Amyloidosis is a disease characterized by deposition of extracellular fibrillar proteins in various tissues mainly derived from the mesoderm. We experienced a case of a 59-year old man with a localized bilateral mass at the angle of the mouth, which was surgically removed. It surrounded the facial arteries bilaterally with the same pattern, and was diagnosed as amyloidosis pathologically. Followed by further workup, he was finally diagnosed with the multiple myeloma. Cases such as this where an amyloidosis mass surrounded the main branches of the facial arteries has not yet been reported in the literature. We present a case of localized amyloidosis at the oral commisure surrounding the facial arteries with the review of its clinical patterns, diagnostic tools, pathologic findings and treatment.
Amyloidosis ; Arteries ; Mesoderm ; Mouth ; Multiple Myeloma ; Proteins

This study was purposed to investigate the expression of heat shock protein 90 (HSP90) in peripheral blood plasma of patients with multipl myeloma (MM), and to explore its possible role in the pathogenesis of MM, and its relationship with treatment, prognosis and the outcome of patients. The peripheral blood samples from 58 patients with MM and 20 healthy volunteers were collected. The plasma concentration of HSP90 in patients and healthy volunteers was measured by ELISA. The results showed that the concentration of HSP90 in peripheral blood of patients with MM was significantly higher than that in the healthy volunteers [(32.398 ± 3.674) vs (25.762 ± 2.916) ng/ml] (P < 0.001). The concentration of HSP90 showed positively correlation with International Staging System(ISS) stage, therapeutic response, frequency of plasmocyte, globulin, immune globulin, M-protein, β2 micro-globulin, and light chain of MM patients (P < 0.05) ; while it showed little correlation with sex, age and type of MM patients (P > 0.05) . It is concluded that the HSP90 may be involved in the occurrence and development of MM. Detection of HSP90 in plasma would contribute to judge the clinical course, therapeutic efficacy and prognosis of MM patients.
HSP90 Heat-Shock Proteins ; blood ; Humans ; Multiple Myeloma ; blood ; Myeloma Proteins ; Prognosis

Two M-protein peaks in serum protein electrophoresis are rarely present in patients with plasma cell discrasia. We describe a 72-year-old male patient with multiple myeloma secreting biclonal M-proteins, which were confirmed by immunofixation. Immunoelectrophoresis has some difficulties to dectect M components when a very small amount of M-protein develops an equivocally abnormal precipitation arc. In this case, serum protein electrophoresis revealed two M peaks, one in beta and the other in gamma globulin region. An immunoelectrophoresis revealed unequivocally abnormal precipitation arcs in IgA and K light chain regions, but the arc in IgG region was equivocal. We performed an immunofixation and confirmed biclonal gammopathy, IgA-K and IgG-K types. This result supports the view that immunofixation is an useful confirmatory test when immunoelectrophoresis results are equivocal.
Aged ; Electrophoresis ; gamma-Globulins ; Humans ; Immunoelectrophoresis ; Immunoglobulin A ; Immunoglobulin G ; Male ; Multiple Myeloma* ; Myeloma Proteins ; Plasma Cells

No abstract available.
Multiple Myeloma* ; Oncogene Proteins* ; Oncogenes* ; Proliferating Cell Nuclear Antigen*

Humans ; Multiple Myeloma ; Clinical Relevance ; Microfilament Proteins ; Biomarkers, Tumor

OBJECTIVETo investigate the relationship of WT1 and VEGF expression with angiogenesis in bone marrow biopsies of multiple myeloma patients.

METHODSVEGF, WT1 expression and microvessel density (MVD) of 62 cases of multiple myeloma and 10 normal bone marrow tissue were detected by in situ hybridization and immunohistochemistry SP method.

RESULTSMicrovessel density (MVD) of the control group was (45±6)/visual field, and while MVD of the multiple myeloma group was (84±26)/sight, and statistical analysis showed that MVD in multiple myeloma group was significantly higher than that in control group (P<0.05); in 62 cases of multiple myeloma the VEGF positive rate was 51.6% (32/62), and MVD in VEGF-positive group was significantly higher than that in the negative group (P<0.05). WT1 positive rate was 30.6% (19/62), and MVD in WT1-positive group was significantly higher than that in the negative group (P<0.05). And statistical analysis showed that WT1 expression significantly correlated with VEGF expression (P<0.05).

CONCLUSIONWT1 high expression of multiple myeloma bone tissue can up-regulate VEGF expression and promote angiogenesis.

