Proliferation Of Monoclonal Plasma Cells Research Articles

PO-057 Genetic interaction and pathway based discovery of key regulators in multiple myeloma

IntroductionMultiple myeloma (MM) is a plasma cell dyscrasia characterised by proliferation of monoclonal plasma cells. As one of the most prevalent haematological malignancies, MM is genetically heterogeneous. Although 17 individual MM susceptibility loci have been identified, their inherent biological mechanism is sparsely understood. We carried out a polygenic interaction analysis with published whole-genome association studies (GWASs) to further characterise MM susceptibility with inflated genomic resolution in order to locate seemingly aberrant variations having temporal aggregation of risk.Material and methodsSingle nucleotide polymorphisms data from two MM GWAS representing 3999 cases and 7266 controls were subjected to genome-wide interaction analysis and followed-up with a meta-analysis. The identified loci were used for consequent gene set enrichment and tissue expression prioritisation to obtain functional evidence. Causal dependency between sentinel loci and functional variants was then interrogated with expression quantitative trait loci subject to summary data-based Mendelian randomization. Patient samples and relevant clinico-pathological information was obtained conditional on written informed consent and was subject to approval of corresponding ethical review boards at respective study centres in accordance with the tenets of Declaration of HelsinkiResults and discussionsA total of sixteen novel interacting pairs were identified along with indication of differential regulation in two principal signalling cascade families. Several previously identified and novel chromosomal regions influencing hematopoietic malignancies and contributing to pluripotent B-cell differentiation, survival, proliferation and apoptosis were identified. These loci were suggested to influence immune response and regulate immunoglobulin class-switch. Tissue/cell-type enrichment analysis showed that the genes associated with the identified loci were highly expressed in hemic-immune system tissue types and immune-related cell types.ConclusionOur polygenic interaction analysis renders novel insight into genetic susceptibility in MM. Related pathways provide some basic understanding of functional relation between discovered candidates as well as a biological basis of predisposition.

The potential function of microRNAs as biomarkers and therapeutic targets in multiple myeloma.

Multiple myeloma (MM), accounting for ~1% of all types of human cancer and 13% of all hematological malignancies, is characterized by the malignant proliferation of monoclonal plasma cells (PCs) in the bone marrow. MM leads to end stage organ impairment, including bone lesions, renal dysfunction, hypercalcemia and anemia. So far, the specific pathogenesis of MM remains unclear and no early-stage sensitive biomarker of MM has been well characterized. Furthermore, treating MM is difficult, as the majority of patients eventually relapse or become refractory following treatment using presently available methods. To date, a number of studies have demonstrated that microRNAs (miRNAs) may serve crucial functions in the progression of numerous cancers, including MM. During the tumorigenesis and pathogenesis of MM, there are multiple carcinogenic events that involve the pernicious transformation from normal to malignant PCs. miRNAs, as oncogenes or tumor suppressors, regulate MM progression-related signaling pathways. In the present review, the up-to-date preliminary basic studies and associated clinical works on the underlying mechanisms of aberrant miRNA profiling in MM have been summarized, including an evaluation of its value as a potential biomarker and a novel therapeutic strategy for MM.

Cardiac Light Chain Amyloidosis, Understanding the Implications of Cellular Toxicity in a 3D Model

Light chain (AL) amyloidosis is a fatal protein misfolding disease characterized by extracellular deposition of immunoglobulin light chains as amyloid fibrils. In AL amyloidosis there is an abnormal proliferation of monoclonal plasma cells that secrete high amounts of monoclonal light chains into the bloodstream. These light chains misfold, aggregate, and deposit in vital organs ultimately leading to organ failure and death. Survival rates for individuals diagnose with AL amyloidosis are often determined by the severity of cardiac involvement. There are two light chain subtypes (kappa and lambda). These subtypes are composed of variable (VL) and constant (CL) domains. Previous research in our laboratory demonstrated that different subtypes and species exert different cytotoxic effects in human RFP-AC16 cardiomyocytes. Soluble kappa proteins (particularly full length) cause apoptosis while fibrillar proteins (particularly VL) cause cell growth arrest. Mesenchymal stromal cells (MSCs) were shown to protect cardiomyocytes from cytotoxic effects of cell growth arrest exerted by fibrillar species. Currently, one major limitation in studying cardiac AL amyloidosis is the lack of an effective model that recapitulates the in vivo environment. In order to better understand the exact mechanisms behind apoptotic and cell growth arrest we have developed a 3D model with cardiomyocytes suspended in a diluted matrigel network. In 3D cell culture, the entire surface of the cardiomyocyte is exposed to its external environment providing an in vitro cell model that mimics cell-to-cell contact of the in vivo environment observed in AL amyloidosis patients. Interestingly, similar effects of clustering and cell growth inhibition observed in 2D cell culture were observed in 3D cell culture. 3D cell culture experiments in progress will determine the mechanisms behind fibrillar species cellular toxicity and the implications of MSCs as a potential therapeutic model.

