Search

In this clinical case, we report an atypical and unique presentation of systemic lupus erythematosus (SLE) in a 39-year-old female with nephrotic syndrome. The patient exhibited class IV plus V lupus nephritis and extensive immune complex deposition within the intracapillary and arteriolar regions suggestive of cryoglobulinemic glomerulonephritis, despite no detectable circulating cryoglobulins. Electron microscopy revealed cryoglobulin-like deposit distribution in all glomerular examined compartments, namely subendothelial, intramembranous, subepithelial, and mesangial, apparently extending from the capillary hyaline thrombi. The case highlights the possibility of severe renal injury in SLE without circulating cryoglobulins and the diverse kidney manifestations associated with the disease. However, the impact on patient outcome was minimal, as classical treatment (id est National Institute of Health regimen) remained effective. [ABSTRACT FROM AUTHOR]

In this rare case of infection-related cryoglobulinemic glomerulonephritis with infective endocarditis, a 78-year-old male presented with an acute onset of fever and rapidly progressive glomerulonephritis. His blood culture results were positive for Cutibacterium modestum, and transesophageal echocardiography showed vegetation. He was diagnosed with endocarditis. His serum immunoglobulin M, IgM-cryoglobulin, and proteinase-3-anti-neutrophil cytoplasmic antibody levels were elevated, and his serum complement 3 (C3) and C4 levels were decreased. Renal biopsy results showed endocapillary proliferation, mesangial cell proliferation, and no necrotizing lesions on light microscopy, with strong positive staining for IgM, C3, and C1q in the capillary wall. Electron microscopy showed deposits in the mesangial area in the form of fibrous structures without any humps. Histological examination confirmed a diagnosis of cryoglobulinemic glomerulonephritis. Further examination showed the presence of serum anti-factor B antibodies and positive staining for nephritis-associated plasmin receptor and plasmin activity in the glomeruli, suggesting infective endocarditis-induced cryoglobulinemic glomerulonephritis. [ABSTRACT FROM AUTHOR]

Background: Cryoglobulinemic glomerulonephritis (Cryo-GN), caused by circulating cryoglobulins, has varied etiology and clinical-pathologic manifestations. This study aimed to investigate the clinicopathological spectrum and outcome of patients with various Cryo-GN in China. Methods: A retrospective review of 74 Chinese patients with biopsy-proven cryoglobulin-related renal lesions in Peking University First Hospital from 2010 to 2020 was performed. Results: The mean age at diagnosis was 52.9 ± 15.0 years, and the female-to-male ratio was about 2/5. For the etiology screening, serum/urine monoclonal immunoglobulin could be detected on immunofixation electrophoresis in 34% of patients, including 6 patients who had hematological malignancies. Fifty-seven percent of patients had HBV infection, far more than HCV infection (5%). Ten percent of patients had other infections, and 27% of patients were classified as essential or idiopathic. Eleven out of the 15 patients with type II cryoglobulinemia had a consistent monotype of serum monoclonal immunoglobulins and monoclonal cryoprecipitate. The clinical manifestations were similar between various types of cryoglobulinemia. Hematuria, proteinuria, hypertension, anemia, and chronic renal insufficiency were the most common features. Fifty-three percent of patients presented with nephrotic syndrome, and 32% experienced acute kidney injury. Hypocomplementemia, serum-positive rheumatoid factor activity, and skin lesions were reported in 45%, 29%, and 28% of patients, respectively. After a median of 24 months follow-up, 18 patients reached end-stage kidney disease. The clone-targeted treatment could retard the renal deterioration compared with immunosuppressive therapy. Conclusions: This was the largest single-center, clinicopathological retrospective study of Cryo-GN in China. Our data strongly support the association between monoclonal gammopathy and type II Cryo-GN. The renal responsive rate of immunosuppressant therapy is still suboptimal. The clone-targeted treatment shows promising effects in patients with type I or II Cryo-GN.

