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Background: Kidney damage in lymphomas/leukemia’s presents with either acute kidney injury (AKI), chronic kidney disease (CKD), or both; and whilst AKI leads to evaluation often based on the clinical data, in some AKI and in almost all CKD cases kidney biopsy gives a clue to the diagnosis. Methods: A single center non-interventional retrospective study identified 36 patients with biopsy-proven kidney damage: 6 with Hodgkin’s lymphoma (HL), 18 with non-Hodgkin’s lymphoma/leukemia (NHL/CLL), and 12 with lymphoplasmacytic lymphoma (LPL). Results: Fifty-eight percent males and 42% females mean age 56.2 ± 17.4 years, presented with nephrotic syndrome in 47.2%, and with AKI in 11.1% of cases; 75% of patients diagnosed with CKD; in 13.9% AKI was superimposed on CKD. Patients with NHL/CLL presented with AKI significantly more often compared to HL and LPL—44% versus 16.6% versus 0 respectively. Monoclonal immunoglobulin (MIg) related glomerulopathies (GP) were found in 83.3% versus 16.6% cases in the LPL and NHL/CLL sub-groups, respectively ( p = 0.013). Patterns of damage included intracapillary monoclonal deposition disease, light and heavy chain amyloidosis, monotypic membranous nephropathy (MN), cryoglobulinemic glomerulonephritis (GN) and C3-GN in the LPL; and monotypic MN and proliferative GN with MIg deposits in the NHL/CLL sub-groups respectively. Paraneoplastic GPs were found in 83.3%, 38.8%, and 16.6% of patients with HL, NHL/CLL, LPL, respectively (HL vs LPL, p < 0.001), and included minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and IgA-nephropathy in the HL; membranoproliferative GN, MN, and MCD in NHL/CLL; and FSGS in the LPL sub-groups. Tubulointerstitial damage revealed in NHL/CLL sub-group only, and found in every other case with 80% of lymphoid infiltration. Conclusions: Pattern glomerular damage depends on lymphoma type: paraneoplastic GPs are typical for HL, MIg-related GPs dominate in LPL, NHL/CLL presents mainly as paraneoplastic with single MIg-related patterns. Tubulointerstitial damage due to specific kidney infiltration attributable to NHL/CLL.

Introduction: According to the recent International Kidney and Monoclonal Gammopathy Research Group Consensus report, the spectrum of monoclonal gammopathies of renal significance merges more than 12 conditions; and combinations of various patterns of monoclonal gammopathies of renal significance–associated kidney damage have already been described. We present a case of a previously unreported combination of C3 glomerulopathy and light-chain proximal tubulopathy. Case description: A 53-year-old Caucasian man presented with general weakness. Three years prior, a check-up revealed proteinuria and microhematuria, later accompanied by arterial hypertension, elevated serum creatinine, and a low serum C3 levels. On admission, his creatinine was 1.5 mg/dL; his protein excretion was 1900 mg/24 h with microhematuria at 150 hpf. His serum C3 was 79 mg/dL with a normal C4 level. Serum protein electrophoresis revealed an M-spike, immunochemistry confirmed monoclonal immunoglobulin G kappa secretion of 920 mg/dL, and traces of Bence–Jones protein kappa in his urine. A kidney biopsy showed mesangial hypercellularity with diffuse fine granular C3 mesangial expression and tubular atrophy with diffuse coarse granular kappa light-chain tubular epithelial cytoplasmic expression. Computed tomography did not reveal any destructive lesions. A bone marrow smear showed 5% plasma cells, and bone marrow cell flow cytometry demonstrated 87.4% of plasma cells with an aberrant phenotype. The patient was diagnosed with an monoclonal gammopathy of renal significance and administered bortezomib–cyclophosphamide–dexamethasone. After four courses, his monoclonal immunoglobulin G kappa secretion halved, but his serum creatinine and proteinuria remained almost unchanged. Chemotherapy was switched to lenalidomide–dexamethasone, and after five courses his immunoglobulin G kappa secretion decreased to traces, Bence–Jones protein was not observed and his urinalysis, serum creatinine, and C3 levels were detected as normal. Conclusion: The combination of kidney damage patterns, one of which was directly and the other indirectly associated with monoclonal gammopathy, complements the monoclonal gammopathies of renal significance spectrum. Chemotherapy led to a very good partial response and kidney function recovery.

