Manifestation Of Multiple Myeloma Research Articles

Ophthalmic manifestations in hematological malignancies: An observational study from a tertiary care health center in Eastern India.

This study aimed to document the spectrum of ocular manifestations of hematological malignancies presenting to a tertiary health center in Eastern India and their association with blood parameters. This hospital-based cross-sectional study was conducted from August 2021 to July 2022. Patients diagnosed with leukemia, lymphoma, and multiple myeloma were enrolled in the study. A comprehensive ophthalmic evaluation was done in each case. A total of 97 patients with a confirmed diagnosis of hematological malignancies and meeting the inclusion and exclusion criteria were included in the study. Ocular manifestations were noted in 48 (49.48%) patients. Acute lymphocytic leukemia accounted for 35.4% of cases, followed by acute myeloid leukemia (31.25%), lymphoma (4.2%), and minimum manifestation in multiple myeloma (2.1%) patients. Among 48 patients with ocular manifestations, anterior segment involvement was found in 6.2% of cases, with subconjunctival hemorrhage being the most common, and the posterior segment was involved in 100% of patients, with intraretinal hemorrhages being the most common manifestation. A statistically significant association was noted between hemoglobin, total red blood cell count, and total platelet count with posterior segment manifestations ( p < 0.001). On multivariable logistic regression, only total leucocyte count and total platelet count were significant predictors for ocular manifestation. Indirect involvement of the retina is the most common ocular pathology in hematological malignancies, with intraretinal hemorrhages being the most common finding. Ophthalmic examination is highly recommended as a part of the routine evaluation at the time of diagnosis of hematological malignancies and periodically thereafter to diagnose any ocular involvement.

Clinical case of giant extramedullar plasmacytoma in a patient with multiple myeloma

One possible manifestation of multiple myeloma may be extramedullary plasmacytoma, manifested by infiltration of plasma cells outside the bone marrow. In the debut of the disease, bone plasmacytomas are most often diagnosed, in contrast to extramedullary ones, which are rare. According to the literature, the presence of extramedullary plasmacytoma is an unfavorable factor characterized by an aggressive disease course, the absence of an antitumor response to standard therapy, and low overall survival rates. Therefore, the pathogenesis, clinic, diagnosis and treatment of this variant of the disease require additional study. This article presents a clinical case of an aggressive course of extensive extramedullary plasmacytoma in a patient with multiple myeloma with an assessment of various combination therapy regimens efficacy.

Cardiac plasmacytoma: multi-parametric tissue characterization of a rare but serious manifestation of multiple myeloma.

Cardiac plasmacytoma: multi-parametric tissue characterization of a rare but serious manifestation of multiple myeloma.

Case Report: Spontaneous pneumothorax revealing multiple myeloma: a case report

Various causes like trauma, infection, pulmonary disease or neoplasm can lead to spontaneous pneumothorax. We report a rare case of a spontaneous pneumothorax as first manifestation of multiple myeloma. A 58-year-old patient presented suffering from dyspnea and right-sided chest pain, with no history of trauma. On examination, the patient had bilateral rib tenderness. The respiratory rate was 30 breaths/min and oxygen saturation was 88%. The chest physical exam revealed unequal breath sounds, an hyperresonance with percussion and decreased wall movement on the right side. The analysis of arterial blood gas revealed hypoxemia (arterial oxygen tension: 7.59 kPa) and hypercapnia (arterial carbon dioxide tension: 5.99 kPa). Laboratory data showed a raised C reactive protein level (133.8 mg/L), hyper-calcemia (serum calcium: 12.18 mg/dL) and a decreased plasma albumin level (31.9 g/L). Chest radiography and thoracic computed tomography revealed multiple ribs and sternum fractures leading to a partial pneumothorax on the right side. Subsequent workup for multiple myeloma showed elevated levels of immunoglobulin. Results of initial laboratory tests revealed an IgG gamma paraprotein, a urine protein electrophoresis of 1450 mg/24 hours and a β-2 microglobulin rate of 3.35. The diagnosis of multiple myeloma was confirmed with a bone marrow infiltration of 20% of atypical plasmatic cells. Cytogenetic investigations did not show any chromosomal abnormalities, especially the t (4,14) translocation. The patient was diagnosed with multiple myeloma stage IIIA according to Durie–Salmon classification. Appropriate treatment with oxygen therapy and systemic analgesic was started, associated with a cure of zoledronic acid in order to decrease the calcium level. The evolution was characterized by the complete resolution of the pneumothorax in 7 days and the normalization of the calcium level. The autologous stem cell transplant was the treatment of choice for this patient.

