Waldenström's macroglobulinemia and nephrotic syndrome with membranous nephropathy

Renal complications of Waldenstr?m's macroglobulinemia (WM) are rarely observed. Nephrotic syndrome in association with WM has most often been secondary to amyloidosis. This article reports a case of WM with nephrotic syndrome as a result of membranous nephropathy with immunoglobulin M (IgM) deposition. A 44-year-old male diagnosed with WM 4 years previously, presented with heavy proteinuria (7.8 g/24 h). Kidney biopsy revealed expanded mesangium, thickened capillary loops and epimembranous spikes, with no significant interstitial inflammation or thickened tubular basement membranes. Immunofluorescence examination demonstrated strong granular staining of IgM and λ chains, with weaker C3 and C1q staining. Electron microscopy showed many subepithelial dense deposits, and fewer large subendothelial dense deposits. Treatment was directed at the patient's WM with maintenance rituximab and fludarabine. Subsequently, decreases were seen in both the patient's serum IgM and serum viscosity. With therapy for WM and the addition of an angiotensin receptor blocker, the patient's proteinuria also improved, from 7.8 g to 4.8 g/24 h. The patient continued to follow up with his hematologist and in 2009 creatinine was 1 mg/dl (76.26 ?mol/l), with a 24 h urine protein excretion of 0.159 g.     

Waldenström's macroglobulinemia and large B-cell central nervous system lymphoma: a case report

BACKGROUND: Waldenstr?m's macroglobulinemia is a low-grade B-cell lymphoma characterized by monoclonal synthesis and secretion of IgM antibodies [3]. The classic symptoms of WM result from blood hyperviscosity. Neurologic symptoms, such as fatigue, dizziness, and blurred vision, are common. The metastatic spread to the CNS, which rarely has been reported, can occur either diffusely or in the form of a mass lesion. CASE DESCRIPTION: We present the case of a 73-year-old woman with intracerebral large B-cell lymphoma and concurrent WM. CONCLUSION: This case illustrates the importance of histopathologic diagnosis by brain biopsy despite an existing diagnosis of systemic neoplasm.     

Nephrotic syndrome due to immunologically mediated hypocomplementic glomerulonephritis in a patient of Waldenström's macroglobulinemia

A 72-year-old man was diagnosed as having nephritic syndrome complicated by Waldenstr?m's macroglobulinemia (WM). A monoclonal IgM lambda protein and decreased serum complements were observed. The renal biopsy disclosed the capillary occluded by thrombi which was stained with IgG, IgA, IgM, C4, lambda light chain and slight kappa light chain in a granular pattern. Electron dense deposits were noted in the subendothelial spaces. An unusual case of WM who developed nephrotic syndrome due to immunologically mediated hypocomplementic glomerulonephritis is described.     

A case with acute renal failure complicated by Waldenström's macroglobulinemia and cryoglobulinemia

We encountered a 53-year-old man associated with acute renal failure caused by Waldenstr?m's macroglobulinemia and type I cryoglobulinemia. Treatment with prednisolone and cyclophosphamide induced a rapid recovery from acute renal failure. Renal histology revealed endocapillary proliferation and lobular formation with scattered subendothelial, amorphous and periodic acid-Schiff (PAS)-positive materials in the glomerular capillaries which were positive for IgM on immunofluorescence study. Although the exact mechanism for pathophysiology of acute renal failure remains unknown, treatment with prednisolone and cyclophosphamide could induce a rapid recovery from acute renal failure accompanied by Waldenstr?m's macroglobulinemia and type I cryoglobulinemia.     

Low-grade T-cell lymphoma of the kidney and Waldenström's macroglobulinemia in a patient presenting with renal failure

Renal failure is rarely the presenting manifestation of non-Hodgkin's lymphoma. We describe the unusual case of a patient who presented with uremia due to lymphomatous infiltration of the kidney by a low-grade T-cell lymphoma. The diagnosis of lymphoma was made by renal biopsy. Extrarenal nodular or extra-nodular involvement could not be detected. However, simultaneously, a lymphoplasmacytic lymphoma was found on bone marrow biopsy associated with IgM paraproteinemia. To our knowledge, this is the first report of a renal T-cell lymphoma associated with Waldenstr?m's macroglobulinemia.     

Clinostatic syndrome in Waldenström macroglobulinemia

We report a case of Waldenstr?m macroglobulinemia revealed by clinostatic syndrome in an 81-year-old woman. A lytic lesion was found in the ilium and acetabulum. There was no evidence of transformation to high-grade lymphoma. Radiation therapy ensured complete resolution of the clinostatic syndrome within 1 month of treatment completion.     

Proliferative glomerulonephritis with monoclonal IgM deposits without Waldenström's macroglobulinemia: case report and review of the literature

A 38-year-old man was presented with nephrotic syndrome associated with hematuria and mild hypocomplementemia. Renal biopsy revealed lobular mesangial proliferation, thickened capillary walls, and intraluminal protein thrombi. Immunofluorescence showed marked and mild depositions of immunoglobulin (Ig) M heavy chains and complement C3, respectively, in a peripheral lobular pattern. On light chain staining, only kappa (κ) light and IgM heavy chains showed a similar pattern. Electron microscopy showed fine granular electrondense deposits in subendothelial areas and numerous globular deposits (varying size and density) in glomerular capillary lumens. Serum levels of Ig κ, but not of free κ, light chains were significantly increased. Bone marrow aspiration revealed a normocellular marrow. Waldenstr?m's macroglobulinemia and cryoglobulinemia were ruled out. Clinically, steroid therapy was ineffective and proteinuria persisted. This report demonstrates that glomerular deposition of monoclonal IgM-κ can produce membranoproliferative- like changes in the glomeruli. This condition may be recognized as proliferative glomerulonephritis with monoclonal IgM deposits similar to the recently recognized proliferative glomerulonephritis with monoclonal IgG deposits.     