Comparing five diagnostic criteria for multiple myeloma: a retrospective study of 227 cases.

Several diagnostic criteria for multiple myeloma are used in clinical practice, and it can be difficult to reach a diagnosis when a patient's clinical presentation is consistent with one criterion but not with another. However, no study to date has compared the superiority of the different diagnostic criteria. The aim of this research is to compare the efficacy of five diagnostic criteria for multiple myeloma and to find the reasons for misdiagnosis of atypical multiple myeloma cases. A total of 227 multiple myeloma cases were retrospectively studied. The clinical data (including plasma cell morphology, flow cytometry, immunofixation electrophoresis, imaging information and clinical manifestations) were scrutinized and the reasons underlying the misdiagnoses analyzed. The Traditional Domestic criteria had the highest misdiagnosis rate due to the high fixed bone marrow plasma cell percentage and serum M-protein thresholds. The WHO criteria and the International Myeloma Working Group 2009 criteria exhibited relatively low misdiagnosis rates due to their lower bone marrow plasma cell percentage thresholds, flexible criteria and detailed end-organ damage descriptions. The 2003 International Myeloma Working Group criteria and the 2011 Chinese Myeloma Working Group criteria exhibited perfect performance, as each focused on monoclonal plasma cell proliferation and not on fixed bone marrow plasma cell percentage and serum M-protein thresholds. The 2003 International Myeloma Working Group criteria and the 2011 Chinese Myeloma Working Group criteria have advantages in diagnosing early or atypical multiple myeloma cases. To avoid misdiagnosing some atypical cases of multiple myeloma, attention should be paid to evidence of monoclonal plasma cell proliferation, and flow cytometry may be a useful tool for discovering monoclonal plasma cell proliferation. Advanced imaging techniques should be used to confirm any suspected or atypical findings on metastatic bone survey.

Survival of patients with multiple myeloma diagnosed at the national center of hematology in Baghdad

Multiple myeloma (MM) is a monoclonal malignant proliferation of plasma cells derived from a single clone. Due to the variety of organ dysfunction caused by this malignant disease with no curative therapy, hence survival becomes a challenge for this group of patients. The aim of this study was to analyze the epidemiology and survival of patients with MM attended the out-patient clinic of the national center of hematology in Baghdad/Iraq. A retrospective study conducted at the national center of hematology, from September 2009 to November 2013 in which the medical records of 46 patients with MM have been reviewed. Survival analysis was estimated by the Kaplan-Meier and multivariate Cox regression analysis. The mean age was 63.4 years, and 54.2% of patients studied were female. The most common clinical manifestations were anemia (93.4%), bone pain (86.9%), and renal impairment (39.1%). In survival analysis, the only variable that achieved statistical significance was renal impairment (p = 0.025). For mortality, renal impairment (p = 0.017) and coagulation abnormalities (p = 0.012) were significant in the Cox regression. In conclusion the epidemiological profile showed a slight predominance in females. Anemia and bone pain were the most frequent complaints. Renal impairment and coagulation abnormalities were associated with mortality for patients with multiple myeloma

Objawy choroby tkanki łącznej u chorej ze szpiczakiem plazmocytowym – opis przypadku

