assigned to the good adherence group. Results: Among the 140 patients, 71 were treated with a frontline VMP and 69 with non-BTZ regimens. The median age was 71 years (range, 65-90 years). The VMP group showed a higher complete response rate than the non-BTZ group: 26.8% vs. 7.2%. More patients in the VMP group achieved ${\geq}$very good partial response (VGPR) and ${\geq}PR$. In the VMP group, 27 patients (38.0%) received less than 4 cycles. The VMP good adherence group showed a higher 3-year overall survival (OS) rate (70.9%) than the poor adherence group (60.2%, p=0.059). In the multivariate analysis, treatment with ${\geq}4$ cycles of VMP was a favorable factor for OS. Conclusion: A good adherence to a frontline VMP regimen resulted in favorable long-term survival. Adequate management of AEs will be needed to achieve favorable outcomes in elderly patients with MM.

Unstable Pathologic Vertebral Fractures in Multiple Myeloma : Propensity Score Matched Cohort Study between Reconstructive Surgery with Adjuvant Radiotherapy and Radiotherapy Alone

  • Park, Hyung-Youl;Kim, Young-Hoon;Ahn, Joo-Hyun;Ha, Kee-Yong;Kim, Sang-Il;Jung, Jae-Woong
    • Journal of Korean Neurosurgical Society
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    • v.65 no.2
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    • pp.287-296
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    • 2022
  • Objective : Although radiotherapy (RT) is recommended for multiple myeloma (MM) involving spine, the treatment of choice between reconstructive surgery with RT and RT alone for pathologic vertebral fractures (PVFs) associated with structural instability or neurologic compromises remains controversial. The purpose of this study was to evaluate the clinical efficacies of reconstructive surgery with adjuvant RT for treatment of MM with PVFs by comparing with matched cohorts treated with RT alone. Methods : Twenty-eight patients underwent reconstructive surgery followed by RT between 2008 and 2015 in a single institution, for management of PVFs associated with structural instability of the spine and/or neurologic compromises (group I). Twenty-eight patients were treated with RT alone (group II) after propensity score matching in a 1-to-1 format based on instability of the spine, as well as age and performance. Clinical outcomes including the overall survival rates, duration of independent ambulation, neurological status, and numeric rating scale (NRS) for back pain were compared. Results : Clinical and radiological features before treatment were similar in both groups. The median survival period was similar between the two groups. However, the mean duration of independent ambulation was significantly longer in group I (88.8 months; 95% confidence interval [CI], 66.0-111.5) than in group II (39.4 months; 95% CI, 25.2-53.6) (log rank test; p=0.022). Deterioration of Frankel grade (21.4% vs. 60.7%, p=0.024) and NRS for back pain (2.7±2.2 vs. 5.0±2.7, p=0.000) at the last follow-up were higher in the group II. Treatment-related complications were similar in both groups. Conclusion : In patients with unstable PVFs due to MM, reconstructive surgery may yield superior clinical outcomes compared with RT alone in maintaining independent ambulation and neurological status, as well as pain control despite similar median survival and complications.

Polymyositis Associated with Pancreatic Ductal Adenocarcinoma

  • Yoon Suk Lee
    • Journal of Digestive Cancer Research
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    • v.10 no.2
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    • pp.112-116
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    • 2022
  • Idiopathic inflammatory myopathy (IIM) is known for its association with malignant diseases. Moreover, various solid organ malignancies, such as ovarian, breast, lung, esophageal, stomach, and colorectal cancers, have been reported to occur with IIM. Furthermore, its relationship with hematologic malignancies, including non-Hodgkin lymphoma, myeloma, and leukemia, has been reported. However, to date, IIM related to pancreatic cancer has scarcely been reported, particularly in patients with polymyositis (PM). Therefore, here we report a case of PM developed immediately after the diagnosis of pancreatic ductal adenocarcinoma.

Diffusion-Weighted Magnetic Resonance Imaging of Spine (척추의 확산강조 자기공명영상)

  • Young Cheol Yoon
    • Journal of the Korean Society of Radiology
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    • v.81 no.1
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    • pp.58-69
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    • 2020
  • In this study, we evaluated the technical characteristics and usefulness of diffusion-weighted magnetic resonance imaging for discrimination between benign and malignant vertebral fractures, for detection and differentiation of multiple myeloma or metastases, and for response monitoring in malignant vertebral lesions after anticancer drug therapy or radiation therapy.

Differential Protein Expression Profile Between CD20 Positive and Negative Cells of the NCI-H929 Cell Line

  • Geng, Chuan-Ying;Liu, Nian;Yang, Guang-Zhong;Liu, Ai-Jun;Leng, Yun;Wang, Hui-Juan;Li, Li-Hong;Wu, Yin;Li, Yan-Chen;Chen, Wen-Ming
    • Asian Pacific Journal of Cancer Prevention
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    • v.13 no.11
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    • pp.5409-5413
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    • 2012
  • At present, multiple myeloma (MM) remains an incurable disease and cologenic cells may be responsible for disease relapse. It has been proposed that CD20+/CD138- NCI-H929 cells could be hallmarks of MM clonogenic cells. Here, the immunology phenotype of NCI-H929 cells is described. Only a small population of CD20+/CD138- cells (<1%) was found in the NCI-H929 cell line, but CD20+/CD138- cells were not detected. We found that CD20+/CD138+ cells were able to exhibit cologenic capacity by colony formation assay and continuous passage culture. Proteins were analyzed by 1D-SDS-PAGE and TMT based quantitative differential liquid chromatography tandem mass spectrometry (LC-MS/MS). 1,082 non-redundant proteins were identified, 658 of which were differentially expressed with at least a 1.5-fold difference. 205 proteins in CD20+ cells were expressed at higher levels and 453 proteins were at lower levels compared with CD20- cells. Most proteins had catalytic and binding activity and mainly participated in metabolic processes, cell communication and molecular transport. These results proved that there are different biological features and protein expression profile between CD20+ and CD20- cells in the NCI-H929 cell line.