Diagnosis Of Myeloma Research Articles

P-155 Comparative analysis of outcomes in patients with CRAB versus SLiM criteria at diagnosis of multiple myeloma

P-155 Comparative analysis of outcomes in patients with CRAB versus SLiM criteria at diagnosis of multiple myeloma

Multiple Myeloma Prognosis From PET Images: Deep Survival Losses and Contrastive Pretraining

Objective: Diagnosis and follow-up of multiple myeloma (MM) patients involve analysing full-body Positron Emission Tomography (PET) images. Towards assisting the analysis, there has been an increased interest in machine learning methods linking PET radiomics with survival analysis. Despite deep-learning’s success in other fields, its adaptation to survival faces several challenges. Our goal is to design a deep-learning approach to predict the progression-free survival of MM patients from PET lesion images. Methods: We study three aspects of such deep-learning approach: i) the loss function: we review existing and propose new losses for survival analysis based on contrastive triplet learning, ii) Pre-training: We conceive two pre-training strategies to cope with the relatively small datasets, based on patch classification and triplet loss embedding. iii) the architecture: we study the contribution of spatial and channel attention modules. Results: Our approach is validated on data from two prospective clinical studies, improving the c-index over baseline methods, notably thanks to the channel attention module and the introduced pre-training methods. Conclusion and significance: We propose for the first time an end-to-end deep-learning approach, M2P2, to predict the progression-free survival of MM patients from PET lesion images. We introduce two contrastive learning approaches, never used before for survival analysis.

MM-368 Iraqi Metadata Analysis of Diagnosis, Risk Stratification, and Management of Multiple Myeloma

MM-368 Iraqi Metadata Analysis of Diagnosis, Risk Stratification, and Management of Multiple Myeloma

CircRNAs in diagnosis, prognosis, and clinicopathological features of multiple myeloma; a systematic review and meta-analysis.

Unlike improved treatment response in multiple myeloma (MM), the mortality rate in MM is still high. The study's aim is to investigate the potential role of circRNAs as a new biomarker for diagnosis, prognosis, and clinicopathological features of MM. We identified studies through Web of Science, Scopus, PubMed and ProQuest databases, and Google Scholar to August 2022. The SEN, SPE, PLR, NLR, DOR, and AUC were combined to investigate the diagnostic performance of circRNAs in MM. Also, HR and RR were used for prognostic and clinicopathological indicators, respectively. 12 studies for prognosis, 9 studies about diagnosis, and 13 studies regarding clinicopathological features. The pooled SEN, SPE, DOR, and AUC were 0.82, 0.76, 14.70, and 0.86, respectively for the diagnostic performance of circRNAs. For the prognostic performance, oncogene circRNAs showed a poor prognosis for the patients (HR = 3.71) and tumor suppressor circRNAs indicated a good prognosis (HR = 0.31). Finally, we discovered that dysregulation of circRNAs is associated with poor clinical outcomes in beta-2-microglobulin (RR = 1.56), Durie-Salmon stage (RR = 1.36), and ISS stage (RR = 1.79). Furthermore, the presence of del(17p) and t(4;14) is associated with circRNA dysregulation (RR = 1.44 and 1.44, respectively). Our meta-analysis demonstrates that the expression analysis of circRNAs is valuable for MM's diagnosis and prognosis determination. Also, dysregulation of circRNAs is associated with poor clinicopathological features and can be used as the applicable biomarkers for evaluating treatment effectiveness.

Disparities in Time to Diagnosis Among Patients With Multiple Myeloma

BackgroundMultiple myeloma (MM) is one of the most diagnosed hematologic malignancies in the United States. Despite improvements in therapy, health disparities persist among patients with MM. Here, we aim to determine whether there are disparities in time to diagnosis (TTD) among MM patients with regard to income, race/ethnicity, and gender. PatientsPatients with a monoclonal protein detected in the serum and/or urine and a subsequent bone marrow biopsy confirmed diagnosis of MM were included in the study. MethodsWe extracted data on patients with MM and assessed whether the predictor variables were associated with the primary outcome of TTD, which we define as the time between detection of a monoclonal protein in the serum or urine and bone marrow biopsy diagnosis of MM. ResultsCompared to patients with commercial insurance, patients receiving Medicaid (HR: 0.408, 95% CI: 0.206-0.808; P = .010) and patients without insurance (HR: 0.428, 95% CI: 0.207-0.885; P = .022) were significantly more likely to have delayed TTD. TTD was also prolonged if the provider who ordered the testing for the detection of a monoclonal protein was not a hematologist (HR: 0.435, 95% CI: 0.284-0.668; P < .0001). No disparities were found with regard to race/ethnicity or gender. ConclusionThis study suggests there may be socioeconomic disparities in TTD among patients with MM. Interventions such as patient navigation may be useful to reduce TTD among socioeconomically disadvantaged patient populations. Further studies need to be conducted to elucidate reasons for delays.