Cast nephropathy and light-chain deposition disease in waldenstr?m macroglobulinemia

Waldenstr?m macroglobulinemia is a rare low-grade hematologic malignancy due to clonal proliferation of B lymphocytes responsible for immunoglobulin M (IgM) monoclonal gammopathy secreted in serum. This disease is characterized by lymphoplasmacytic tumoral infiltration of bone marrow and various organs, especially the liver and spleen. Kidney involvement in Waldenstr?m macroglobulinemia has been described previously with reports of various forms of glomerular injury: large intracapillary IgM pseudothrombi, cryoglobulinemia-associated membranoproliferative glomerulonephritis, or amyloidosis. Interstitial infiltration by tumoral B lymphocytes is another classic pattern. Conversely, tubular involvement in the form of myeloma-like casts or basement membrane deposition of monoclonal light chain (light-chain deposition disease) is unusual. We report the occurrence of cast nephropathy associated with light-chain deposition disease in 2 patients with Waldenstr?m macroglobulinemia, which resulted in severe and prolonged kidney failure.     

Renal involvement in Waldenström’s macroglobulinemia: case report and review of literature

Waldenström’s macroglobulinemia (WM) is a rare lymphoid neoplasia, accounting for 2% of all hematological malignancies. Renal complications occur rather rarely compared to multiple myeloma. The most common renal manifestations are mild proteinuria and microhematuria. We describe a case of MW presenting with acute renal failure and NS. A 67-year-old man was referred to our hospital for sudden onset nephrotic syndrome. Electrophoresis revealed a monoclonal component in the gamma region, which was classified as an IgM k. During hospitalization, acute kidney injury developed, with creatinine up to 5?mg/dL, despite adequate hydration and alkalinization. A kidney biopsy was performed, showing minimal change disease (MCD) with interstitial and capsular lymphoid infiltrates of B-Lymphocytes CD20+. B-lymphocytes infiltration suggested the possibility of renal localization of lymphoproliferative disorder. So, bone marrow histology was performed, revealing lymphoplasmacytic lymphoma (WM). The patient was treated with bortezomib, desamethasone, and rituximab, with partial recovery of renal function (creatinine 1.5?mg/dL) and complete remission of proteinuria after 8-month follow-up. The remission of NS in our patient with rituximab seems to emphasize the pathogenetic role of B cells in MCD, although a coincident effect of immunosuppression on both the underlying renal disease and the hematologic disease cannot be excluded.     

Amyloid arthropathy and Waldenström macroglobulinemia

Amyloid arthropathy occurs chiefly as a manifestation of beta(2) microglobulin amyloidosis in patients receiving chronic hemodialysis. AL amyloidosis complicating multiple myeloma is a less common cause. Amyloid arthropathy is exceedingly rare in patients with Waldenstr?m macroglobulinemia. We report a case characterized by lymphoplasmocytoid malignancy with monoclonal IgM production and amyloid arthropathy manifesting as bilateral symmetric polyarthritis. A synovial membrane biopsy established the diagnosis. Chemotherapy was effective in alleviating the joint manifestations. Joint symptoms in patients with monoclonal gammopathies, including those characterized by IgM secretion, should suggest amyloid arthropathy. Treatment of the blood disease may improve the joint symptoms.     

Waldenström's Macroglobulinemia: A possible cause for impairment of skin flap circulation

Summary A patient with Waldenström's Macroglobulinemia (WM) underwent bilateral radical vulvectomy followed by one stage reconstruction of the vulva using bilateral supero-medial thigh skin flaps. Necrosis of the distal portion of the left flap, observed post surgery, could be attributed to three findings associated with WM : IgM macroglobulin, cryoprecipitation and protein-protein interaction. Both careful pre-operative assessment of the patient's haematorheologic profile and consideration of ways of decreasing blood viscosity may be helpful in improving flap survival. Monitoring of body temperature and warming the flap during surgery is considered important. Infusion with low molecular weight dextran intra-operatively and post surgery may also help in limiting thrombosis within the flap.     

A case of Henoch-Schönlein purpura in a patient on hemodialysis

Henoch-Sch?nlein purpura(HSP) is a systemic vasculitis and characterized by the tissue deposition of IgA-containing immune complexes. A 50-year-old man with end stage renal failure due to diabetic nephropathy on maintenance hemodialysis, presented purpura, hematuria, abdominal pain, and joint pain. He also presented a high fever with neutrophilia. Biopsy of skin lesions revealed inflammation of the small vessel accompanied by vascular IgA deposition. Based on the clinical symptoms and skin biopsy, we made the diagnosis of HSP. Oral prednisolone was administered resulting in an improvement of the clinical symptoms. A skin biopsy should be performed for histological and immunofluorescence studies in the case of clinical suspicion of HSP with end stage renal disease on hemodialysis.     

Glomerulonephritis without IgA deposits in a case of Henoch-Schönlein purpura

A boy aged 3 years 8 months with Henoch-Schönlein purpura (HSP) developed significant proteinuria with hematuria 2 days after the appearance of purpura rash. Although thought to be purpura nephritis, a percutaneous renal biopsy revealed diffuse mesangial proliferative glomerulonephritis (MesPGN) without deposition of immunoglobulin A or complement. Since his urine screening test during a health check at the age of 3.5 years had been unremarkable, HSP might have played a role in the pathogenesis of his non-IgA MesPGN. To our knowledge, non-IgA MesPGN is an uncommon renal manifestation of HSP.