Unusual case of crescentic glomerulonephritis associated with malignant lymphoma. A case report and review of the literature

Renal lesions in non-Hodgkin's lymphoma are rare. Furthermore, to the best of our knowledge, only 5 cases of crescentic glomerulonephritis associated with non-Hodgkin's lymphoma have been previously described. We report a case of crescentic glomerulonephritis and renal failure which preceded the diagnosis of non-Hodgkin's lymphoma. Following steroid therapy there was a resolution of these histological findings a year later.     

Proliferative glomerulonephritis associated with mantle cell lymphoma--natural history and effect of treatment in 2 cases

We describe 2 patients with mantle cell lymphoma who presented with dialysis-dependent acute renal failure and in whom the renal biopsies showed proliferative glomerulonephritis. The first patient had lymphadenopathy and the second splenomegaly, but no cause was initially identified in either case. The first patient was treated with immunosuppressive drugs, the second was given no specific therapy; renal function recovered in both. However, more than 1 year later, both again became dialysis-dependent but had also developed generalized lymphadenopathy. A diagnosis of mantle cell non-Hodgkin's lymphoma was made in both cases. The association of active lymphoma and renal disease supports a paraneoplastic mechanism for the occurrence of the glomerulonephritis in these patients. The literature describing the association between non-Hodgkin's lymphoma and glomerulonephritis is reviewed.     

Renal failure associated with polyarteritis nodosa

We reported 5 patients with renal failure associated with polyarteritis nodosa (PN). In all patients the renal dysfunction became apparent from 2 to 4 weeks after the onset of fever or neuromuscular symptoms with laboratory findings supporting marked inflammatory process and took the course of either acute or rapidly progressive renal failure. The clinical diagnoses on admission were variable; fever of unknown origin, peptic ulcer disease, polyneuritis multiplex, disseminated intravascular coagulation and Buerger's disease. Microscopic examination of the kidneys in 3 by renal biopsy and in 2 by autopsy revealed crescentic glomerulonephritis without immunoglobulin deposit, segmental necrotizing glomerulonephritis, marked inflammatory infiltrate around the glomeruli, granulomatous lesion, tubular necrosis and cortical necrosis other than necrotizing vasculitis. The outcomes were favorable with prednisolone monotherapy in two patients who had biopsy-proven diagnosis and treatment early in the course. Of all PN the prevalence of renal involvement is considered to be about 70%, the prognosis of which has been considered very poor. However it seems possible to alter fatal outcome of this disease by starting treatment with confident histological proof early in the disease process.     

A case of acute renal failure caused by Hodgkin's lymphoma: concurrent membranous glomerulonephritis and interstitial HL-CD 20 lymphoid infiltration

Although acute renal failure developing due to malignancies is a frequent condition, malignant renal infiltration is rarely observed among these causes. Among all malignant diseases, the hematolymphoid malignancies are the most prone to renal infiltration. Other types involved in cases with lymphoma are glomerulopathies, including immune-complex glomerular diseases such as minimal change disease, membranous glomerulonephritis, membranoproliferative glomerulonephritis, and focal segmental glomerulosclerosis. We present herein the rare case of a 22-year-old male with both membranous glomerulonephritis and CD20 (+) lymphoid infiltration related to Hodgkin's lymphoma in the renal interstitial tissue, as detected on biopsy. The patient was treated with adriamycin, bleomycin, vinblastine, and dacarbazine protocol after pulse corticosteroid treatment, and a dramatic improvement in renal function was observed after 2 days of treatment. In this article, an exceptional renal involvement of Hodgkin's lymphoma is discussed in light of the related literature.     

Glomerulonephritis and non-Hodgkin's lymphoma: a report of two cases and review of the literature.

Two cases of glomerulonephritis associated with non-Hodgkin's lymphoma (NHL) are described. The first patient presented with the nephrotic syndrome and normal renal function, whereas the second suffered from recurrent acute renal failure together with a unique pattern of IgM deposition within glomerular capillaries. Our review of the literature suggests that this association, although rare, has been documented in a sufficient number of cases to show that it is more than coincidental. Whereas the most common renal lesion associated with Hodgkin's disease is minimal change disease, more advanced glomerular changes are found in the patients with NHL. This is reflected in the higher incidence of renal failure in the latter patients. Treatment of the lymphomas has been shown to result in improvement or even cure of the renal disease, although long-term follow-up is rarely available in the reported cases.     