Under-representation of black patients with multiple myeloma in studies supporting International Myeloma Working Group guidelines

IntroductionMultiple myeloma (MM) is more common in Black persons when compared to non-Hispanic White persons. The International Myeloma Working Group (IMWG) provides consensus for diagnosis and treatment of MM. Our study aimed to assess the racial composition of supporting studies used by IMWG to publish their guidelines MethodsWe performed a cross sectional study that included all IMWG publications up to July 2022. References cited in each publication were reviewed. Review articles, comments, editorials, case reports, and animal-based studies were excluded. ResultsA total of 59 IMWG publications with 3956 references were reviewed. Final analysis included 2047 references of which 39 % (n = 804) were clinical trials, 35 % (n = 712) were observational studies, 20 % (n = 401) were diagnostic and or genetic testing-based studies, 3 % (n = 65) were population-based analysis and 3 % (n = 65) classified as others. Only 10.4 % of included references (n = 213/2047) reported race/ethnicity of studied patients. The total number of patients in all referenced studies were 5,747,920, only 2.6 % (n = 150,790) black patients. Of the trials referenced and done exclusively in the US, 41 out of 282 (14.5 %) reported race/ethnicity with a total number of patients of 38,050 of which 2493 (6.5 %) were black patients. ConclusionIMWG guidelines were based mainly on studies that did not include enough Black patients. Guidelines should consider inclusion of observational, diagnostic and population-based studies with more black patients to allow for better reflection of disease prevalence, clinical characteristics and/or outcomes.

Nonrestrictive diet does not increase infections during post-HSCT neutropenia: data from a multicenter randomized trial

AbstractInfections are a major cause of morbidity and mortality during neutropenia after hematopoietic stem cell transplantation (HSCT). The use of a low-microbial protective diet (PD) in the peritransplantation period is a standard of care, although its efficacy has never been tested prospectively. We conducted a multicenter, randomized, noninferiority trial, enrolling all consecutive adult patients undergoing high-dose induction chemotherapy or HSCT with the objective to compare nonrestrictive diet (NRD) vs PD. Overall, 222 patients were enrolled, randomly assigned, and analyzed. One hundred seventy-five subjects (79%) received autologous HSCT (auto-HSCT), 41 (18%) received allogeneic HSCT (allo-HSCT), and 6 (3%) patients received high-dose induction chemotherapy. There was no significant difference in terms of incidence of grade ≥2 infections and death during neutropenia in the 2 arms. In multivariable analysis, only multiple myeloma diagnosis, fluoroquinolone prophylaxis, and the absence of mucositis were associated with a lower incidence of grade ≥2 infections. We did not report any significant variation in terms of hospitalization length, incidence of mucositis and gastrointestinal infections, body weight, and serum albumin variations in the 2 arms. In allo-HSCT recipients, the incidence of acute graft-versus-host disease grade ≥3 was similar. NRD was associated with higher patient-reported satisfaction. In conclusion, NRD is not inferior to a traditional PD during neutropenia after HSCT, and our results demonstrated that implementing a restrictive diet unnecessary burdens patients' quality of life. The clinical trial was registered prospectively in the clinical trial registry of the Istituto Nazionale dei Tumori of Milan as INT54/16.

Features of diagnostics and treatment of various plasmacytoma options

Aim. Demonstration of clinical cases of plasmacytoma, both solitary and one of the manifestations of multiple myeloma. Materials and methods. A brief literature review on the diagnosis of bone and extramedullary plasmacytoma and clinical observations of various tumor options from the personal practice of the authors is presented. Results. Two clinical observations of the primary diagnosis of solitary plasmacytoma are presented. In one case, there was a bone tumor, in the other − an extramedullary tumor. In both observations, a generalization of the process was subsequently noted. Three clinical cases of primary diagnosis of multiple myeloma accompanied by the development of plasmacytoma are also presented. In two cases, there was a lesion of the bone marrow, in one – a multiple-focal form of the disease. In two situations, a non-secreting variant of multiple myeloma was diagnosed. The features of diagnosis and treatment were analyzed. Conclusion. In the diagnosis of focal forms of plasmacytoma (without lesions to the bone marrow and secretion), the leading role belongs to histological and immunohistochemical studies.