In this rare case of infection-related cryoglobulinemic glomerulonephritis with infective endocarditis, a 78-year-old male presented with an acute onset of fever and rapidly progressive glomerulonephritis. His blood culture results were positive for Cutibacterium modestum, and transesophageal echocardiography showed vegetation. He was diagnosed with endocarditis. His serum immunoglobulin M, IgM-cryoglobulin, and proteinase-3-anti-neutrophil cytoplasmic antibody levels were elevated, and his serum complement 3 (C3) and C4 levels were decreased. Renal biopsy results showed endocapillary proliferation, mesangial cell proliferation, and no necrotizing lesions on light microscopy, with strong positive staining for IgM, C3, and C1q in the capillary wall. Electron microscopy showed deposits in the mesangial area in the form of fibrous structures without any humps. Histological examination confirmed a diagnosis of cryoglobulinemic glomerulonephritis. Further examination showed the presence of serum anti-factor B antibodies and positive staining for nephritis-associated plasmin receptor and plasmin activity in the glomeruli, suggesting infective endocarditis-induced cryoglobulinemic glomerulonephritis.

Introduction:  Type I cryoglobulinemia is a rare disease which affects the skin, central nervous system and kidneys. It is usually associated with lymphoproliferative disorders such as multiple myeloma, lymphoma and monoclonal gammopathy of renal significance. Proteinuria and membranoproliferative glomerulonephritis are the most common renal manifestations; Case presentation: Here we report the case of a female patient in her late 40 s who had proteinuria accompanied by Raynaud's phenomenon, high blood and plasma viscosity, hearing loss, and cardiac and central nervous system involvement. Monoclonal immunoglobulin G-λ protein was detected and serum was positive for cryoglobulin. Renal biopsy revealed massive cryo-plugs with unique ultrastructural appearance in the glomerular and peritubular capillary lumina. Immunofluorescence showed predominant IgG3/λ deposition in cryo-plugs. As reported, the clinical manifestations of this patient resulted from cryoprecipitate and hyperviscosity syndrome; Conclusion: Cryoglobulinemia should be considered as a possible diagnosis in patients with Raynaud's phenomenon, hyperviscosity syndrome and monoclonal immunoglobulin. [ABSTRACT FROM AUTHOR]

The paper presents the steps for studying cryoglobulinemia from essential to cryoglobulinemic vasculitis associated with hepatitis C virus. It shows advances in the study of the etiology of cryoglobulinemia, diagnosis, specific features of renal injury, and current approaches to treating HCV infection-related cryoglobulinemic vasculitis with renal involvement, by using 3 clinical cases (with a difference of a few decades during a follow-up).

Abstract Objective To assess the effectiveness of apheresis therapy (AT) in treating the clinical manifestations of patients with complicated cryoglobulinemic vasculitis (CV). Methods A retrospective cohort study of 159 CV patients attending 22 Italian Centers who underwent at least one AT session between 2005 and 2015. The response to AT was evaluated on the basis of a defined grading system. Results Peripheral neuropathy was the most frequent clinical condition leading to AT. Therapeutic plasma exchange was used in 70.4% of cases. The outcome of AT was rated very good in 19 cases, good in 64, partial/transient in 40, and absent/not assessable in 36. Life-threatening CV-related emergencies and renal impairment independently correlated with failure to respond to AT. The independent variables associated with an increased risk of death were age at the time of the first AT session, multi-organ life-threatening CV, the presence of renal impairment and failure to respond to AT. The time-dependent probability of surviving until CV-related death in the second year was 84%, with an AHR in patients with absent/not assessable response to AT of 11.25. Conclusion In this study AT is confirmed to be a safe procedure in patients with CV. Early AT should be considered in patients with severe CV, especially in cases with impending renal involvement, in order to prevent irreversible kidney damage. Although its efficacy in patients with multi-organ failure is limited, AT is the only treatment that can rapidly remove circulating cryoglobulins, and should be considered an emergency treatment. [ABSTRACT FROM AUTHOR]

Cryoglobulinemic glomerulonephritis (CG) is rare in children. We report a 13-year-old Thai boy who presented with acute nephrotic nephritis syndrome, arthralgia, and purpura. Renal pathology revealed membranoproliferative glomerulonephritis and CG. No secondary causes of CG were found. He responded well to 12 weeks of oral cyclophosphamide plus low dose prednisolone and azathioprine for maintenance. He was doing well at 31 weeks but long term follow up is required as chronic renal failure and neoplasia were reported.

