Monoclonal Immunoglobulin Deposition Research Articles

Monoclonal Membranous Glomerulopathy in a Patient with Positive Serology for Lupus Nephritis

Abstract Introduction/Objective Monoclonal membranous glomerulopathy (M-MGN) is a rare entity; only two reports are available in the literature. Monoclonal membranous glomerulopathy has been called either monoclonal immunoglobulin deposition disease associated with membranous features in a 3-patient small series report or membranous glomerulopathy with light chain restriction in another larger series with 28 cases. We report M-MGN in a patient with positive serology lupus. Methods/Case Report The patient was a 74-year-old man with hypertension and nephrotic range proteinuria (protein/creatinine ratio at 8.5). His serum creatinine remained normal at 0.6 mg/dL. The patient was positive for double-stranded DNA and ANA at low titer, but had reduced complement C3 and C4. Immunofixation was negative for monoclonal protein. The light microscopy of the renal biopsy revealed thickened loops of glomeruli with mild interstitial nephritis. Immunofluorescent studies showed a positive linear/granular pattern of IgG and lambda but negative kappa staining along the glomerular loops. Electron microscopy revealed smooth contoured stage 2 subepithelial deposits. An outside consultation confirmed our diagnosis and found IgG2 restriction in glomeruli. Electrophoresis revealed no monoclonal protein in the serum. Results (if a Case Study enter NA) NA Conclusion Our patient demonstrated M-MGN with mild interstitial nephritis; the latter may be related to his lupus status. As 30% of patients with M-MGN have either monoclonal serology or B-cell lymphoproliferative disorders, with most having a restricted IgG subtyping, a close follow-up and even chemotherapy for a B-cell clone should be considered.

Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits in a Patient with Recent Deceased Donor Kidney Transplant Successfully Treated with Rituximab Monotherapy

Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits in a Patient with Recent Deceased Donor Kidney Transplant Successfully Treated with Rituximab Monotherapy

Dépôts tissulaires non amyloïdes d’immunoglobuline monoclonale (hors atteinte rénale)

Monoclonal immunoglobulins can form deposits other than amyloidosis in various tissues. Immunofluorescence is the key analysis for the identification of monoclonal immunoglobulin deposits. Pulmonary light chain deposition disease is a diagnosis to be considered when dealing with diffuse cystic lung disease.

Cytogenetic abnormality in non-systemic light chain amyloidosis monoclonal gammopathy of renal significance: A field needs to be explored.

The role of cytogenetic abnormalities in non-systemic light chain amyloidosis monoclonal gammopathy of renal significance diseases still needs to be clarified. Bhutani etal. present the results of a study investigating the underlying plasma cell cytogenetic abnormalities in monoclonal immunoglobulin deposition disease (MIDD). The results show that translocation (11;14) is a common abnormality in MIDD and affects the presentation and outcomes. Commentary on: Bhutani etal. Translocation (11;14) is a common cytogenetic abnormality in clonal plasma cells in monoclonal immunoglobulin deposition disease. Br J Haematol 2024 (Online ahead of print). doi: 10.1111/bjh.19748.