Features of routing, diagnosis and combined treatment of pathological fractures of long tubular bones of tumor etiology

Introduction. In the structure of fractures of long tubular bones, pathological fractures account for about 1 %, and 2/3 of all cases are pathological fractures associated with bone metastases. Primary bone tumors are complicated by the development of a pathological fracture in 1– 10 % of cases, bone metastases in 8–30 % of cases. With a pathological fracture, as the primary manifestation of the tumor process, as a rule, patients come to the attention of orthopedic traumatologists providing emergency care. In the practice of oncologists, there are often patients with bone tumors who have performed deliberately non-radical surgical interventions associated with insufficient oncological alertness of traumatologists.Aim. To study and present the results of combined treatment of patients with bone tumors complicated by a pathological fracture.Materials and methods. The results of treatment and observation of 159 patients with tumors of long tubular bones complicated by a pathological fracture or the threat of its development, who received combined or surgical treatment and were observed in the polyclinic of the Republican Oncological Dispensary from 2008 to 2020, were analyzed.Results. A pathological fracture that has taken place, in the absence of somatic contraindications, is not a contraindication to neoadjuvant treatment, which allows performing organ-preserving surgical treatment with optimal local and systemic control. However, with verified bone metastases, when segmental bone resections are not shown, and the diagnosis of myeloma (solitary plasmocytoma) is established, it is permissible to perform surgery in a trauma hospital, after consulting an oncologist.Conclusion. The combined approach in the treatment of malignant bone tumors complicated by a pathological fracture makes it possible to perform a functional-saving, organ-preserving surgical intervention, significantly reducing the risk of tumor recurrence and systemic progression.

Primary Cranio-Orbital Presentation of Multiple Myeloma.

A 49-year-old man presented with left upper eyelid ptosis noted after minor head trauma. Clinical examination revealed a soft, mildly tender mass of the superior orbit with left hypoglobus and relative proptosis. He had moderate limitation in supraduction, without visual or pupillary deficit. MRI imaging showed a superior extraconal cystic mass (Fig. 1C, D), and CT demonstrated erosion of the superior orbital roof and invasion of the frontal sinus (Fig. 1A, B).FIG. 1Excisional biopsy was performed via a bifrontal craniotomy with a frontal bone flap, exposing a well-encapsulated vascular lesion eroding through the orbital roof and frontal sinus (Fig. 2A). The mass was dissected from the surrounding dura, sinus mucosa, and periorbita, and removed in toto (Fig. 2B). The frontal sinus was obliterated and a split-thickness calvarial bone graft was used to reconstruct the orbital rim and roof, secured with mini plate fixation (Fig. 2C).FIG. 2Pathological analysis revealed plasma cell neoplasm (Fig. 2D) with clonal kappa-light proliferation (Fig. 2E), consistent with an extramedullary plasmacytoma. The full-body PET scan revealed multiple lytic lesions of the clavicle, rib, and vertebrae, conferring a diagnosis of multiple myeloma. He underwent an autologous stem cell transplant and started maintenance Bortezomib therapy with a good serologic response. Multiple myeloma accounts for 10% of hematologic malignancies and presents as a malignant plasma cell proliferation leading to bone marrow destruction. There are few reports of multiple myeloma initially presenting in the orbit. Solitary plasmacytoma without systemic disease may be treated with radiation therapy. Systemic disease mandates oncologic evaluation and consideration of chemotherapy or transplant.

Hepatocellular carcinoma presenting as a chest wall mass - An unusual case diagnosed on cytology with review of literature.