Recently, the term monoclonal gammopathy of renal significance (MGRS) was used to describe monoclonal gammopathies capable of causing kidney damage resulting in chronic kidney impairement and end-stage renal disease. By definition, patients with MGRS have small plasma-cell or B-cell clones that do not meet the criteria for multiple myeloma or lymphoma, and are frequently mistaken as monoclonal gammopathies of undetermined significance (MGUS). As treatment is not recommended for MGUS, appropriate therapy is commonly withheld. Therefore, a high index of clinical suspicion for MGRS and a multidisciplinary approach are essential to avoid delayed diagnosis and the development of MGRS-related end-stage renal disease. [ABSTRACT FROM AUTHOR]

The paper considers the specific features of renal involvement developing in chronic infection caused by hepatitis C virus (HCV) and the current possibilities of treatment. It details the clinical and morphological manifestations of HCV-related cryoglobulinemic glomerulonephritis, and criteria for its diagnosis and prognosis. The author discuss new approaches to treating (severe cryoglobulinemic vasculitis with renal involvement in particular) - antiviral therapy (pegylated interferon-α/ribavirin) in combination with biological agents (anti-CD monoclonal antibodies, such as rituximab) to achieve clinical, virological, immunological remissions and a response at a molecular level - to eliminate oligo- and monoclonal B lymphocyte proliferation.

Aim. To analyze clinical and laboratory features of cryoglobulinemic vasculitis (CGEV) associated with HCV infection. Material and methods. We examined 61 patients with clinical manifestation of CGEV in 2006-2011. CGEV was associated with autoimmune diseases in 31 patients (51 %), with HCV infection in 21 patients (34,4 %), essential (idiopathic) cryoglobulinemia in 8 patients (13 %) and lymphoproliferative diseases in 1 patient (1,6 %). 21 patients with HCV-associated CGEV were studied for main clinical and laboratory manifestations of the disease. Kidney and liver involvement was confirmed morphologically and immunomorphologically. Electroneurophysiological investigation evaluated peripheral nervous system involvement. Biopsy of parotid, lacrimal glands, peripheral lymph nodes, splenectomy and bone marrow trephine biopsy with morphological study and immunohistochemistry were used to identify type of lymphoma. Characteristics of monoclonal secretion were assessed with high-resolution electrophoresis in agarose gel with subsequent immunofixation of sera and concentrated urine. Results. Liver involvement was detected in 66 % of patients with HCV-associated CGEV., 34% patients were chronic HCV carriers with persistently normal liver function tests. Common rheumatologic manifestations of HCV-associated CGEV were skin lesions (90%), arthralgia (85%), frequent peripheral nervous system involvement (52%) and glomerulonephritis (38%). Prevalent immunological markers of CGEV associated with HCV were mixed monoclonal cryoglobulinemia with rheumatoid factor activity (62%), rare polyclonal (34%) and olygoclonal (4%) cryoglobulinemia, low levels of С4 compliment fraction (80%). Patients with mixed monoclonal cryoglobulinemia often developed clinical manifestations of Sjögren s syndrome (23 %) and malignant lymphoproliferative diseases (14 %). CGEV is a prognostically adverse sign in HCV infected patients and caused death of 14% patients even in a short period of follow-up (1-2 years).

Copyright of Der Nephrologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Cryofiltration ( CF) is a technique in which separated plasma is chilled before being subjected to a plasma fractionator ( PF), leading to cryoglobulin precipitation or cryogel formation. In CF using the Evaflux-5A as the PF, there is no consensus on the necessity of albumin supplementation, and when or how often the PF column should be washed. We analyzed the sieving effects of various solutes (albumin, IgG, IgM, LDL, HCV-RNA, and cryoglobulin) depending on transmembrane pressure ( TMPPF) during CF using the Evaflux-5A in a patient with hepatitis C virus-associated cryoglobulinemic glomerulonephritis. Five CF treatments were initially performed and a sixth one later, at disease recurrence. Quantitative detection of cryoglobulin and a marked rise in TMPPF to 400 mm Hg were observed only at the first and sixth treatment, and albumin losses during these treatments were very high, at 16.8 g, and 14.6 g, respectively, while those of others (from the second to fifth) were 6.7 g, 6.4 g, 5.9 g, and 7.0 g, respectively. The sieving coefficients ( SCs) of both albumin and IgG were stable (0.8-1.0) at TMPPF < 200 mm Hg, but significantly decreased at TMPPF ≥ 200 mm Hg ( P < 0.01). The SC of IgM tended to decrease at TMPPF ≥ 200 mm Hg, but not significantly, while that of LDL was zero regardless of the TMPPF. Albumin loss per treatment likely depends on degree of TMPPF rise, which is mainly affected by the patient's cryoglobulinemic status. In CF using Evaflux-5A, washing the PF column to keep TMPPF < 200 mm Hg during treatment may be a recommended for selective removal and albumin salvage. [ABSTRACT FROM AUTHOR]