Hemato-Renal Profile of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits

Background Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a rare entity classified under the umbrella of monoclonal gammopathy of renal significance. The clinical implications of circulating monoclonal immunoglobulin (MIg), light chain restriction on immunofluorescence (IF) microscopy, histopathological pattern, and type of therapy on renal outcomes are not clearly defined. Materials and Methods Sixteen patients of PGNMID diagnosed between 2013 and 2020 were included from a biopsy registry of 11,459 patients at a single center. Follow-up data was collected from electronic medical records until June 2021. Results The mean age of the cohort was 41.7 ± 13.5 years. Forty-four (7/16) percent showed monoclonal protein on serum or urine electrophoresis, 25% (3/12) had IgG kappa by serum immunofixation electrophoresis (IFE) and 38% (5/13) had abnormal kappa: lambda free light chain (FLC) ratio. The predominant light microscopy pattern, membranoproliferative glomerulonephritis (MPGN) was seen in 7/16 (43.7%) patients. The predominant heavy chain detected by IF microscopy was IgG (13/16, 81.3%). Kappa and lambda light chain restriction were seen in 56.3 (9/16) and 43.8 (7/16) percent of patients respectively. Circulating monoclonal kappa light chains were detected in 50 and 29% of kappa-PGNMID patients by IFE and FLC assay respectively. None of the lambda-PGNMID patients had detectable circulating monoclonal lambda light chains. Patients with circulating MIg had more proteinuria, lower estimated glomerular filtration rate, and a higher percentage of plasma cells on bone marrow biopsy. Thirty-eight percent of our cohort (5/13) progressed to kidney failure over a median (range) period of 3 (IQR, 1-7) months. Of these, 4/5 received immunosuppression, and 1/5 were treated with plasma cell-targeted chemotherapy. Conclusion PGNMID is a rare disease with a biopsy incidence of 0.1%. Only a quarter of patients with PGNMID have circulating MIg. Presence of circulating MIg, type of monoclonal light chain restriction in kidney biopsy, and type of therapy did not predict renal outcomes. Patients with MPGN pattern had favorable renal outcomes despite a higher degree of proteinuria at presentation.

Light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a clinicopathological study of three cases

AimsTo explore the clinical and pathological features of light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID-LC).MethodsFrom January 2010 to December 2022, patients who were diagnosed with...

Pulmonary light chain deposition disease from a respiratory physician's perspective

Pulmonary light chain deposition disease (PLCDD) is a rare monoclonal immunoglobulin deposition disease characterized by the deposition of specific immunoglobulin light chains in lung tissue. In its early stages, PLCDD presents with mild clinical symptoms, while pulmonary imaging shows multiple nodules and thin-walled cysts. Pathologically, there is a deposition of eosinophilic amorphous protein-like material in the tissues, which stains negative for Congo red. The exact pathological mechanism of PLCDD remains unclear, and its clinical presentation lacks specificity. Challenges associated with this condition include difficulties in diagnosis, selection and evaluation of treatment options, lack of clear monitoring criteria, and standards for prognosis assessment. Further research is needed to elucidate the pathogenesis of PLCDD, to establish standardized diagnostic and therapeutic protocols, and to evaluate treatment efficacy and prognostic factors.

Heavy-chain deposition disease associated with multiple myeloma: a case report

Heavy-chain deposition disease (HCDD) is a rare disorder characterized by the deposition of monoclonal immunoglobulin. Due to the disease's rarity and diagnostic challenges, its prognosis is generally poor. Herein, we report a case of successful treatment of HCDD coexisting with multiple myeloma. A 56-year-old man presented at an outpatient clinic with complaints of whole-body pain, edema, and dyspnea that had persisted for 2 weeks. Diagnostic tests confirmed nephrotic syndrome, hematuria, and progressive renal failure. Serum immunofixation electrophoresis identified the presence of IgG kappa paraprotein. A diagnosis of multiple myeloma was established following a bone marrow biopsy. A renal biopsy revealed antibodies specific to the heavy chains of IgG on immunofluorescence, and electron microscopy showed diffuse electron-dense "powdery" densities in the glomerular basement membrane. Based on these findings, the patient was diagnosed with both HCDD and multiple myeloma. Following the diagnosis, the patient immediately began anti-plasma cell therapy using bortezomib, lenalidomide, and dexamethasone. Intermittent hemodialysis was initiated due to persistent azotemia during the diagnostic process; however, renal function improved significantly after only 1 month of therapy, allowing the discontinuation of dialysis. Early intervention with anti-plasma cell therapy, such as bortezomib, is known to improve prognosis in the early stages of the disease. This case report is presented to enhance understanding of HCDD and underscore the importance of prompt diagnosis and treatment in managing this rare condition.