Hepatocellular carcinoma (HCC) is associated with an aggressive behavior and a strong tendency for extrahepatic metastasis. Although 5%-15% patients have metastases at diagnosis, presentation with symptoms exclusively related to extrahepatic metastases is rare. An 82-year-old male presented with an isolated left anterolateral chest wall swelling. Ultrasonography revealed a soft tissue mass involving the anterior chest wall with adjacent rib erosion. Serum protein electrophoresis showed increase in beta-2 region. A clinical diagnosis of multiple myeloma was considered. Fine needle aspiration cytology from the swelling showed loosely cohesive clusters of polygonal cells with traversing blood vessels. Cells showed abundant vacuolated to granular cytoplasm, round nuclei with frequent intranuclear cytoplasmic inclusions. A differential of metastatic HCC and renal cell carcinoma was considered. Subsequent imaging showed a 12 cm mass in the liver. Biopsy from chest wall mass with immunohistochemistry confirmed the diagnosis. Lungs and lymph nodes are the commonest sites for metastatic HCC; presentation as chest wall metastasis is rarely reported. The classical cytomorphology of HCC proved useful in diagnosing metastasis at a rare site. Recent studies have shown that beta-2-globulin is a promising biomarker for early diagnosis of HCC in patients with chronic liver disease.

Multiple Myeloma: Case of a “Moving” Sternal Wire

Multiple myeloma generally occurs in older adults, with the clonal proliferation of plasma cells and accumulation of monoclonal protein resulting in a broad range of clinical manifestations and complications, including hypercalcemia, renal dysfunction, anaemia, and bone destruction (termed CRAB features). A 64-year-old man with no history of malignancy presented with an enlarging precordial lump occurring three years post-sternotomy for uneventful coronary artery bypass grafting surgery. Initial investigations showed anaemia and impaired renal function. Multimodal imaging performed for further evaluation showcases the radio-pathological features which can be encountered in haematological malignancy. Subsequent percutaneous biopsy confirmed an underlying plasma cell neoplasm, and a diagnosis of multiple myeloma was achieved. The prompt resolution of the lesions upon the initiation of treatment highlights the importance of early diagnosis and treatment.

ANAPLASTIC MYELOMA IN TONSIL TISSUE: A CASE REPORT

A 61-year-old male patient, former-smoker and social drinker, presented a swelling in the left tonsil area, with an ulcerated surface, necrosis, bleeding, and no cervical lymphadenopathy. Previously, in another service, a diagnosis of chronic tonsillitis was given, but the lesion continued to grow. A new incisional biopsy was taken, which revealed plasmablastic neoplasia with lambda monoclonality. PET-CT showed glycolytic hypermetabolism in expansive lesions with bone lysis located in the frontal bone, in the roof of the right orbit, in the left tonsil, and in the sternal end of the left clavicle. The patient was referred to hematology, which performed clinical and laboratory examinations, concluding the diagnosis of anaplastic myeloma. He was initially treated with a VTD protocol and is a bone marrow transplant candidate. It was concluded that the multidisciplinary approach contributed to the final diagnosis of this rare case, initiating its early treatment and improvements treatment results. A 61-year-old male patient, former-smoker and social drinker, presented a swelling in the left tonsil area, with an ulcerated surface, necrosis, bleeding, and no cervical lymphadenopathy. Previously, in another service, a diagnosis of chronic tonsillitis was given, but the lesion continued to grow. A new incisional biopsy was taken, which revealed plasmablastic neoplasia with lambda monoclonality. PET-CT showed glycolytic hypermetabolism in expansive lesions with bone lysis located in the frontal bone, in the roof of the right orbit, in the left tonsil, and in the sternal end of the left clavicle. The patient was referred to hematology, which performed clinical and laboratory examinations, concluding the diagnosis of anaplastic myeloma. He was initially treated with a VTD protocol and is a bone marrow transplant candidate. It was concluded that the multidisciplinary approach contributed to the final diagnosis of this rare case, initiating its early treatment and improvements treatment results.