Purpose: Cryoglobulinemic glomerulonephritis (CGGN) is a type of membranoproliferative glomerulonephritis (MPGN) that develops in patients with systemic cryoglobulinemia. To date the exact pathogenesis of CGGN remains unclear. It has been suggested that macrophages may be significant contributors to the glomerular injury in this disease. In our study we attempt to characterize the macrophages in human CGGN using classical activation and regulatory macrophage markers. Material and Method: We searched our database for renal biopsy cases of CGGN. Macrophages were detected using a monoclonal anti-CD68 antibody. Two groups of macrophage markers were used: classical activation markers, including iNOS, CXCL9 and CCL20, and regulatory markers: SPHK1 and LIGHT. The stains were performed using immunohistochemical method. Results: Five patients with CGGN were identified. Four patients had systemic cryoglobulinemia and two had a serological evidence of hepatitis C virus infection. In all cases the glomeruli contained numerous macrophages. Staining for activatory macrophage markers revealed a strong nuclear staining for CXCL9 in numerous cells, including those corresponding to the macrophage location. Staining for the other activatory markers, as well as staining for regulatory markers, was not significant. Conclusion: In this study of human CGGN we showed a striking expression of cytokine CXCL9, a classical macrophage activation marker, by the macrophages and possibly other cell types within the glomeruli. This observation points to the possible role of classically activated macrophages in the pathogenesis of MPGN. If this observation is confirmed on a larger group of patients, the cytokine CXCL9 could become a potential therapeutic target for human CGGN. [ABSTRACT FROM AUTHOR]

The clinicopathologic characteristics and long-term outcome of non-hepatitis-associated cryoglobulinemic glomerulonephritis (CryoGN) are not well-defined and cases with undetectable serum cryoglobulin (seronegative CryoGN) have not been investigated. To resolve this, we retrospectively identified 81 patients with biopsy-proven non-hepatitis CryoGN, including 22 with seronegative CryoGN. The median age was 61 years and 76% presented with nephritic syndrome. A hematologic condition was found in 89% of patients, including monoclonal gammopathy of renal significance (65%) and symptomatic lymphoproliferative disorder (35%). In the seropositive group, 56% had type II, 29% type I, and 8% type III cryoglobulin. Extrarenal manifestations, mostly of skin, were present in 64% and were significantly less common in seronegative CryoGN. Glomerular deposits by immunofluorescence were IgM dominant (84%) and polytypic (70%) in the seropositive group, whereas 52% of seronegative cases had monotypic deposits (i.e., type I cryoglobulin). Ultrastructurally, the deposits were organized in 77% of cases. Substructure appearance significantly differed according to the type of CryoGN, forming most commonly short cylindrical structures in type II and other organized substructures in type I CryoGN. Most patients were treated with clone-directed therapy. On follow up (median 33 months), 77% had partial or complete remission, 10% reached kidney failure and 14% died. Predictors of kidney failure on univariate analysis were AKIN stage 3, positive rheumatoid factor and biclonal gammopathy at diagnosis. We conclude that most CryoGN cases (types I and II) are due to a hematologic condition and are associated with favorable outcome after clone-directed therapy. Seronegative CryoGN accounts for about a quarter of cases and is mostly a kidney-limited disease. Thus, further investigations are needed to unravel the pathophysiology of seronegative CryoGN., (Copyright © 2022 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