Background:Monoclonal immunoglobulin–mediated kidney disease with various patterns of damage may occur in patients with B-cell malignancies and non-malignant monoclonal gammopathies, and the latter are actually merged under the umbrella of monoclonal gammopathy of renal significance. Amyloidosis is the most well-known monoclonal immunoglobulin–related kidney damage. We focused on the rarer conditions and aimed to evaluate the non-amyloid spectrum of monoclonal immunoglobulin–mediated patterns of renal damage in real clinical practice.Methods:A single-center non-interventional retrospective study included 45 patients with pathology-proven non-amyloid monoclonal immunoglobulin–mediated kidney disease, followed during 2002–2018. Disease duration, proteinuria, serum creatinine, need for dialysis at the time of kidney biopsy, clinical diagnosis, and kidney pathology findings were analyzed.Results:No significant differences in the median age, disease duration at the time of biopsy, or main clinical presentation of kidney disease were found between patients with monoclonal gammopathy of renal significance and patients with B-cell malignancies. Pathology patterns like proliferative glomerulonephritis with monoclonal immunoglobulin deposits, membranous nephropathy, C3 glomerulopathy, cryoglobulinemic glomerulonephritis, and combinations of light chain proximal tubulopathy with monoclonal immunoglobulin deposition disease, and of C3 glomerulopathy with light chain proximal tubulopathy were found in monoclonal gammopathy of renal significance setting only. In contrast, light chain proximal tubulopathy alone, anti-glomerular basement glomerulonephritis, and combinations of cast nephropathy with light chain proximal tubulopathy, and cast nephropathy with monoclonal immunoglobulin deposition disease were associated with multiple myeloma only. Monoclonal immunoglobulin deposition disease, intracapillary monoclonal immunoglobulin M deposits, and cast nephropathy alone were seen in both settings.Conclusion:The presence of monoclonal gammopathy in patients with proteinuria and/or impaired kidney function demands kidney biopsy. Neither duration of kidney disease nor its clinical presentation allows differentiating malignant and non-malignant causes of monoclonal immunoglobulin–mediated renal damage. Several pathology patterns, even cast nephropathy, can be found both in cases of monoclonal gammopathy of renal significance and in cases of B-cell malignancies. Dual patterns of damage, including combinations of organized and non-organized deposits, or organized deposits with monoclonal immunoglobulin–induced damage without monoclonal immunoglobulin deposition, constitute up to 9%, mostly in multiple myeloma cases.