EXTENSIVE MANDIBULAR LESION AS A PRIMARY SIGN OF NEOPLASM OF PLASMA CELL ORIGIN: A CASE REPORT

A 57-year-old woman complained of jaw pain and presented no swelling on intraoral examination. Imaging examinations showed extensive osteolytic lesions throughout the mandible with signs of manifestation in the maxilla, including the involvement of other bones. Computed tomography revealed several hypodense osteolytic areas with cortical disruption in the left lingual posterior region of the mandible. Moreover, the incisional biopsy showed diffuse and monotonous layers of plasmacytoid cells with different degrees of differentiation. Immunohistochemical analysis showed positivity in plasmacytoid cells for anti-kappa, CD138, MUM1, and CD79a antibodies, and negative results for anti-lambda and CD20. The diagnosis of multiple myeloma was confirmed based on the histopathological and immunohistochemical data along with the imaging finding of diffuse lytic lesions. The patient was referred for further examinations and treatment. She remained under follow-up for six months after the biopsy and manifested a pathological fracture of the left clavicle, probably related to the underlying disease. A 57-year-old woman complained of jaw pain and presented no swelling on intraoral examination. Imaging examinations showed extensive osteolytic lesions throughout the mandible with signs of manifestation in the maxilla, including the involvement of other bones. Computed tomography revealed several hypodense osteolytic areas with cortical disruption in the left lingual posterior region of the mandible. Moreover, the incisional biopsy showed diffuse and monotonous layers of plasmacytoid cells with different degrees of differentiation. Immunohistochemical analysis showed positivity in plasmacytoid cells for anti-kappa, CD138, MUM1, and CD79a antibodies, and negative results for anti-lambda and CD20. The diagnosis of multiple myeloma was confirmed based on the histopathological and immunohistochemical data along with the imaging finding of diffuse lytic lesions. The patient was referred for further examinations and treatment. She remained under follow-up for six months after the biopsy and manifested a pathological fracture of the left clavicle, probably related to the underlying disease.

#6719 ACUTE KIDNEY INJURY AND MULTIPLE MYELOMA-THE ROLE CYTOGENETICS: A COHORT STUDY

Abstract Background and Aims Multiple myeloma(MM) is the 2nd most common haematological neoplasm and a largely heterogeneous malignancy in what regards cytogenetics, clinical presentation, therapy response and overall survival. Renal impairment related to MM is known to be diagnosed in at least 50% of patients during the course of this disease. Recent studies have shown an association between high-risk cytogenetic abnormalities(CA) with lower disease-free survival and lower overall survival. However, there is still scarce knowledge in what regards the kidney implications of MM CA. The purpose of this study is to identify an association between MM cytogenetic profile and acute kidney injury(AKI) at presentation. Method This was a single-centre retrospective cohort study of all adult patients diagnosed with MM between Jan./2018-Dec./2021 in a tertiary care university hospital in Lisbon. Diagnosis of MM was made according to the International Myeloma Working Group criteria. Patient variables were collected from individual clinical records. Categorical variables were described as the total number and % and continuous variables as median(P25-P75). Normally distributed continuous variables were compared with the Student-t-test, non-normally distributed continuous variables with the Mann-Whitney U-test and categorical variables with the chi-squared test. A multivariable backward stepwise regression model was performed to investigate predictors for AKI. Statistical significance was defined as p&amp;lt;0.05. Analyses were performed with the statistical software package STATA 16.0. Results 114 patients were included[median age 69.8±11.4 years, 48.1% male, 90.4% caucasian]. 61.5% had hypertension, 21.2% diabetes and 33.9% CKD with baseline GFR of 75.2(49.5-92.3)mL/min/1.73m2. 25% were on RAAS inhibitors, 5.8% on NSAID and 9.6% on bisphosphonate. The most common heavy chain was IgG(58.8%), the most common light chain was Kappa(52.4%) and 12.8% were isolated light chain-MM. 56.5% had a Revised International Staging System(R-ISS) of 2. Median serum M component was 4.5(2.8-7.9)g/dL, dFLC 247.8(48.9-1059.2) mg/L and plasma cells in bone marrow(BM) aspirate 39.0(15.5-67.0)%. 15.4% had hypercalcaemia, 68.9% Bence Jones protein, 3.8% secondary amyloidosis and 18.3% extramedulary disease. 38 patients had CA: 36.5% primary, 35.6% secondary and 33.7% high risk CA. Most patients were treated with proteasome inhibitors(75.0%), 26.9% were submitted to BM transplant and 59.2% had a complete or very good partial response to treatment. 13(12.5%) relapsed and 26(25.0%) died. AKI was present in 31.1% of patients and 11.7% needed renal replacement therapy.On univariable analysis, variables with impact on AKI were diabetes (34.3% vs 15.5%, p = 0.03), CKD (62.5% vs 21.1%, p&amp;lt;0.001), hypercalcemia (28.1% vs 9.9%, p = 0.018), higher R-ISS (p&amp;lt;0.001), beta 2-mycroglobulin (p&amp;lt;0.001), % of plasma cells in BM aspirate (p = 0.002) and dFLC (p = 0.01), lower baseline GFR (p&amp;lt;0.001) and the presence of CA (53.1% vs 28.2%, p = 0.015), primary CA (53.1% vs 28.2%, p = 0.015) and high-risk CA (46.9% vs 28.2%, p = 0.064). On multivariable analysis, the presence of CA[OR 3.094 (1.018-9.403), p = 0.046] and previous CKD[OR 8.069(2.430-26.796), p = 0.001] showed an independent association with AKI at presentation. Conclusion Our study suggests there is an association between CA and AKI at presentation, which may implicate a worse overall survival, have treatment implications and highlights the need for prevention and early diagnosis.MM cytogenetics is being studied since the 2000s and research is still on going in the attempt of creating a MM profile that can guide prevention, treatment and predict outcomes. It is known that renal impairment in MM particularly at diagnosis is associated with lower overall survival. However, to the best of our knowledge, the potential association between cytogenetic profile and AKI has not been thoroughly studied.