Cerebral Infarction as the First Manifestation of Multiple Myeloma and AL Amyloidosis: a Case Report

Cerebral Infarction as the First Manifestation of Multiple Myeloma and AL Amyloidosis: a Case Report

A prognostic model for patients with primary extramedullary multiple myeloma.

Background: Extramedullary disease is a manifestation of multiple myeloma, the prognosis of which remains poor even in the era of novel drugs. Therefore, we aimed to develop a predictive model for patients with primary extramedullary multiple myeloma (EMM). Methods: Clinical and laboratory data of patients diagnosed with primary EMM between July 2007 and July 2021 were collected and analyzed. Univariate and least absolute shrinkage and selection operation Cox regression analyses (LASSO) were used to select prognostic factors for overall survival (OS) to establish a nomogram prognostic model. The performance of the model was evaluated using concordance index which was internally validated by bootstraps with 1,000 resample, area under the curve (AUCs), and calibration curves. Results: 217 patients were included in this retrospective study. Patients with EMM had a higher rate of belonging to the male sex, age >50years, advanced Durie-Salmon stage III, hypercalcemia, and low hemoglobin level. Compared with patients with bone-related extramedullary disease, those with extraosseous-related extramedullary disease had a higher frequency of advanced Durie-Salmon stage III, lower rate of hypercalcemia, and elevated prothrombin time. The OS and progression-free survival (PFS) of patients with bone-related extramedullary disease were significantly higher than those of patients with extraosseous-related extramedullary disease. After the univariate and LASSO analyses, six prognostic factors, including performance status, number of extramedullary involved sites, β2-microglobulin, lactate dehydrogenase, monocyte-lymphocyte ratio, and prothrombin time, were integrated to establish a nomogram. The model showed robust discrimination with a concordance index (C-index) of 0.775 (95% confidence interval [CI], 0.713-0.836), internally validated with the corrected C-index of 0.756, and excellent performance in time-dependent AUCs compared with other staging systems. The AUCs for 1-, 3-, and 5-year OS were 0.814, 0.744, and 0.832, respectively. The calibration curves exhibited good consistency between the observed and nomogram-predicted OS. The 5-year OS of patients in the high-risk group (23.3%; 95% CI, 13.9%-39.3%) was much worse than that in the low-risk group (73.0%; 95% CI, 62.5%-85.4%; p < 0.001). Conclusion: The nomogram predictive model based on six clinical variables showed good prognostic performance and could better predict individual survival in patients with EMM.

Unusual Extramedullary Manifestation in Multiple Myeloma: Bilateral Synchronous Testicular Infiltration.

A 73-year-old man with multiple myeloma (initial diagnosis 21 months earlier) was referred to our center for a whole-body 18 F-FDG PET/CT. We detected a bilateral synchronous testicular manifestation, which was confirmed by histopathology after orchiectomy. Besides hypermetabolic lesions in the spine and ribs (most likely old fractures), known osteolysis showed no uptake. Extramedullary manifestation occurs in 13% to 20% of cases, among these 4% demonstrate testicular manifestation, which is associated with poor survival rates. Optimal therapy management is still unclear, due to limited data. To the authors' knowledge, so far only 3 comparable cases have been described.

Une vasculite leucocytoclasique au cours d’un myélome multiple

Leukocytoclastic vasculitis is a rare cutaneous manifestation in multiple myeloma and only twenty cases have been described to date. We report an unusual case with kappa IgA multiple myeloma in a 63 years old woman presented with leukocytoclastic vasculitis type of digital necrosis and leg ulcer. The vasculitis seems to be paraneoplastic as skin lesions regressed following multiple myeloma treatment.

Multiple myeloma and kidney disease

Abstract&#x0D; &#x0D; Background: Multiple myeloma is a clonal plasma cell neoplasm which affects 1% of all malignancies. In this article we reviewed epidemiology, symptoms with particular attention to renal failure and its manifestation in multiple myeloma (MM).&#x0D; Material and methods: This paper was based on medical articles collected in PubMed from 2001 to 2022, medical websites and books. The research has been done by looking through key words such as: „multiple myeloma”, „myeloma cast nephropathy”, „bortezomib”, „RANKL”&#x0D; Results: Multiple myeloma has indolent course and many patients present renal disorders at the moment of diagnosis. The use of novel therapies can reverse renal failure.&#x0D; Conclusions: Treatment of the patients with MM and kidney disease is challenging for health care professionals. Doctors have to adjust treatment to provide the best therapeutic effects.