Wstęp: Szpiczak plazmocytowy jest chorobą nowotworową układu limfatycznego, która charakteryzuje się proliferacją i gromadzeniem monoklonalnych plazmocytów wytwarzających zwykle monoklonalną immunoglobulinę. Występuje najczęściej u ludzi starszych. Pierwsze objawy są nieswoiste, należą do nich m.in. bóle kostne oraz osłabienie związane z niedokrwistością. W zaawansowanym klinicznie szpiczaku objawy wynikają na ogół ze współwystępowania zmian osteolitycznych, a także złamań patologicznych. Szpiczak może przypominać obraz wielu innych chorób, w tym także chorób reumatycznych.Opis przypadku: Przedstawiono przypadek chorej, która zgłaszała objawy choroby tkanki łącznej w przebiegu szpiczaka plazmocytowego.Wnioski: Objawy układowej choroby tkanki łącznej mogą wyprzedzać lub maskować objawy choroby nowotworowej. W przypadkach niecharakterystycznego przebiegu zawsze należy poszerzyć diagnostykę w kierunku procesu rozrostowego.

The histopathology of myeloma in the bone marrow.

Myeloma is characterized by the neoplastic proliferation of monoclonal plasma cells. A diagnosis of myeloma is based on the criteria proposed by the International Myeloma Working Group and the pathological findings.Myeloma cells are classified into four types: mature, immature, pleomorphic, and plasmablastic. There are three patterns in which myeloma infiltrates bone marrow - nodular, interstitial, and diffuse. Dutcher bodies are highly specific to neoplastic myeloma cells. On immunohistochemical staining, the specificity of CD138 is high for plasma cells. As a clear image is often not obtained from the immunohistochemical staining of the immunoglobulin light chain, in situ hybridization is recommended. Abnormal expression of CD56 is seen in 70-80% of cases by flow cytometry analysis. CD56 expression definitively indicates myeloma, suggesting its high diagnostic value. Evaluation of the infiltration pattern, monoclonality, and abnormal antigen expression of plasma cells is more important than the plasmocytic ratio to determine whether a case is reactive or neoplastic.Multiple gene abnormalities function in the onset and progression of myeloma. In our department, we analyze CCND1, FGFR3, MAF, and del (17p13) by FISH for all myeloma cases. None of the cases with genetic abnormalities were recognized by G-banding. Therefore, FISH is more effective than G-banding for the evaluation of genetic abnormalities in myeloma.

Primary extraosseous plasmacytoma of the parotid gland: A case report and literature review.

Extraosseous plasmacytoma (EOP) is an uncommon malignant tumour that is characterised by the monoclonal proliferation of abnormal plasma cells in soft tissue; however, EOP lacks the defining features of multiple myeloma or medullary plasmacytoma. Although the majority of EOP lesions occur in the head and neck, EOP of the parotid gland is extremely uncommon. The present study aimed to explore the clinical features of parotid plasmacytoma, in addition to the diagnostic and therapeutic options for its management. Using the Medline database, a search was conducted for articles published on the topic of ‘parotid plasmacytoma’ up until the year 2016. A total of 20 cases were evaluated, including 19 clinical cases from the literature and 1 new clinical case from our hospital. Among the 19 previously published cases, the mean age at the time of diagnosis of EOP was 65.1±10.9 years (range, 38–78 years). Plasmacytomas were located unilaterally in all cases: On the right side in 9 patients (47.4%), on the left side in 10 patients (52.6%). Treatment included chemotherapy in 3 cases, radiotherapy in 11 cases and surgical removal in 15 cases. The diagnosis of EOP is based on the presence of a localised tumour comprising monoclonal plasma cells, and EOP is identical to multiple myeloma in this regard; however, EOP, in contrast to multiple myeloma, does not exhibit the signs that are indicative of disseminated disease, such as additional lesions on skeletal radiological examination, plasmacytosis in the bone marrow, and hypercalcaemia, anaemia, or renal failure. Thus, EOP must be considered in the differential diagnosis of parotid gland lesions in order to avoid confusion with other tumoural diseases.

Down-regulation of long non-coding RNA MALAT1 by RNA interference inhibits proliferation and induces apoptosis in multiple myeloma.