#6722 A RARE CASE OF ACUTE KIDNEY INJURY IN A PATIENT GRANULOMATOSIS WITH POLYANGIITIS, MONOCLONAL GAMMOPATHY WITH RENAL SIGNIFICANCE AND AMYLOIDOSIS

Abstract Introduction Anti - neutrophil cytoplasmic antibody /ANCA/- associated vasculitis is a small vessel vasculitis. It is a rare, autoimmune, inflammatory disease, with undetermined etiology, including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis. It could lead to pauci - immune necrotizing and crescentic glomerulonephritis and rapidly progressive glomerulonephritis. Monoclonal gammopathy of renal significance /MGRS/ is a plasma cell or B-cell disorder that could lead to renal impairment due to production of monoclonal protein, which do not meet the criteria for myeloma multiplex. There is a significant higher risk in progression to end stage renal disease/ESRD/ in groups with renal vasculitis and concomitant disease due to nonrenal factors. Clinical case We present a rare clinical case of a patient with combination of ANCA -vasculitis, MGRS and amyloidosis. A 60 year-old male patient with deteriorated renal function was presented to the clinic. Chronic congestive heart failure, arterial hypertension, situs inversus, anemic syndrome, hepatosplenomegaly were reported as concomitant diseases. Due to progressive worsening of renal function emergency hemodialysis treatment was initiated through temporary vascular access. Against the background of several chronic diseases, which could lead to chronic kidney failure, the echographic image of the kidneys stands out. Both kidneys are enlarged, with increased echogenicity of the parenchyma as in a diffuse renal process. The performed immunological tests confirm the diagnosis of granulomatosis with polyangiitis /formerly called Wegener's/, PR3-105,7. The results for the screened viral infections was negative. An episode of severe pulmonary edema was registered in the context of obstruction of the hemodialysis catheter due to coagulation disorders. The renal biopsy supports the diagnosis of vasculitis, crescentic glomerulonephritis - in advanced stage of development, probably a morphological manifestation of secondary nephropathy. An interesting finding is the presence of amyloidosis /AL/. The immunofixation blood test identify monoclonal IgG kappa type component. A sternal puncture, SPECT/CT was performed and diagnosis of multiple myeloma was excluded. Pathogenic treatment with corticosteroids and cyclophosphamide was initiated. In addition a short-term change in vision is observed in the context of the underlying disease. A consultation with ophthalmologist and neurologist was held, confirming cerebral arteritis. At this stage the recovery of renal function is incomplete. High flux membrane were used during dialysis treatment to reduce large and middle sized molecules. In addition, for the treatment of light chain amyloidisis, bortezomib was considered. Conclusion This clinical case emphasizes on the need to precisely clarify the cause that led to acute kidney injury. A thorough search for the underlying disease causing the deterioration of kidney function is critical for the renal outcome. The reasons for incomplete recovery of renal function during active treatment of vasculitis should be sought in underlying diseases-arterial hypertension, chronic heart failure, amyloidosis. Discussion Younger patients with lower eGFR at the onset of ANCA-associated vasculitis and no concomitant disease show significant improvement in renal function by the end of the first year regardless the need of dialysis treatment. Patients with severe accompanying disease have slow and insufficient improvement in renal function due to the prevalence of higher percentage of globally sclerotic glomeruli. Considering the severe prognosis of ANCA-associated vasculitis it is crucial to start early treatment. The state of the renal function at the first year of pathogenic treatment along with the pathological findings of the renal biopsy could be a predictors for the renal function course.