Renal failure in patients with hematological malignancies (literature review)

Dysfunction of the natural detoxification organs remains a significant problem in patients with hematological malignancies. The reasons for the development of renal failure are associated with the individual characteristics of the malignant process, the patient’s comorbid background, the toxic effects of anticancer treatment and its complications. The efficacy of many anticancer drugs correlates with their dose, an increase in which is associated with increased toxic effects on healthy organs, including the kidneys. The main reasons for the renal failure development in hematological cancer patients and syndromes that prevent adequate antitumor therapy are considered. Diagnostic algorithm optimization and supportive intensive care of acute renal failure is the key to the successful application of highly effective modern protocols of drug anticancer treatment.A special group is represented by patients suffering from monoclonal gammopathies with acute renal injury and hyperproduction of immunoglobulins free light chains. Renal failure can be the onset and dominant clinical manifestation of multiple myeloma in 18–56 % of cases, of which 10 % require programmed hemodialysis. Antitumor therapy in presence of renal failure is limited, and in some cases impossible, while the renal function recovery is associated with an increase in survival.Organ damage in oncohematological patients can be a manifestation of paraneoplastic syndromes. Tumor lysis syndrome is an urgent problem of oncohematological practice associated with the development of acute renal injury and high mortality.The development of organ failure in oncohematological patients causes significant difficulties in antitumor therapy; a combination of organ dysfunction and the resulting change in anticancer therapy regimens worsen the prognosis. Modern methods of organ failure prevention and treatment can successfully solve complex clinical problems.

Prognostic impact of 18F-FDG PET/CT in patients with multiple myeloma presenting with renal impairment.

Renal insufficiency (RI) is a frequent manifestation of multiple myeloma (MM) at time of diagnosis but there is no reliable prognostic factor for patients with MM presenting with RI. This study investigated the prognostic impact of 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) in patients with MM with RI at diagnosis. The records of 209 patients with MM between June 2011 and November 2018 were retrospectively analyzed. PET/CT positivity was defined as the presence of more than three focal lesions or the presence of extramedullary disease. Of 209 patients, 90 (43.1%) had RI and showed similar survival outcomes to patients who had normal renal function. In total, 113 patients (54.0%) were PET/CT-positive, and 46.6% of patients with RI were PET/CT-positive at baseline. In patients with RI, those who were PET/CT-positive showed significantly inferior survival outcomes to those who were PET/CT-negative [progression-free survival (PFS), 12.7 vs. 34.0months, P < 0.001; overall survival (OS), 42.2months vs. not reached, P = 0.001]. On multivariate analysis, PET/CT positivity was significantly associated with PFS and OS in patients with RI. In conclusion, PET/CT is a reliable imaging technique for predicting survival outcomes in patients with MM with RI.

Resolution of high output pulmonary edema due to hyperviscosity with plasma exchange in a patient with multiple myeloma- a case report

Symptomatic hyperviscosity is an oncologic emergency which is suspected when patients with certain hematogical malignancies present with neurological symptoms. Hyperviscosity syndrome is a rare clinical manifestation in multiple myeloma. We describe a case of a 61 year old lady who developed pulmonary infiltrates after being diagnosed with multiple myeloma. Treated as an infection with antibiotics and intravenous immunoglobulin, she had neurological symptoms which suggested the possibility of hyperviscosity syndrome. The patient received one session of plasma exchange with which her symptoms improved and chest infiltrates resolved (high output pulmonary edema). The case highlights a known but rare manifestation of multiple myeloma, which when diagnosed and treated quickly can have gratifying results.