Multiple myeloma (MM) is a neoplastic plasma-cell disorder characterized by abnormal proliferation of monoclonal plasma cells in the bone marrow. Metastasis-associated lung adenocarcinoma transcript 1 (MALAT1), an evolutionarily highly conserved long non-coding RNA was originally identified in metastatic non-small cell lung cancer and has been reported to be up-regulated in many other cancers. However, the function of MALAT1 in MM remains unknown. In the present study, by transfecting MM cells with MALAT1-specific short hairpin RNA (shRNA) expression plasmids, the role of MALAT1 in the proliferation and apoptosis of MM cells was investigated in vitro, and the tumorigenicity of MALAT1-silenced cells was evaluated in vivo. MALAT1 was found to be highly expressed in RPMI8226 and U266 cells. Down-regulation of MALAT1 via RNA interference significantly inhibited the proliferation of MM cells through cell cycle arrest at G1 phase. Moreover, knockdown of MALAT1 induced apoptosis, which was closely associated with the activation of caspase-3/-9, down-regulation of Bcl-2 and up-regulation of Bax. In addition, silencing of MALAT1 by intratumoral injection of MALAT1 shRNA attenuated the tumour growth in mice bearing myeloma xenograft and led to massive apoptosis in the xenograft tumour. Therefore, MALAT1 may serve as a promising target in the genetic therapeutic strategy for MM treatment.

Solitary Plasmacytoma.

Solitary plasmacytoma is a rare disease characterized by a localized proliferation of neoplastic monoclonal plasma cells, without evidence of systemic disease. It can be subdivided into solitary bone plasmacytoma if the lesion originates in bone, or solitary extramedullary plasmacytoma if the lesion involves a soft tissue. The incidence of solitary bone plasmacytoma is higher than solitary extramedullary plasmacytoma. Also, the prognosis is different: even if both forms respond well to treatment, overall survival and progression-free survival of solitary bone plasmacytoma are poorer than solitary extramedullary plasmacytoma due to its higher rate of evolution in multiple myeloma. However, the recent advances in the diagnosis of multiple myeloma can better refine also the diagnosis of plasmacytoma. Flow cytometry studies and molecular analysis may reveal clonal plasma cells in the bone marrow; magnetic resonance imaging or 18 Fluorodeoxyglucose positron emission tomography could better define osteolytic bone lesions. A more explicit exclusion of possible occult systemic involvement can avoid cases of misdiagnosed multiple myeloma patients, which were previously considered solitary plasmacytoma and less treated, with an unavoidable poor prognosis.Due to the rarity of the disease, there is no uniform consensus about prognostic factors and treatment. Radiotherapy is the treatment of choice; however, some authors debate about the radiotherapy dose and the relationship with the response rate. Moreover, the role of surgery and chemotherapy is still under debate. Nevertheless, we must consider that the majority of studies include a small number of patients and analyze the efficacy of conventional chemotherapy; few cases are reported concerning the efficacy of novel agents.

Strategy for the treatment and follow-up of sinonasal solitary extramedullary plasmacytoma: a case series

BackgroundExtramedullary plasmacytoma is a rare neoplasm characterized by monoclonal proliferation of plasma cells outside bone marrow. It accounts for 4% of all non-epithelial sinonasal tumors. According to the literature, radiotherapy is the standard therapy for extramedullary plasmacytoma. However, the conversion rate of extramedullary plasmacytoma to multiple myeloma is reported to be between 11 and 33% over 10 years. The highest risk of conversion is reported during the first 2 years after diagnosis, but conversion has been noted up to 15 years after diagnosis. Once conversion to multiple myeloma is complete, less than 10% of patients will survive 10 years.Case presentationWe present three cases of sinonasal extramedullary plasmacytoma who underwent radiotherapy: a 61-year-old white man, a 60-year-old white man, and a 37-year-old white woman. We found long-term survival with stable disease in all three cases.ConclusionsThe management of solitary extramedullary plasmacytomas of the sinonasal tract is not well established yet. However, the possibility of recurrence and progression to multiple myeloma requires a thorough follow-up protocol. Due to the absence of a standardized protocol for these tumors, we tried to design a tailored long-term follow-up scheme.