Monoclonal immunoglobulin deposition disease a spectrum? The treatment of an unusual biopsy presentation

Introduction: Monoclonal gammopathy of renal significance (MGRS) is characterized by clonal cell deposition of monoclonal immunoglobulin causing renal manifestations without overt malignancy. MIDD is a subtype of MGRS characterized by deposition of monoclonal light (LCDD), heavy chains (HCDD), or both (LHCDD) along the renal glomerular and tubular basement membranes. Virtually all organs can be involved, and if left untreated, MIDD can progress to MM and end stage renal disease. Although these patients classically did not receive treatment, current MIDD management is primarily focused on controlling the underlying clonal plasma cell disorder. Case description: We present a 56-year-old male with atypical MIDD treated successfully with plasma cell directed therapy consisting of daratumumab, cyclophosphamide, bortezomib, and dexamethasone (DARA-CYBOR-D). Despite a primary diagnosis of diabetic kidney disease, renal biopsy suggested myeloma-related MIDD based on linear immunofluorescent staining for IgG and kappa light chains along the tubular and glomerular basement membranes. Notably there were no electron dense deposits in the mesangium or tubular basement membranes typical to MIDD electron microscopy. Despite the presentation of MIDD on IF only, DARA-CYBOR-D treatment led to hematologic and renal improvement and remission from MIDD, with a GFR increase from 22 to 59 mL/min/BSA and a drop in urine protein from up to 9 to 0.6 g/dL. Conclusions: In conclusion, our case illustrates the efficacy of the DARA-CYBOR-D regimen in a patient with IF-only MIDD. We highlight the importance of obtaining a kidney biopsy when there are other potential causes for real decline and enhance our understanding of pathological and clinical spectrum of MIDD.

#1297 Approaches to renal response assessment in rare monoclonal immunoglobulin related glomerulopathies

Abstract Background and Aims AL-amyloidosis (AL) and monoclonal immunoglobulin (MIg) deposition disease are common types of MIg-related glomerulopathies (MIgG) and are well-studied including approaches to treatment and hematological and renal responses (RR) assessment. Other rare MIgG comprise very diverse patterns of glomerular injury e.g. proliferative glomerulonephritis with MIg deposition (PGNMID), C3-glomerulopathy, fibrillary glomerulonephritis etc. Although RR defined as decrease in 24 h proteinuria by 30% without kidney function decline was validated only for renal AL it is widely used for MIg deposition disease and rare MIgG without validation. Nevertheless, the best approach to RR assessment in rare MIgG is still unclear. To shed some light on this problem we performed this study. Method We retrospectively analyzed 31 patients with rare MIgG who were treated with clone-specific treatment and had available follow-up (FUP) ≥6 months (mo). The following morphological patterns were diagnosed: PGNMID (n = 9), C3-glomerulopathy (n = 9), thrombotic microangiopathy (n = 5), cryoglobulinemic glomerulonephritis (n = 3), fibrillary glomerulonephritis (n = 3), immunotactoid glomerulonephritis (n = 1) and membranous nephropathy (n = 1). Patients’ baseline parameters are shown on Fig. 1 as well as treatment modalities. Hematological response defined according to currently accepted criteria is demonstrated on Fig. 1. We evaluated two approaches to the RR assessment: 1) at least partial renal response (PR) defined as 24 h proteinuria decreased by 50% from the baseline and <3.5 g, and 2) according to Palladini's criteria for AL (AL-RR) i.e. 24 h proteinuria decreased by 30% from the baseline or <0.5 g. Both RR were considered as achieved only in the absence of estimated glomerular filtration rate (eGFR) decline >25%. Time points for RR assessment were 6, 12 and 24 mo from the clone-specific treatment initiation. Renal outcome was estimated as initiation of renal replacement therapy or eGFR <15 ml/min/1.73 m2 at the end of follow-up. Death-censored renal survival was analyzed by the Kaplan-Meier method. Cox regression analysis was applied to determine which RR was independently associated with long-term kidney outcome. P-value was assumed at <0.05. Median FUP was 19 (7; 50) mo. FUP ≥12 and ≥24 mo were available in 26 and 17 patients, respectively. Results Total number of patients who achieved AL-RR at any RR assessment time point was 22 (71%). At 6, 12 and 24 mo the achievement of AL-RR was registered in 17, 16 and 10 patients, respectively. PR cumulatively was achieved in 19 (61%) patients: at time points of 6, 12, and 24 mo it was registered in 14, 14 and 9 cases, respectively. The 5-year cumulative renal survival in those who had AL-RR was better than in not responders (Fig. 2A). Patients who ever achieved PR had better cumulative renal survival compared with patients with no renal remission (Fig. 2B). In univariate Cox regression analysis the achievement of AL-RR (Exp(β) 0.158 (95% CI 0.03; 0.819)) was associated with renal outcome as well as the achievement of PR (Exp(β) 0.074 (95% CI 0.09; 0.626)). In multivariate Cox regression model adjusted for eGFR at the time of kidney biopsy AL-RR vs no response was estimated as an independent predictor of renal long-term prognosis (Exp(β) 0.082 (95% CI 0.009; 0.725)) as well as eGFR (Exp(β) 0.89 (95% CI 0.821; 0.985)). The only independent predictor of renal outcome in the model including eGFR at the time of kidney biopsy (Exp(β) 0.85 (95% CI 0.72; 0.99), p = 0.05) was PR (Exp(β) 0.011 (95% CI 0; 0.547), p = 0.021). Conclusion The classical concept of complete and partial renal remission seems to be more appropriate for rare MIgG and requires further validation studies.