Multiple myeloma(MM) as one of the most common tumors of hmatologic system, is characterized by malignant proliferation of plasma cells, and the chemotherapy is the main therapeutic method. MM is an incurable disease because of drug-resistance of MM cells. Although the pathogenesis of MM remains unknown, the chromosome abnormalities exit in half of the patients, particularly the highly expressed gene C-MYC. Furthermore, plenty of clinical researches indicated a high expression level of C-MYC implied worse progression and/or poor prognosis of MM. Recently, the work exploiting the compounds targeting MYC has made substantial progress, even in the MM therapy. In this article, briefly the recent advances of the research on C-MYC proto-oncogene in multiple myeloma are reviewed.
Chromosome Aberrations ; Genes, myc ; Humans ; Multiple Myeloma ; Plasma Cells ; Proto-Oncogene Proteins c-myc

OBJECTIVETo investigate the expression of PIGF and its receptor Flt-1 in patients with multiple myeloma, and to analyze their correlation with the efficacy of thalidomide-based chemotherapy so as to provide further theoretical basis for individualized treatment.

METHODSA total of 35 patients diagnosed as multiple myeloma from June 2012 to March 2013 in our hospital and 15 non-tumor patients as controls were enrolled in this study. MM patients were treated with thalidomide-based chemotherapy for 3 months, and then were grouped according to the curative effecacy. The expression levels of PIGF and Flt-1 were detected in bone marrow of control and MM patient group by RT-PCR and Western blot before and after chemotherapy, their correlation with chemotherapeutic efficacy was analyzed. Serum concentrations of PITG and Flt-1 were detected in control and MM patients before and after chemotherapy by ELISA and their correlation with the chemotherapeutic efficacy was analyzed.

RESULTSThe effective rate of three-months-thalidomide-based chemotherapy was 54.3% in MM patients. The expression levels of PIGF and Flt-1 in MM patients' bone marrow were obviously higher than those in controls. After chemotherapy, PIGF and Flt-1 expression levels significantly reduced and the decline level was positively correlated with curative effecacy(r=0.71). The serum concentrations of PIGF and Flt-1 in MM patients' bone marrow were obviously higher than those in control. After chemotherapy, serum concentrations of PIGF and Flt-1 were significantly decreased and the decline level positively correlated with curative effecacy(r=0.87).

CONCLUSIONPIGF and FLT-1 are highly expressed in patients with multiple myeloma, and their expression levels positively correlates with curative effecacy of thalidomide-based chemotherapy.

Cereblon（CRBN) is a brain-associated protein with ionic protease activity, which interacts with DNA damage-binding protein-1 (DDB1), Cullin 4 (Cul4A or Cul4B), and regulator of Cullins 1 (RoC1) to form the functional E3 ubiquitin ligase complex(CRBN-CRL4) that performs proteolysis via the ubiquitin-proteasome pathway. And CRBN is a necessary target protein for the anti-myeloma effect of immunomodulators. The combination of lenalidomide and CRBN recruited a new substrate that binds to the CRBN-CRL4 complex, leading to increased ubiquitination and proteasome-dependent degradation, thus resulting in anti-myeloma activity. The substrates binding to this complex are IKZF1, IKZF3 proteins and GS, etc. The CRBN-dependent degradation of IKZF1 and IKZF3 after lenalidomide treatment is also the result of HO-mediated oxidative stress. In addition to ubiquitination, lenalidomide also mediates ubiquitin-independent pathways that prevent CRBN from binding to CD147-MCT1 in a competitive manner to regulate its antitumor activity. Lenalidomide can also play a role in multiple myeloma(MM) cells by modulating miRNA levels and CRBN binding to downstream protein AGO2 expression. Thus, there are many molecular mechanisms of lenalidomide anti-myeloma activity. This review summarizes the molecular mechanisms of CRBN in lenalidomide against myeloma activity in terms of ubiquitin-dependent and ubiquitin-independent pathways.
Cullin Proteins ; Humans ; Hydrogen Peroxide ; Multiple Myeloma ; Peptide Hydrolases ; Proteolysis ; Thalidomide ; Ubiquitination

OBJECTIVETo investigate the expression of extracellular matrix protein Reelin (RELN) and its relationship with the prognosis of patients with multiple myeloma.

METHODSThe mononuclear cells were collected from bone marrows of multiple myeloma patients by Ficoll gradient density centrifugation, the CD138 cells were then purified by flow cytometry, the mRNA level of RELN was detected by real time PCR. The myeloma patients were divided into 2 groups according to the relative expression levels of RELN. Then the relationship of RELN expression level with clinical data was analyzed statistically.

RESULTSThe high expression of RELN significantly correlated with the percentage of CD138 cells, progress-free and total survival, but did not correlate significantly with DS (P>0.05) and ISS stages (P>0.05). The RELN high expression group showed higher levels of serum M protein, uric acid and serum calcium, lower hemoglobin, and more abnormal FISH results (including RB1 deletion, 1q21 amplification, IgH recombination, P53 deletion, D13S319 deletion) than those in RELN low expression group.

CONCLUSIONReelin correlates with the progression of multiple myeloma.