Mieloma múltiplo em uma cadela de 10 anos

Background: The malignant neoplasms, in general, constitute one of the main causes of death in dogs and cats. The multiple myeloma is a neoplasm characterized by the monoclonal proliferation of plasma cells in the bone marrow, causing several systemic symptoms and it is rare in pets, representing less than 1% of the malignant neoplasms in these animals. It usually occurs in older dogs. Thus, the purpose of this report was to describe a case of multiple myeloma in a 10-year-old poodle dog, occurred in the Veterinary Hospital Unit of the State University of Ceará.Case: A 10-year-old Poodle bitch was admitted in the Veterinary Hospital Unit of the State University of Ceará presenting lump erythematosus, alopecic, soft and ulcers in the region of the digits of the left front leg, pain and lameness. The blood count evidenced normocytic and normochromic anemia, presence of rouleaux, lymphopenia and monocytopenia, and the biochemical exams have demonstrated an elevated value of alanine aminotransferase (ALT). The cytology of the nodule indicated probable diagnosis of plasma cell neoplasm and radiographic examination showed the presence of bone lysis, and therefore the patient underwent amputation of the affected limb surgery. A biopsy of the nodule indicated neoplastic proliferation, with an arrange compatible to plasmacytoma of cleaved type. A follow-up consultation was realized 30 days after surgery, and 2 new nodules appeared in the superior lip region and close to the surgery site. A new blood count indicated anemia with presence of rouleaux, thrombocytopenia, leucopenia, lymphopenia and monocytopenia, and the biochemical results showed a discrete diminution of aspartate aminotransferase(AST) and a considerable increase on the alkaline phosphatase levels. The cytology of the new nodules indicated plasmacytoma, and was realized a myelogram, that showed dysplastic marrow plasma cells, with more than 20% of plasma cells.Discussion: In the present case, the patient’s lump grew rapidly with bleeding and ulcer formation. The animal presented bone involvement, which characterizes the most suggestive sign of multiple myeloma. Justified by the stimulating factor of osteoclasts, promoting bone lysis, which can cause pathological fractures and pain. The amputation was a partial treatment to attenuates the discomfort and pain presented by the patient. The appearance of new nodules stimulated a more detailed investigation for multiple myeloma. The enzyme AST presented a discrete diminution, and ALT initially was higher than normal, but then returned to normal values. So, the elevated activity of alkaline phosphatase directed the suspects to multiple myeloma, since the activity of AST and ALT was normal, the alkaline phosphatase level was ligated to bone diseases. The rouleaux was constant at the hemograms, this is justified by the excess of immunoglobulins produced in marrow. Based on the chart presented by the patient, the bone marrow was punctured for myelogram. The symptoms displayed by the animal along with the cytological analysis, histopathological, hematological, biochemical and with myelogram indicated diagnosis of multiple myeloma. Other exams could be investigated, for example, detection of Bence Jones proteinuria, serum calcium dosage and inmunoelectroforesis for determination of the type of immunoglobulin produced by the plasma cells in the bone marrow. The definitive diagnostic was established approximately three months after the first consult, emphasizing the importance of a multiple myeloma differential diagnostic in case of plasma cell neoplasm.

Anorectal involvement in a patient with multiple myeloma

Multiple myeloma is a neoplastic proliferation of monoclonal plasma cells. Symptomatic gastrointestinal involvement is uncommon. We report the case of a 45-year-old patient admitted with an anorectal polypoid lesion, which...

18FDG-PET Positivity Post Radiotherapy is a Biomarker for Relapse in Patients With Plasmacytoma