The clinical course of cryoglobulinemic glomerulonephritis (CGGN) is usually slowly progressive, and only a minority of these patients progress to end-stage renal failure. This report describes an atypical case of a patient with CGGN who demonstrated a rapidly progressive and irreversible renal deterioration. After presenting with symptoms of an upper respiratory infection, the patient exhibited oliguric acute renal failure, which was followed by systemic efflorescence accompanied by a fever. The laboratory analyses of his serum revealed the patient to be cryoglobulin-positive with markedly decreased serum C4 levels and an increased anti-streptolysin O titer. A serological test for the hepatitis C virus was negative. A renal biopsy showed that the patient had diffuse endocapillary and extracapillary proliferation together with marked endoluminal thrombi and subendothelial deposits in glomeruli. An electron microscopic examination demonstrated the presence of electron-dense subendothelial, subepithelial and huge intraluminal deposits with a specific annular and cylindrical structure. These features were consistent with active and severe CGGN. Despite aggressive treatment with corticosteroid pulses and a plasma exchange, a second renal biopsy demonstrated further advanced renal injury and revealed no signs of recovery. [ABSTRACT FROM AUTHOR]

Waldenström's macroglobulinemia (WM) is a slowly progressive, low-grade B cell lymphoproliferative disorder. In contrast to the indolent progression, the development of cryoglobulinemic glomerulonephritis associated with WM is a rare, aggressive, and life-threatening complication. We describe the case of a 53-year-old man who suffered from WM, which was accompanied by cryoglobulinemic glomerulonephritis. WM was diagnosed on the basis of an increase in monoclonal IgM kappa and infiltration of abnormal lymphoplasmacytic cells in the bone marrow. Moreover, the case was complicated by increase in the levels of urinary protein, serum creatinine, and serum cryoglobulin. Histological findings showed endocapillary glomerulonephritis with hyaline plugs. Electron microscopy demonstrated the accumulation of electron-dense deposits in the subepithelial, subendothelial, intramembranous, and mesangial areas, which revealed cryoglobulinemic proliferative glomerulonephritis. The patient received four courses of rituximab therapy followed by four courses of R-CHOP therapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) in combination with cryofiltration. Subsequently, the patient underwent high-dose chemotherapy (melphalan [L-PAM]) followed by tandem autologous peripheral blood stem cell transplantation. After these treatments, the patient remained disease-free for 26 months. Histological findings of cryoglobulinemic glomerulonephritis were markedly improved after these treatments. Our case suggests that these treatments may be a feasible, safe, and effective strategy for critical cryoglobulinemic glomerulonephritis derived from WM. [ABSTRACT FROM AUTHOR]

Type II mixed cryoglobulinemia (MC) is a systemic vasculitis, associated in most cases with hepatitis C virus (HCV) infection, sustained by proliferation of oligoclonal cells. Systemic B cell depletion and clinical remission can be achieved in non-Hodgkin lymphoma by human/mouse chimeric monoclonal antibody that specifically reacts with the CD20 antigen (rituximab). Similar effects could be expected in type II MC. Twelve patients, mean age 61.9 years (range 37–76), 11 with HCV infection genotype 2a2c (4 cases) or 1b (6 cases) and 3 (1 case) and symptomatic type II MC with systemic manifestations, including renal involvement, marrow clonal restriction, large necrotizing ulcers, and polyneuropathy, were considered eligible for rituximab therapy because of resistance or intolerance to conventional therapy or important bone marrow infiltration. Rituximab was administered intravenously at a dose of 375 mg/m2 on days 1, 8, 15, and 22. Two more doses were administered 1 and 2 months later. No other immunosuppressive drugs were added. Response was evaluated by assessing the changes in clinical signs, symptoms, and laboratory parameters. Levels of proteinuria, hematuria, erythrocyte sedimentation rate, cryocrit, rheumatoid factor, and IgM decreased while C4 values increased and HCV viral load remained stable during short- and medium-term observation. Bone marrow abnormalities were found to reverse to normal. Constitutional symptoms disappeared or ameliorated. No acute or delayed side effects were seen. Based on this experience and a number of reports published in the last 5 years, Rituximab appears to be a safe and effective therapeutic option in symptomatic patients with HCV-associated MC with signs of systemic vasculitis. [ABSTRACT FROM AUTHOR]