P0868ZONULIN AS A THERAPEUTIC POTENTIAL BIOMARKER OF ANEMIA IN PATIENTS WITH MULTIPLE MYELOMA AND CHRONIC KIDNEY DISEASE

Abstract Background and Aims Zonulin is a haptoglobin 2 precursor, that regulates the intestinal permeability. As a double-chain form it takes part in scavenging haemoglobin. Chronic inflammation is common complication of chronic kidney disease which affects iron metabolism. The most frequent manifestation of multiple myeloma is anaemia in up to 73% of patients. Serum concentrations of zonulin are associated with inflammation process, age, kidney failure and iron status and anaemia. The aim of the study was to analyse zonulin as a marker of anaemia in MM patients and investigate its relationship with acclaimed parameters of renal failure, inflammation, bone metabolism and stages of MM. Method The studied group of seventy-three patients with MM (67 symptomatic, 6 smoldering) included 35 women and 38 man, with mean age 69 ± 10 years. Median (IQR) of time from initial MM diagnosis was 36 (17; 69) months. Forty patients were in ISS stage 1, 15 in stage 2, and 12 in stage 3 at the time of blood collection. Remission of MM was diagnosed in 52 patients and stable or progressive disease in 21. Twenty-six patients had eGFR &amp;lt;60 ml/min/1.73 m2. The examined parameters included creatinine, urea, serum monoclonal protein, albumin, ferritin, blood hemoglobin and NT-proBNP. The association between zonulin, markers of MM stages and renal and bone markers were determined by the Pearson's test and multivariate stepwise regression analysis. The p-value &amp;lt;0.05 was considered statistically significant. Results Median (IQR) serum zonulin in the studied group was 23.9 (19.9; 27.4) ng/ml. There were no differences in zonulin concentrations between patients with smoldering versus symptomatic MM (p=0.4), with ISS 1 to 3 (p=0.7), with remission versus stable or progressive MM (p=0.9), or with eGFR &amp;lt;60 ml/min/1.73 m2 versus those with higher eGFR (p=0.6). Also, zonulin did not differ between subjects with and without anemia (Hb&amp;lt; the lower reference limit) (p=0.4). In whole studied group, significant correlations were observed between zonulin and serum albumin (R=0.30; p=0.009), creatinine (R=-0.28; p=0.018), eGFR (R=0.26; p=0.025), ferritin (R=0.34; p=0.013), NT-proBNP (R=-0.32; p=0.006). Moreover, in patients with symptomatic MM, zonulin correlated with monoclonal protein in serum (R=-0.29; p=0.046), blood hemoglobin (R=0.27; p=0.027), and age (R=-0.24; p=0.044). In multiple regression, serum concentrations of monoclonal protein (beta=-0.48 ± 0.16; p=0.006) and ferritin (beta=0.34 ± 0.14; p=0.023) as well as ISS stage 3 (beta=0.40 ± 0.18; p=0.034) were identified as independent predictors of zonulin concentrations. Conclusion Zonulin as a biomarker may promote diagnosis of etiology and management of MM-associated anaemia, which can contribute to biomarker-targeted therapeutic interventions. Early treatment may result in improved life expectancy in patients with MM and increase their quality of life.

Cold Agglutinin Disease in Non-Secretory Multiple Myeloma: A Case Report

Skin involvement is not common as the first manifestation of Multiple Myeloma (MM). Although extremely rare, leucocytoclastic vasculitis, plasmasytoma, autoimmune bullous disease, livedo reticularis and Raynaud’s phenomenon may be the initial presentation of MM. Raynaud’s phenomenon and livedo reticularis related to cold exposure can be due to Cold Agglutinin Disease (CAD) or cryoglobulinemia and can be seen as the first manifestation of MM. In this case study, we described a 55-year-old man complaining of limbs livedo reticularis and Raynaud’s phenomenon during cold weather. Further evaluations revealed anemia and elevated ESR. Skull X-ray showed multiple punched-out lesions. Finally, serum protein electrophoresis and bone marrow aspiration confirmed the diagnosis of non-secretory MM as the underlying disease of CAD. The patient was referred to the hematologist for the treatment of MM.

Successfully non-surgical management of flail chest as first manifestation of multiple myeloma: A case report

Successfully non-surgical management of flail chest as first manifestation of multiple myeloma: A case report

Plasmacytoma as a cause of small bowel obstruction in a virgin abdomen in a patient with multiple myeloma: a case report

BackgroundMultiple myeloma is a hematological malignancy that classically results in an abnormal clonal proliferation of plasma cells in the bone marrow. Extramedullary disease in the setting of multiple myeloma, referred to as secondary extramedullary plasmacytoma, is found in 7–17% of cases of multiple myeloma at the time of diagnosis and can involve any organ system. Small bowel obstruction is a rare but important gastrointestinal manifestation of multiple myeloma that should be considered in patients with multiple myeloma who present with concerning abdominal symptoms.Case presentationWe present the case of a 52-year-old African-American man with a history of deep venous thrombosis (he is on anticoagulation) and pathologic fracture secondary to multiple myeloma diagnosed 4 months prior to our encounter. He presented with abdominal pain, constipation, nausea, and vomiting. An abdominal X-ray showed distended bowel loops concerning for bowel obstruction and a contrast-enhanced computed tomography scan of his abdomen and pelvis showed a 5.4 cm soft tissue mass involving a loop of distal ileum. He underwent laparoscopic exploration of his abdomen with small bowel resection and primary anastomosis for a small intussusception. He had an uneventful postoperative course and was discharged on postoperative day 6.ConclusionsMultiple myeloma has myriad presentations. Gastrointestinal involvement, although rare, can manifest as small bowel obstruction for which early recognition and appropriate surgical management are key to improving outcome. Intussusception is the most common mechanism of obstruction from extramedullary plasmacytoma causing small bowel obstruction and this has been seen in five of six case reports, including this case. It is important to recognize and consider the risks of immunosuppression, venous thromboembolism, and malnutrition in the surgical management of gastrointestinal complications of multiple myeloma.