#537 Proliferative glomerulonephritis with monoclonal immunoglobulin deposits: effects of pretreatment on posttransplant outcomes

Abstract Background and Aims Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) has a poor renal prognosis and a high rate of recurrence after kidney transplantation, up to 90%, typically within the first-year post-transplant. Nearly 50% of patients lose their renal allograft within 3 years of recurrence. It has been suggested that pretreatment therapy (with B cell depleting or plasma-cell directed therapy) can help prevent recurrence or modulate the course of disease post-transplant. The aim of our study is to evaluate the effect of pretreatment therapy given within 6 months prior to kidney transplant on outcomes including PGNMID recurrence and all cause graft loss. Method Between 1996 and 2022, a total of 28 patients with biopsy-proven PGNMID as the cause of end stage kidney disease received a total of 31 kidney transplants. All patients had at least one year of follow-up data, for all but one patient this included surveillance kidney transplant biopsies completed at our center. Recurrence was defined histologically by immunofluorescence finding of monoclonal immunoglobulin deposits, independent of other histological or clinical parameters. The incidence of recurrence and all cause graft loss were analyzed by Kaplan-Meier plots. Results Median age of patients at transplant was 57 years, 87% were white, 65% were male, 74% received living donor transplant, 59% received thymoglobulin induction, 81% received maintenance with tacrolimus, mycophenolate, and prednisone. Median follow up time was 80 months. A total of 9 patients received pretreatment within 6 months of transplant, 8 received Rituximab and one received Daratumumab. The pretreatment group was generally younger at transplant (53 years vs 57 years) and had a shorter median follow up time (65 months vs 96.5 months), but neither were statistically significant. Overall recurrence rate was 78% for pretreatment and 91% for control, though difference in time to recurrence was not statistically significant (Fig. 1). The difference in all cause graft loss between groups was not statistically significant, however, none in the pretreatment group had graft loss by 60 months (Fig. 2). One patient who received daratumumab had no recurrence by 1 year post-transplant. Conclusion In kidney transplant recipients with PGNMID, pretreatment (primarily with rituximab) may be modulating the post-transplant course of disease. Further studies are needed to determine the extent of this effect and if plasma-cell directed vs B-cell directed therapy may be superior.