#5831 LIGHT CHAIN REMOVAL IN MULTIPLE MYELOMA PATIENTS WITH HFR-SUPRA HEMODIALYSIS: OUR EXPERIENCE

Abstract Background and Aims Kidney injury is a common complication in multiple myeloma (MM) and it has a negative prognostic implication. Most common cause of Acute Kidney Injury (AKI) in these patients is Light chain Cast Nephropathy, where free light chains precipitate in the tubules and bind with uromodulin, turning into intratubular casts that obstruct the tubules and also promote local giant cell reaction and interstitial inflammation and fibrosis. Free light chains (FLCs) can also damage the kidneys due to direct tubular toxicity when excessive amounts are reabsorbed by the proximal tubules. Targeted therapy to reduce FLC load can help recover renal function. Both total reduction and reduction speed are relevant for prognosis. FLC removal through extracorporeal techniques can be used as an adjuvant therapy, having an important part on the evolution of the disease. We gathered data from our experience treating MM patients with AKI with HFR-supra hemodialysis (HD) and analized the evolution and possible influence of this technique on renal recovery. Method This is an observational retrospective study. We included all patients with a diagnosis of multiple myeloma and acute kidney injury who received HFR-Supra hemodialysis in between years 2016-2022 in Hospital Virgen Macarena (Seville). We initially performed 6-10 daily HFR-Supra HD sessions and then modulated the frequency based on renal response (if renal replacement therapy had to be continued they underwent a usual hemodialysis regime 3 days a week). We continued these sessions until renal recovery was achieved or free light chain levels were reduced in agreement with the Hematology team. Measurement of pre and post dialysis FLCs was made always at first and last session and at least once in between, depending on the total number of sessions. Results 12 patients, with mean age at diagnosis 63.6 (43-86) years, presented with AKI stage KDIGO 3. 1 of them was oliguric. Median serum creatinine at diagnosis was 4.4 mg/dL [2,2-17], mean proteinuria was 4,1g/24 h [0,7-8,7] and 66,7% had positive Bence Jones proteinuria (mean 2,3g/24 h). 2 of the patients had previous chronic kidney disease stage 3a. All of them were diagnosed with Light Chain Multiple Myeloma (75% kappa, mean 10346 mg/L; 25% lambda, mean 5990 mg/L). Mean clonal bone marrow plasma cell was 20,7% [2-55]. According to the Revised International Staging System (R-ISS), 25% were stage 2 and 75% were stage 3. Renal biopsy was performed in 4 patients, all showed evidence of Cast Nephropathy. The indication for starting HFR-Supra HD was FLC removal in 9 patients, need of renal replacement therapy in 1 and both in 2 patients. We have experience in our center with using this therapy as an adyuvant treatment and often we start the technique in patients who present with AKI but would not necessarily have immediate need for renal replacement therapy. The goal is to remove FLCs and avoid further damage to the kidney tissue. Out of the 12 patients, 9 were able so stop dialysis (75%). They received a mean number of 12,4 [3-41] sessions in 3,7 [0,2-25] months). Free light chain removal per session was 24% on average [5-43%]. All of them were started on bortezomib-dexamethasone regime as initial chemotherapy for MM. As per renal recovery, at 3 months 33,3% achieved complete response, 11,1% partial response and 55,6% minimal response. At 1 year, 42,9% achieved complete response, 14,3% partial response and 42,9% minimal response. One-year survival rate was 91,7% (1 patient died from respiratory sepsis less than 1 month after diagnosis). Conclusion HFR-Supra hemodialyisis achieved a 24% free light chain removal per session on average. After presenting severe AKI (KDIGO 3), almost 43% of patients who received this adyuvant therapy obtained full renal recovery at 1 year and in 75% of patients withdrawal from hemodialysis was possible. 1 year survival was 91,7%.