E026 Seronegative erosive arthritis as initial manifestation of multiple myeloma

E026 Seronegative erosive arthritis as initial manifestation of multiple myeloma

Epigenetics of Multiple Myeloma Bone Disease

Multiple myeloma bone disease (MMDB) is a devastating clinical manifestation of multiple myeloma associated with excessive bone osteolysis, which results from enhanced osteoclastogenesis and suppression of bone marrow stromal cell (BMSC) differentiation into osteoblasts. Impaired osteogenesis and functional alterations of myeloma-exposed BMSCs (MM-BMSCs) during the course of disease evolution significantly contribute to myeloma growth, metastasis, and chemoresistance. This review highlights new studies demonstrating that epigenetic modalities including chromatin-mediated gene silencing and non-coding RNA contribute to pathogenesis of MM-BMSCs. Inhibitors targeting histone-modifying enzymes EZH2, JMJD3, HDACs, and BET proteins have been successfully used to revert osteogenic suppression of MM-BMSCs. Aberrant expression of non-coding RNA cause functional changes associated with senescence, osteogenic suppression, and tumor-promoting phenotype of MM-BMSCs. Epigenetic events guiding transformations of the surrounding BMSC compartments are ultimately linked to disease onset and progression and open new therapeutic opportunity to target dissemination of MM tumors and reliably repair bone lesions.

Fluorine-18-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in a Giant Left Pectoral Muscle Plasmacytoma and Multiple Myeloma Case.

Extramedullary plasmacytoma is an unusual manifestation in multiple myeloma (MM). It can present as a solitary bone lesion and/or soft-tissue mass. Plasmacytoma can be presented at any location, but it is more common in the head and neck, usually without systemic involvement. The presence of plasmacytoma in MM is a predictor of rapidly progressive disease. The value of fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (PET-FDG) is increasing, in the diagnosis, detection of occult lesions, and therapeutic monitoring. We describe a patient with rapidly-progressive, refractory, left pectoral muscle plasmacytoma and MM. A PET-FDG guided the therapy and allowed to identify the presence of disease relapse.

Pemphigus vulgaris as the first manifestation of multiple myeloma: a case report

BackgroundThe association between pemphigus and malignancy has been well documented for decades but an association between pemphigus vulgaris and multiple myeloma is unusual. We report a case of pemphigus vulgaris revealing multiple myeloma.Case presentationA 55-year-old Malagasy man, with no significant past medical history, presented with bullous and erosive skin lesions involving his trunk and scalp for the last 2 months. He had no mucous membrane involvement. A diagnosis of pemphigus vulgaris was made on skin biopsy and direct immunofluorescence of perilesional skin revealing immunoglobulin G deposition in the intercellular spaces in the epidermis. In an enzyme-linked immunosorbent assay, his serum autoantibody index against desmoglein-1 and 3 was found to be 112 RU/mL and 34 RU/mL respectively. Serum immunoelectrophoresis showed a monoclonal gammopathy with a markedly elevated immunoglobulin G level (2880 mg/dL) in association with a lambda free light chain. Bone marrow aspirate showed 6% plasma cell infiltration. Further investigations, including creatinine blood test and whole body radiographic examinations, showed that he had initially clinical stage I multiple myeloma of the immunoglobulin G-λ type. Six months later, bone tomography revealed vertebral compression fractures of the thoracic and lumbar spine that correlated with his back pain topographically. Anti-myeloma treatment including melphalan and prednisone led to an immediate decline in monoclonal immunoglobulin G concentration. Skin and hematologic remission were maintained for 12 months.ConclusionsAbsence of mucosal involvement, lack of vacuolar degeneration at the interface, and absence of apoptotic, dyskeratotic keratinocytes ruled out paraneoplastic pemphigus in our case. Pemphigus vulgaris should be considered even if possible underlying disease for which paraneoplastic pemphigus is recognized is present.