#3051 Light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a case report

Abstract Background and Aims Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is an entity characterized by glomerular deposition of monoclonal immunoglobulins, typically IgG. Recently, a cohort of 17 patients presenting with light chain only variant of PGNMID was published. Strikingly, in this cohort, glomerular deposition of C3 was detected in all cases. We herein report a case of PGNMID with a rare presentation on the kidney biopsy. Case presentation We present the case of a 73-year-old female with a past medical history of rheumatoid arthritis and hypertension, under methotrexate 10 mg per week, prednisone 2.5 mg per day, amlodipine, nebivolol, azilsartan, and a recurrent naproxen intake. She presented with fatigue, anorexia, weight loss of about 12% of her body weight, epistaxis, oral bleeding ulcers, and lower extremities oedema. Pancytopenia, acute kidney injury (urea 150 mg/dL, creatinine 2.92 mg/dL), and nephrotic syndrome (serum albumin of 2.7 mg/dL and proteinuria of 12 g/day) were noted. Further evaluation showed an imbalance of the free light chains’ ratio with kappa light chain's level of 1250 mg/dL. Serum protein electrophoresis and immunofixation were normal. A CT scan showed numerous cervical, mediastinal, and iliac enlarged lymph nodes. Kidney biopsy showed an increase in mesangial cells and matrix and capillary walls’ thickening without nodular lesions under a light microscope. Immunofluorescence microscopy showed granular deposits of kappa light chains and no deposition IgA, IgG, IgM, C1q, C3, fibrin and lambda. Considering the previous literature, C3 was reanalyzed, with a consistent negative staining. Based on the above findings, a diagnosis of Light chain only variant of PGNMID was made. Concomitantly, evaluation of the bone marrow sample showed Small lymphocytic lymphoma. The patient was started on Bortezomib, cyclophosphamide and dexamethasone. Three months after proteinuria was 0.47 g/g and creatinine 1.7 mg/dL. Conclusion This case highlights the rare occurrence of a PGNMID with light chains only deposition (without C3 co-deposition) as a complication of small lymphocytic lymphoma. This contributes to the growing body of evidence surrounding this entity, with the particularity being that C3 was not detected. The authors hypothesized some pathophysiologic mechanisms to justify the absence of C3 co-deposition.

#2284 Clinical characteristics and renal survival of patients with monoclonal gammopathy of renal significance (MGRS)

Abstract Background and Aims Monoclonal gammopathy of renal significance (MGRS) comprises a diverse group of renal disorders related to a nephrotoxic monoclonal immunoglobulin. Due to the rarity of the disease and the recent definition of MGRS-associated kidney lesions, data about long-term clinical outcomes remain limited. Method A single-center, retrospective cohort study was conducted including data of all patients with biopsy-proven MGRS who were followed at our center between 2005 and 2023. We examined the clinicopathologic characteristics and renal survival of the patients. Disease progression was defined as an eGFR decline >30% or end-stage kidney disease (ESKD). Univariate Cox regression analysis was used to assess the relationship between clinical features and disease progression. Results A total of 23 adults (males 43.5%) with a median age of 65 years (IQR 55-70) were included in our study. At presentation, median eGFR was 51 ml/min/1.73 m2 (IQR 40-80) and proteinuria 3.94 g/24 h (IQR 2.00-6.26). Serum or urine monoclonal protein was not detected in 4 patients (18.2%). MGRS-associated renal lesions included Ig-related amyloidosis (n = 8), monoclonal immunoglobulin deposition disease (n = 7), cryoglobulinemic glomerulonephritis (n = 2), C3 glomerulopathy (n = 2), proliferative glomerulonephritis with monoclonal IgG deposits (n = 1), immunotactoid glomerulopathy (n = 1) and miscellaneous (n = 2). Most patients were treated with a plasma cell-directed regimen (77.3%) and the rest with B-cell-directed (13.6%) or conservative therapy (9.1%). Median follow-up for the cohort was 45 months (IQR 27-67). Thirteen patients responded to treatment (52.1%), achieving a reduction in proteinuria (0.44 vs 3.94 g/24 h, p:001) and maintenance of eGFR (60 vs 56 ml/min, p:0.363) at the last follow-up visit. All responders had received clone-directed therapy. Disease progression occurred in 8 patients (34.8%), three of whom reached ESKD (13.0%). Renal survival, defined as survival without ESKD, was 84.6% at 48 months (CI 73.3-101.7). Factors that were associated with disease progression were Ig-related amyloidosis (HR: 6.94, CI: 1.30-36.84, p:0.023) and eGFR < 45 ml/min at diagnosis (HR: 14.73, CI: 1.62-132.95, p:0.017). Conclusion MGRS is a heterogeneous clinical entity manifesting with varying degrees of proteinuria and kidney injury. Impaired kidney function at diagnosis and Ig-related amyloidosis correlated with worse renal prognosis in our cohort. Early identification and clone-directed treatment of MGRS may improve long-term renal survival.