A 65-year-old-woman presented with edema, ascites, proteinuria and abnormal liver function tests. A small amount of mixed cryoglobulin was detected in her serum. Liver biopsy revealed mild chronic active hepatitis, but tests for hepatotropic viral infection were negative. Electron microscopy of the renal biopsy revealed glomerular electron-dense deposits that contained numerous tubular structures. Renal amyloidosis and light chain deposition disease were ruled out by appropriate histological techniques. The ultrastructural findings of renal biopsy suggested either cryoglobulinemic glomerulonephritis or immunotactoid glomerulopathy. Although the exact interrelationship among the peculiar glomerulopathy, cryoglobulinemia and chronic active hepatitis in the present case remains undetermined, this report enlarges the spectrum of glomerulopathy characterized by extracellular deposition of microtubules. [ABSTRACT FROM AUTHOR]

Background: Cryoglobulinemic glomerulonephritis (Cryo-GN), caused by circulating cryoglobulins, has varied etiology and clinical-pathologic manifestations. This study aimed to investigate the clinicopathological spectrum and outcome of patients with various Cryo-GN in China., Methods: A retrospective review of 74 Chinese patients with biopsy-proven cryoglobulin-related renal lesions in Peking University First Hospital from 2010 to 2020 was performed., Results: The mean age at diagnosis was 52.9 ± 15.0 years, and the female-to-male ratio was about 2/5. For the etiology screening, serum/urine monoclonal immunoglobulin could be detected on immunofixation electrophoresis in 34% of patients, including 6 patients who had hematological malignancies. Fifty-seven percent of patients had HBV infection, far more than HCV infection (5%). Ten percent of patients had other infections, and 27% of patients were classified as essential or idiopathic. Eleven out of the 15 patients with type II cryoglobulinemia had a consistent monotype of serum monoclonal immunoglobulins and monoclonal cryoprecipitate. The clinical manifestations were similar between various types of cryoglobulinemia. Hematuria, proteinuria, hypertension, anemia, and chronic renal insufficiency were the most common features. Fifty-three percent of patients presented with nephrotic syndrome, and 32% experienced acute kidney injury. Hypocomplementemia, serum-positive rheumatoid factor activity, and skin lesions were reported in 45%, 29%, and 28% of patients, respectively. After a median of 24 months follow-up, 18 patients reached end-stage kidney disease. The clone-targeted treatment could retard the renal deterioration compared with immunosuppressive therapy., Conclusions: This was the largest single-center, clinicopathological retrospective study of Cryo-GN in China. Our data strongly support the association between monoclonal gammopathy and type II Cryo-GN. The renal responsive rate of immunosuppressant therapy is still suboptimal. The clone-targeted treatment shows promising effects in patients with type I or II Cryo-GN., Competing Interests: The authors have no conflicts of interest to declare., (Copyright © 2022 by S. Karger AG, Basel.)

Infection with hepatitis C virus has been associated with a number of extrahepatic manifestations, including kidney disease. Of the glomerular pathologic states described with hepatitis C virus infection, cryoglobulinemic glomerulonephritis is the most prevalent. On kidney biopsy, cryoglobulinemic glomerulonephritis has a variable appearance, with a membranoproliferative pattern of injury as the most common light microscopic finding. Ultrastructurally, curved and paired microtubules are the most characteristic finding, but these also can be variable. We present a case of cryoglobulinemic glomerulonephritis with distinct and highly unusual ultrastructural findings., (Copyright © 2013 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder associated with bone marrow involvement of lymphoplasmacytic lymphoma (LPL) and an IgM monoclonal gammopathy. Generally B-lymphocytes in LPL do not express CD5 that is important for differential diagnosis of B-cell lymphoproliferative disorders. In WM, various renal diseases and type I cryoglobulinemia are well described separately, but cryoglobulinemic glomerulonephropathy is very rarely reported. A 61-yr-old woman complained of generalized edema, cyanosis of the extremities in cold weather, visual disturbance, and pancytopenia. Bone marrow and renal biopsy showed CD5+ expressing B-cells and cryoglobulinemic glomerulonephropathy. With the diagnosis of WM, she received cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy and got complete remission. Here, we report a rare case of WM associated with unusual expression of CD5+ B-lymphocytes and cryoglobulinemic glomerulonephropathy, and emphasize the importance of the clinical features in differentiating CD5+ B-cell lymphoproliferative disorders.