Solitary plasmacytomas are characterised by focal proliferation of monoclonal plasma cells in either soft tissue or bone. The management of solitary plasmacytomas is distinct from other plasma cell dyscrasias with radiotherapy forming the mainstay of treatment, with curative intent. Precise diagnosis of a solitary plasmacytoma is therefore essential, to ensure that the patient is treated on the appropriate pathway. Patients with solitary plasmacytoma can also progress with multiple plasmacytomas or Multiple Myeloma. Established risk factors for progression from solitary plasmacytoma to myeloma/multiple plasmacytomas are raised light chain ratio, aberrant plasma cells in marrow and size of plasmacytoma. Imaging biomarkers of relapse remains an active area of investigation. 18F FDG PET-CT has emerged as a useful tool to diagnose plasmacytomas and stage patients, but data is limited on use of 18F FDG PET-CT to measure treatment response. In Plasmacytoma patients, the role of residual PET positivity post therapy as a biomarker for disease burden and correlation with biochemical results, is not yet known. Likewise, the role of 18F FDG PET-CT as a biomarker to estimate the cure fraction and as a marker of disease progression requires further study. To answer these questions we conducted a retrospective analysis of twenty-four patients, referred with a histological diagnosis of plasmacytoma, over a three year period. We recorded patient baseline characteristics, size and location of their plasmacytoma, imaging findings, paraprotein/light chain and bone marrow results. We then compared their 18F FDGPET-CT and skeletal surveys to analyse what proportion of patients were subsequently upgraded to myeloma on imaging alone and whether their treatment pathway had changed. We also recorded the proportion of patients with solitary plasmacytoma who progressed with progression recorded on 18F FDG PET CT scan on follow-up imaging or until the first sign of biochemical relapse. Progression free and overall survivals were then calculated from time of diagnosis of plasmacytoma. Of the 24 patients, 9 patients were imaged with both skeletal survey and FDG PET-CT. On comparing the imaging findings, FDG PET-CT detected additional lesions in 55.6% of cases (5/9 patients), which were missed by conventional radiography and significantly changed treatment pathway. Follow up post treatment PET/CT was performed in 15/24 (62.5%) of patients. During the follow up period we found that 50% (12/24) patients with plasmacytoma progressed to Myeloma. In 66.7% of these cases, (8/12 patients) post therapy FDG PET-CT was positive, and only 1 PET-CT negative patient progressed. Only 50% (6/12 patients) of patients had biochemical evidence of either persistent disease or relapse at the time of clinical progression suggesting that FDG PET-CT is a useful imaging tool to identify plasmacytoma patients at risk of progression to Myeloma.

Retroperitoneal fibrosis: A rare manifestation of extramedullary dissemination of multiple myeloma

Multiple myeloma is a neoplasm that occurs due to monoclonal proliferation of plasma cells. Anemia, hypercalcemia, renal failure, and osteolytic lesions are the common manifestations. Less than 5% of patients present with extramedullary dissemination at the time of diagnosis. The common sites of extramedullary dissemination are liver, spleen, adrenals, and lung. Spread of myeloma into retroperitoneum causing retroperitoneal fibrosis is one of the rare manifestations. Patients with disseminated myeloma are usually young and have a poor disease-free survival rate. Aggressive therapy in the form of stem cell transplant has shown to be of benefit in such patients.

A case study of extramedullary plasmacytoma in a tertiary care centre

Extramedullary plasmacytoma is a rare soft tissue neoplasm characterized by monoclonal proliferation of plasma cells. We report a case of extramedullary plasmacytoma in a 45 year old male patient with a history of nasal obstruction and a polypoid mass was noticed in the Left Maxillary sinus. The mass was completely excised and histopathology, immuno-histochemistry findings lead to a diagnosis of Plasmacytoma of Left Maxillary Sinus

P2.04-047 A Rare Case of Extramedullary Plasmacytoma Occurring in the Posterior Mediastinum: Topic: Esophageal Cancer and Other Malignancies

Extramedullary plasmacytoma (EMP) is a rare neoplasm that is derived from a monoclonal proliferation of plasma cells in the soft tissues or organs outside the bone marrow and is present in about 3% of all plasma cell neoplasms. The average age of patients is about 60 years. The most frequent site is the upper respiratory tract (approximately 80%). The endotoracic forms usually manifest as nodules or pulmonary masses. Rarely may it present with mediastinal mass as a primitive solitary lesion. We present a case of extramedullary plasmacytoma of the posterior mediastinum. A 82 years old female presented to us with a history of chest pain, persistent cough, dysphagia, asthenia and dyspnea for few weeks. She denied smoking and had ischemic heart disease and atrial fibrillation as comorbidities. Her serum protein electrophoresis and hemocythometric parameters were normal. Chest X-ray showed a posterior voluminous endothoracic opacity and chest Computed Tomography showed an expansive hypodense lesion of the posterior mediastinum of 15.6 × 9.1 cm, which surround and displaced the thoracic aorta causing compression of the esophagus and central airways without pathological lesions of the lung parenchyma and the presence of a modest bilateral pleural effusions. Transbronchial needle aspiration under eco-endoscopic guide (EBUS-TBNA) has revealed the presence of isolated elements plasma cell-like (CD138+, lambda chains+). For a precise (correct) histological definition we performed a surgical biopsy of the mediastinal mass through right uniportal video-assisted thoracoscopic surgery (VATS). Pathological examination revealed solid tissue with massive infiltration of elements plasma cell-like. Immunohistochemical analysis showed positive staining for CD138 (plasma cell marker), CD56 (pathological plasma cells marker), monoclonal light chains lambda and negative for CD3 (marker of T line lymphoproliferative diseases). Therefore, a final diagnosis of extramedullary plasmacytoma was made. Our case of EMP without systemic signs of multiple myeloma is extremely rare, especially like a mediastinal mass as a primitive solitary lesion. In terms of posterior mediastinal manifestations of EMP, it should be differentiated from neurogenic tumor, lymphoma, and lymphangioma. 1 EMP could be concurrent with multiple myeloma or the sequences of proceedings for multiple myeloma, the prognosis is very poor and worse than primitive forms. Therefore, precise and timely framing (classification) of the disease is essential for diagnostic and therapeutic purposes.