Primary adrenal insufficiency developed 22 years after the diagnosis of light and heavy chain deposition disease: a case report.

Monoclonal immunoglobulin deposition diseases (MIDDs), including light and heavy chain deposition disease (LHCDD), are rare and heterogeneous disorders associated with underlying B-cell clonal disorders. Adrenal involvement is a potential extrarenal manifestation of MIDDs; however, limited data are available regarding its prevalence and clinical presentation. Herein, the present report describes, for the first time, a case of primary adrenal insufficiency that developed twenty-two years after a diagnosis of LHCDD had been made. A 69year-old woman with a 10year history of hemodialysis suddenly became bedridden after falling down stairs in the absence of focal neurological deficits. Subsequently, she experienced appetite loss, nausea, vomiting, a fever of unknown origin, and unexplained hypotension. Several months later, primary adrenal insufficiency and normal pressure hydrocephalus were diagnosed and successfully managed. The long-term clinical prognosis of MIDDs has not been fully elucidated despite recent advances in the management of the disorders. This report may contribute to improving our understanding of the disease course.

WCN24-1318 The characteristics of IgM variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits

WCN24-1318 The characteristics of IgM variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits

Clinicopathological characteristics and prognosis in patients with monoclonal gammopathy and renal damage in central China: a multicenter retrospective cohort study

Renal involvement is common in monoclonal gammopathy (MG); however, the same patient may have both MG and non-paraprotein-associated renal damage. Accordingly, distinguishing the cause of renal damage is necessary because of the different clinical characteristics and associated treatments. In this multicenter retrospective cohort study, we described the clinicopathological characteristics and prognosis of 703 patients with MG and renal damage in central China. Patients were classified as having MG of renal significance (MGRS), MG of undetermined significance (MGUS), or hematological malignancy. 260 (36.98%), 259 (36.84%), and 184 (26.17%) had MGRS, MGUS, and hematological malignancies, respectively. Amyloidosis was the leading pattern of MGRS (74.23%), followed by thrombotic microangiopathy (8.85%) and monoclonal immunoglobulin deposition disease (8.46%). Membranous nephropathy was the leading diagnosis of MGUS (39.38%). Renal pathological findings of patients with hematological malignancies included paraprotein-associated lesions (84.78%) and non-paraprotein-associated lesions (15.22%). The presence of nephrotic syndrome and an abnormal free light chain (FLC) ratio were independently associated with MGRS. The overall survival was better in patients with MGUS than in those with MGRS or hematological malignancies.

Daratumumab therapy in a pediatric case of C3 nephritic factor-positive proliferative glomerulonephritis with monoclonal IgG deposits.

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is an exceedingly rare cause of glomerulonephritis among children for which prognosis is generally poor, with low incidence of remission and high rates of recurrence after transplant. While there are more cases reported in the adult literature, substantial differences in pediatric vs. adult PGNMID render it essential that we further characterize pediatric cases to optimize management. We report the case of a 12-year-old male presenting initially with edema and hypertension who was subsequently diagnosed with IgG3/Kappa-dominant PGNMID. In the absence of any proven therapy and though without a detectable clone, he was empirically treated with daratumumab with positive effect to date. This is the first reported case of daratumumab monotherapy in pediatric PGNMID, as well as the first PGNMID case to detect presence of C3 nephritic factor.