Glomerulonephritis, non-Hodgkin's lymphoma and leucocytoclastic vasculitis

2 cases of active focal proliferative glomerulonephritis, lymphocytic malignant lymphoma and leucocytoclastic vasculitis are described. 1 presented with the nephrotic syndrome and progressive renal impairment, the other with acute renal failure. Cryoglobulins were detected in the serum of 1 patient, but not in that of the other. Initial renal and skin biopsies in the 2 patients were strikingly similar. As well as demonstrating glomerular lesions, the renal biopsies contained interstitial lymphomatous infiltrate which, in 1 case, was initially incorrectly interpreted and resulted in delay in specific treatment. Immunosuppressive therapy markedly improved renal function and induced remission of the vasculitis and lymphoma. Repeat renal biopsy in 1 case confirmed regression of the glomerulonephritis. A common immunological aetiology may explain the co-existence of these three conditions in the 2 patients.     

Membranoproliferative glomerulonephritis, mantle cell lymphoma infiltration, and acute kidney injury

Mantle cell lymphoma (MCL) is a rare aggressive lymphoid neoplasm occurring in about 3–7 % of non-Hodgkin lymphomas in the United States and Europe. Although lymphomas infiltrations are recognized in about half of post-mortem studies, in available literature we found only eight cases of mantle cell lymphoma with renal involvement. Five of them present MCL related glomerulonephritis, two show renal MCL infiltration with acute kidney injury and the last one describes MCL infiltration with acute tubulo-intrerstitial nephritis. We present the first case of a patient with the coexistence of renal mantle cell lymphoma infiltration, subacute membranoproliferative glomerulonephritis and acute kidney injury.     

Childhood microscopic polyangiitis associated with MPO-ANCA

We reviewed the clinical, histological and serological parameters of microscopic polyangiitis (MPA) associated with antineutrophil cytoplasmic antibodies (ANCA) specific to myeloperoxidase (MPO). Six girls and one boy aged 12.0±2.6 years (7–15 years) met the following inclusion criteria: (1) clinical manifestations of systemic small vessel involvement; (2) histological demonstration of pauci-immune necrotizing glomerulonephritis; and (3) serological findings of increased concentration of MPO-ANCA by ELISA test. The main clinical manifestations were: influenza-like symptoms (100%), hematuria/proteinuria (100%), purpura (100%), pulmonary-renal syndrome (57%), acute renal failure (ARF) (29%), ischemic cerebral insults (29%), and necrotizing vasculitis of the skin (29%). All patients underwent renal biopsy examined by immunohistochemistry with expression of alpha-smooth muscle actin (alpha SMA) in glomerular and interstitial spaces. Patients were followed from 6 months to 5.5 years (35.4± 23.2 months). None of the patients died. Two of seven children who had ARF progressed to end stage renal disease; one developed chronic renal failure, and four normalized renal function. ARF and central nervous system involvement at presentation were parameters of poor renal outcome. A high score of fibro-cellular glomerular crescents was associated with worse prognosis. Early treatment enables a favorable prognosis of MPO-ANCA-associated MPA in children.     

Fortuitous vasculitis

A 43-year-old man with a cardiac device for dilated cardiomyopathy presented with fever, night sweats, and weight loss. Investigations revealed pancytopenia, acute renal failure, abnormal lung function, and raised inflammatory markers. A renal biopsy demonstrated pauci-immune necrotizing crescentic glomerulonephritis. He was diagnosed with pulmonary-renal antineutrophil cytoplasmic antibody-negative systemic small vessel vasculitis. He commenced immunosuppression with prednisolone and cyclophosphamide with recovery from pancytopenia and improvement in renal function 3 months later. Subsequently, a bone marrow culture grew Mycobacterium fortuitum. Isolation on repeat peripheral mycobacterial blood cultures prompted treatment with ciprofloxacin and clarithromycin. Four months later, he presented with neutropenic sepsis, influenza A/H1N1, and Aspergillus flavus pneumonia. Despite treatment he deteriorated. A transthoracic echocardiogram revealed a vegetation on the right ventricular pacing wire. The device was removed. The vegetation revealed acid and alcohol fast bacilli on Ziehl-Neelsen staining and grew M. fortuitum on culture, sensitive to ciprofloxacin and clarithromycin. Despite device removal and antimicrobial therapy, the patient succumbed to treatment-related complications. The association between glomerulonephritis and endocarditis is well known; however, this is the first case to our knowledge describing pauci-immune necrotizing crescentic glomerulonephritis in the context of M. fortuitum endocarditis. Clinicians should maintain a high index of suspicion for endocarditis in patients with a cardiac device who present with fever and pauci-immune necrotizing crescentic glomerulonephritis. Patients should be investigated with mycobacterial blood cultures, at least three sets of standard blood cultures and transthoracic and transesophageal echocardiography. Clinicians should beware the perils of immunosuppression in the face of an occult sepsis.     

IgA glomerulonephritis associated with microscopic polyangiitis or Churg-Strauss syndrome.