Background and Aims Kidney damage in non-Hodgkin lymphoma/leukemia (NHL/CLL) and in Hodgkin’s lymphoma (HL) are caused by several mechanisms: tumor mass localization, clonal cell expansion, hormones, cytokines and growth factors secretion, metabolic, electrolyte and coagulation disturbances, infectious, drug-induced and radiation complications etc., which can affect urinary tract, renal arteries and veins and all renal parenchyma compartments. Clinical presentation of kidney involvement in the patients with NHL/CLL and HL include both acute kidney injury (AKI) and chronic kidney disease (CKD), the latter - with or without nephrotic syndrome (NS). Pre-renal AKI, tumor-lysis syndrome (TLS), or urinary tract compression by lymph nodes usually diagnosed on the clinical basis, but one cannot differentiate other conditions, particularly NS, in the clinical setting. Importantly, symptoms of kidney damage may dominate or even preclude overt NHL/CLL or HL, and only pathology data give the clue to the main diagnosis. Kidney biopsy or post-mortem pathology findings include various patterns of paraneoplastic glomerulonephritis (GN) as well as amyloidosis; as also as tubulointerstitial damages, mainly specific lymphoid interstitial infiltration and sometimes pyelonephritis Method In this retrospective study from a single nephrology centre, we retrieved charts of the patients with NHL/CLL and HL. Collected data were age, gender, main diagnosis, diagnosis of AKI or CKD, CKD stage, NS presence, the cause of AKI and CKD based on the clinical presentation, and pathology pattern, if available. Results Study group included 57 patients, 36 males and 21 females, median age 48 [18; 80] years. Kidney biopsy was performed in 22 (39%) of cases. Main clinical data and pathology shown in the Figure. Patients with B-cell NHL/HLL comprised almost ¾ of the study group, with AKI and CKD in a roughly equal proportion. Pathology showed specific infiltration in about ¼ of AKI and in a few CKD cases, and various paraneoplastic GN in every fifth case - minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), membranoproliferative GN (MPGN), and IgA-nephropathy (IgAN); two cases of AA amyloidosis, and one case of tubulointerstitial nephritis. There were only three patients with T-cell NCL/CLL, pathology findings were MCD in one case and lymphoid infiltration in another. Four out of 13 cases of HL demonstrated paraneoplastic GN – MCD, FSGS and IgAN, with just one case of AA amyloidosis. Totally 17 cases of AKI - pre-renal and caused by TLS or urinary tract compression by lymph nodes diagnosed on the clinical basis. Interestingly, in five cases we found a combination of various clinical settings and pathology patterns – compression with lymph nodes and TLS or acute pyelonephritis, AA amyloidosis and lymphoid infiltration or acute pyelonephritis, and finally IgAN and lymphoid infiltration. In 14 (24.5%) of the study group, the cause pf CKD remained unknown, as kidney biopsy was not performed due to severe anaemia and/or thrombocytopenia. Conclusion It is impossible to decipher many of AKI and most of CKD causes in the patients with NCL/CLL and HL without pathology evaluation. Nephrologist must perform kidney biopsy in all patients with NCL/CLL of HL CKD, and those with AKI of unknown origin, unless contraindicated, especially if the hematological diagnosis is uncertain.

Introduction Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare condition with unknown pathogenesis, which is sometimes associated with hematological disorders. We present the case of renal and splenic AA amyloidosis associated with SANT and essential thrombocythemia – a previously unreported combination of pathologies. Case description A 42-year-old Caucasian woman presented with a 6-year history of progressive thrombocytosis, low-grade fever, and elevated C-reactive protein (CRP). An abdominal ultrasound discovered a splenic mass, and a bone marrow biopsy revealed megakaryocytosis with atypical features. Under interferon-α treatment, her fever resolved and her platelet count decreased, but the CRP remained elevated. Within 2.5 years she developed nephrotic syndrome and kidney failure. A kidney biopsy revealed amyloidosis. She was started on hemodialysis and underwent a splenectomy. Splenic pathology revealed SANT and AA amyloidosis of the spleen. Further review of her biopsy specimens confirmed renal AA amyloidosis and myeloproliferative disorder. Polymerase chain reaction studies showed a JAK2 V617F mutation in 1% of nucleated cells. Anti-Epstein-Barr virus (EBV) immunoglobulin G (IgG)-Epstein-Barr nuclear antigen (EBNA) and IgG-viral capsid antigen were >600 U/mL. At the latest follow-up visit 1 year after the splenectomy, she is doing well; her platelet count and CRP are normal. Conclusions Our patient has SANT and essential thrombocythemia associated with AA amyloidosis. The high titers of anti-EBV IgG suggest that chronic EBV infection may have been causative for the former 2 conditions. The return of her high CRP level to the normal range after surgical removal of the pseudotumor may suggest an association of AA amyloidosis with SANT.