Acute kidney injury in Hodgkin’s lymphoma, non-Hodgkin lymphoma/leukemia, and lymphoplasmacytic lymphoma: One center experience

Background: Acute kidney injury (AKI) is a common complication in patients with leukemia and lymphoma. In this single-center non-interventional retrospective study from 2010 to 2022, we aimed to evaluate causes, clinical features, and pathology finding in patients with Hodgkin lymphoma (HL), non-Hodgkin lymphoma/leukemia (NHL/CLL), or lymphoplasmacytic lymphoma (LPL) and AKI. Methods: We identified 101 patients with HL, NHL/CLL, and LPL. We analyzed AKI incidence and various clinical, laboratory, and pathology parameters and outcomes. Results: About 46.5% of patients with HL, NHL/CLL, and LPL presented with AKI. The incidence of AKI was the highest in the CLL setting (65.0%), it was lower and almost equal (48.0% and 47.0%) in NHL and HL, respectively, and the lowest (14.2%) in LPL. Study group comprised of 47 patients, M/F 31/16, median age was 64.0 [52.0; 71.0] years, and the patients with HL were significantly younger ( p = 0.022). The median disease duration prior to the AKI presentation was 14 [5; 44] months. Pre-renal AKI was diagnosed in 48.9% of cases: tumor lysis syndrome (TLS), specific lymphoid infiltration (LI), and post-renal AKI presented in 12.7% each. Kidney biopsy in 49% of patients revealed LI in 39.1% of cases. Focal segmental glomerulosclerosis presented in 21.7% of cases, proliferative glomerulonephritis with monoclonal immunoglobulin deposits in 13.0%, AA amyloidosis, IgA nephropathy, and AA amyloidosis in 8.6% each. AKI recovered in 63.8% of patients and 59.5% of patients were alive and dialysis-free at last evaluation: 100% with HL versus 58.3%, 46.1%, and 0% with NHL, LL, and LPL, respectively ( p = 0.025); and 8.5% remained alive on dialysis. About 25.5% of patients died after discontinuation or without need for dialysis; and 6.3% died on dialysis. Conclusion: AKI in patients with leukemia and lymphoma is heterogeneous and most commonly caused by volume depletion. AKI recovery, though not complete, occurred in 63.8% of cases with 68% survival.

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits in the native or posttransplant kidney.

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits in the native or posttransplant kidney.

Proliferative Glomerulonephritis with Section Monoclonal Immunoglobulin Deposits: A Case Report

Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits (PGNMID) is a relatively rare entity. Patient’s usually present with proteinuria, elevated creatinine, and renal failure. The present case report details the clinical presentation, diagnostic assessments, and treatment outcomes of a 56-year-old female with a complex renal pathology. The patient, with a history of hypertension, presented with generalised anasarca and decreased appetite. Initial examinations revealed pedal oedema, low serum albumin, elevated Blood Urea Nitrogen (BUN), and markedly high serum creatinine levels, indicating impaired kidney function. Further investigations, including urine analysis and immunological tests, identified significant proteinuria and complement system involvement. Radiological studies demonstrated ascites and elevated renal cortical echogenicity. Renal biopsy revealed Proliferative Glomerulonephritis with conventional Monoclonal Immune Deposits-Immunoglobulin G3 (PGNMID-IgG3-kappa). Direct immunofluorescence analysis exhibited a specific staining pattern, confirming the diagnosis. Overall, the diagnostic impression suggested Proliferative Glomerulonephritis with Membranoproliferative Glomerulonephropathy (MPGN) injury pattern, emphasising the complexity of the renal pathology. Despite extensive medical interventions, including haemodialysis and immunosuppressive measures, the patient’s condition deteriorated, leading to a fatal outcome on the 67th day of admission. The present case underscores the challenges in managing advanced renal diseases and emphasises the importance of precise diagnostic methods for guiding targeted therapeutic interventions in such complex cases.