New Strategies in Multiple Myeloma: Immunotherapy as a Novel Approach to Treat Patients with Multiple Myeloma.

Multiple myeloma is a B-cell malignancy characterized by proliferation of monoclonal plasma cells in the bone marrow. Although new therapeutic options introduced in recent years have resulted in improved survival outcomes, multiple myeloma remains incurable for a large number of patients, and new treatment options are urgently needed. Over the last 5 years, there has been a renewed interest in the clinical potential of immunotherapy for the treatment of multiple myeloma. Clinical progression of myeloma is known to be associated with progressive immune dysregulation and loss of immune surveillance that contribute to disease progression in association with progressive genetic complexity, rendering signaling-based treatments less effective. A variety of strategies to reverse the multiple myeloma-induced immunosuppression has been developed either in the form of immunomodulatory drugs, checkpoint inhibitors, mAbs, engineered T cells, and vaccines. They have shown encouraging results in patients with relapsed refractory multiple myeloma and hold great promise in further improving patient outcomes in multiple myeloma. This review will summarize the major approaches in multiple myeloma immunotherapies and discuss the mechanisms of action and clinical activity of these strategies. Clin Cancer Res; 22(24); 5959-65. ©2016 AACR.

Possible intrinsic association of anti-neutrophil cytoplasmic antibody-associated vasculitis coexisting with multiple myeloma.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a life-threatening condition that causes renal failure. Multiple myeloma (MM) is a malignant proliferation of monoclonal plasma cells in the blood that can also cause renal failure. The two diseases have high morbidity and mortality rates in the elderly, with a poor prognosis. A 64-year-old female presented to Xinqiao Hospital (Chonqing, China) with fatigue and a poor appetite that had been apparent for 6 weeks. Laboratory tests revealed a serum creatinine level of 10.31 mg/dl, a cytoplasmic ANCA titer of 1:10, a positive result for myeloperoxidase and a serum globulin level of 3.96 g/dl. A renal biopsy revealed crescent glomerulonephritis, combined with the rapid progression of renal function. Based on these observations (ANCA titer, crescent glomerulonephritis and rapid progression of renal function) a diagnosis of AAV was established. MM was confirmed by serum immunofixation electrophoresis combined with bone marrow aspiration. The present study discusses what is, to the best of our knowledge, the first case of AAV coexisting with MM in order to highlight it as a clinical concern.

Extramedullary Plasmacytoma of Nasal Cavity

ABSTRACT Extramedullary plasmacytoma is a rare neoplasm characterized by monoclonal proliferation of plasma cells in soft tissues. Extramedullary plasmacytomas constitute 3 to 5% of all plasma cell neoplasms. Most lesions occur in the head and neck, primarily in the upper aerodigestive tract, with 75% occurring in nose and paranasal sinuses. To the best of our knowledge, only few cases of sinonasal extramedullary plasmacytomas have been reported in the literature. It is important for otolaryngologists to acquire knowledge of this disease as 80 to 90% occur in the head and neck region. We report a rare case of extramedullary plasmacytoma of the nasal cavity in a 60-year-old male managed by surgery and radiotherapy. How to cite this article Tandon S, Meher R, Chauhan A. Extramedullary Plasmacytoma of Nasal Cavity. Clin Rhinol An Int J 2016;9(1):50-52.