#3709 KIDNEY IMPAIRMENT AT DIAGNOSIS IN MULTIPLE MYELOMA: THE NEPHROLOGIST PERSPECTIVE

Abstract Background and Aims Kidney impairment (KI) at multiple myeloma (MM) diagnosis ranges between 20 and 50% and is associated with high mortality. Therefore, an important percentage of the newly diagnosed cases of MM come from nephrologists. We aimed to evaluate the new cases of MM with KI diagnosed in our nephrology tertiary care center for presentation features, initial treatment, and survival. Method We performed a unicentric retrospective study on 89 consecutive patients newly diagnosed with MM (median age 66 years, 38% male) between 2015-2020. Patients were followed until death or end of study (September 2022), whichever came first. Results The most common MM type was free light chain (FLC) only (55%) followed by IgG (30%), IgA (14%) and IgM (1%). Kappa FLC was more frequent than lambda FLC (53 vs 47%); the median FLC level was 1760 (IQR 450, 7373) mg/L, and the median marrow plasmocytosis was 30 (IQR 15, 60) %. Among the 89 patients, 81% presented with acute kidney injury, 12% with chronic kidney disease, 5% with isolated proteinuia and 2% with nephrotic syndrome. Baseline median serum creatinine and eGFR were 5 mg/dL and 9 mL/min, respectively. Median proteinuria was 3.3 (IQR 1.3, 6) g/g with median albuminuria of 0.27 (IQR 0.11, 0.66) g/g. More than one third (34%) of the studied patients needed hemodialysis (HD) at diagnosis, and 50% progressed to renal replacement therapy by the end of the follow up period (CKD5D). The most frequent clinical features at presentation were asthenia (75%), followed by bone pain (51%), orthostatic hypotension (14%), peripheral neuropathy (10%), edema (10%) and dyspnea (6%). Arterial hypertension (71%), systemic atherosclerosis (58%), ischemic heart disease (44%) and diabetes mellitus (12%) were the most frequent recorded comorbidities. Treatment with dexamethasone was started by the nephrologist in 74% of the patients at a median dose of 128 (IQR 96, 160) mg, afterwards the patients were reffered to hematology for a bortezomib based regime. During the follow up period 58 (65%) patients died; they had higher serum creatinine (5.8 vs 2.5 mg/dL, p 0.02), needed HD at diagnosis more often (41 vs 20%, p 0.01), had lower serum albumin (3.7 vs 4.4 g/dL, p 0.001), higher LDH (236 vs 201 U/L, p 0.01) and increased inflammation (C-reactive protein: 14 vs 3 mg/L, p 0.001). In univariate logistic regression, the risk factors at diagnosis associated with mortality were increased age (OR 1.07, 95%CI1.03-1.12), atherosclerotic burden (OR 5.51, 95%CI 2.13-14.22), low serum albumin (OR 0.28, 95%CI 0.13-0.63), inflammation (OR 1.05, 95%CI 1.01-1.09), increased LDH (OR 1.01, 95%CI 1.00-1.02) and the need for HD at diagnosis (OR 2.94, 95%CI 1.04-8.26). However, in multivariate analysis only low serum albumin (OR 0.22, 95%CI 0.05-0.90) and the need for HD at diagnosis (OR 10.64, 95%CI 1.71-66.14) were significantly associated with mortality. Conclusion Patients with MM and kidney impairment at diagnosis are more frequent kappa FLC, present most often as AKI, around one third of them require HD at diagnosis, and half of them progress to CKD5D. Therefore, these patients are a distinct group of MM with a high mortality rate (&amp;gt;60% at five years) who need interdisciplinary care.

Multiple Facets of Multiple Myeloma in Kidney Biopsy: A Multicenter Retrospective Study.

Multiple myeloma is a type of plasma cell dyscrasia, which causes clonal proliferation of plasma cells and deposition in various organ systems. At presentation, 50% of patients with multiple myeloma have kidney dysfunction, which is considered a poor prognostic indicator. Data on the histopathological manifestations of multiple myeloma are sparse. To look at the kidney histopathological lesions in patients with the clinical diagnosis of multiple myeloma. A retrospective analysis of all kidney biopsies in patients with the clinical diagnosis of multiple myeloma was performed from June 1, 2020 to May 30, 2022, from three tertiary care nephrology referral centers. A total of 61 patients with multiple myeloma and biopsy-proven kidney involvement were included in the study. The mean age at presentation was 55.39 ± 11.91 years, with male predominance (male to female ratio -1.6:1). The most common lesion on kidney biopsy was myeloma cast nephropathy (72.1%), followed by light chain deposition disease (21.3%) and AL amyloidosis (18%). About 26% of patients had dual lesions on kidney biopsy, 3% had three types of lesions on kidney biopsy In 48% of patients, the diagnosis of multiple myeloma was made only after the kidney biopsy. Patients with multiple myeloma and kidney involvement should be biopsied as the type of histopathological lesion influences the treatment options and prognosis.

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.