When renal insufficiency occurs in classical antineutrophil cytoplasm antibody- (ANCA) associated vasculitides, histological examination usually finds pauci-immune focal segmental glomerulonephritis. We report on 2 cases of histologically proven necrotizing vasculitis associated with IgA nephropathy. Concomitant vasculitis and IgA nephropathy has only rarely been reported but this joint occurrence may not be coincidental as its pathophysiology is not known. Among vasculitides, IgA nephropathy has more frequently been associated with Henoch-Schoenlein purpura: one microscopic polyangiitis unusual because the patient simultaneously presented ANCA and microaneurysms, and the other Churg-Strauss syndrome associated with mild renal insufficiency. This uncommon association might represent a possible overlap syndrome between these ANCA-associated vasculitides and IgA nephropathy or simply a new type of glomerulonephritis that must be taken into account in these vasculitides.     

Reversible acute renal failure from gross haematuria due to glomerulonephritis: not only in IgA nephropathy and not associated with intratubular obstruction

Seven patients with acute renal failure due to gross haematuriacaused by glomerulonephritis are described. Gross haematurialasting 4–40 days led to acute impairment of renal functionof variable severity (peak plasma creatinine 1.3–12 mg/dl)and duration. While partial recovery of renal function occurredin all patients within few days, complete remission was observedonly some months later. Three patients had IgA nephropathy (2the primary form and 1 nephritis secondary to Schönlein-Henochpurpura), two patients had acute postinfectious glomerulonephritis,andtwo others had focal necrotizing (pauci-immune) glomerulonephritis.The glomerular changes seen in renal biopsy were not enoughto explain per se the renal function impairment. Tubular changes,however, were severe and consisted of tubular necrosis, erythrocytecasts, erythrocyte phagocytosis by tubular cells, accompaniedby interstitial damage (oedema, red-cell extravasation, andinflammatory infiltrates). Study of the renal biopsies by immunofluorescencerevealed retrodiffusion of Tamm-Horsfall protein into the glomerularBowman’s space, a sign of obstructed tubular flow in anycase. It is concluded that acute renal failure due to grosshaematuria in glomerulonephritic patients may not occur onlyin IgA nephropathy, as reported so far, and is not associatedwith intratubular obstruction.     

Association of IgA nephropathy with Clostridium difficile colitis

Immunoglobulin A (IgA) nephropathy, the most common cause of glomerulonephritis worldwide, is usually idiopathic in origin and renal limited. Secondary IgA nephropathy has been associated with systemic disease, including such gastrointestinal tract disturbances as celiac sprue and inflammatory bowel disease. We describe gross hematuria and reversible acute renal failure from IgA nephropathy in a patient with cephalosporin-induced Clostridium difficile colitis. In addition to mesangial IgA and C3 deposition, renal histological examination showed glomerular bleeding, intratubular red blood cell casts, and acute tubular necrosis. To the best of our knowledge, this is the first report of an association between IgA nephropathy and C difficile colitis.     

Fortuitous Vasculitis

A 43-year-old man with a cardiac device for dilated cardiomyopathy presented with fever, night sweats, and weight loss. Investigations revealed pancytopenia, acute renal failure, abnormal lung function, and raised inflammatory markers. A renal biopsy demonstrated pauci-immune necrotizing crescentic glomerulonephritis. He was diagnosed with pulmonary–renal antineutrophil cytoplasmic antibody-negative systemic small vessel vasculitis. He commenced immunosuppression with prednisolone and cyclophosphamide with recovery from pancytopenia and improvement in renal function 3 months later. Subsequently, a bone marrow culture grew Mycobacterium fortuitum. Isolation on repeat peripheral mycobacterial blood cultures prompted treatment with ciprofloxacin and clarithromycin. Four months later, he presented with neutropenic sepsis, influenza A/H1N1, and Aspergillus flavus pneumonia. Despite treatment he deteriorated. A transthoracic echocardiogram revealed a vegetation on the right ventricular pacing wire. The device was removed. The vegetation revealed acid and alcohol fast bacilli on Ziehl–Neelsen staining and grew M. fortuitum on culture, sensitive to ciprofloxacin and clarithromycin. Despite device removal and antimicrobial therapy, the patient succumbed to treatment-related complications. The association between glomerulonephritis and endocarditis is well known; however, this is the first case to our knowledge describing pauci-immune necrotizing crescentic glomerulonephritis in the context of M. fortuitum endocarditis. Clinicians should maintain a high index of suspicion for endocarditis in patients with a cardiac device who present with fever and pauci-immune necrotizing crescentic glomerulonephritis. Patients should be investigated with mycobacterial blood cultures, at least three sets of standard blood cultures and transthoracic and transesophageal echocardiography. Clinicians should beware the perils of immunosuppression in the face of an occult sepsis.     

Mesangial proliferative glomerulonephritis with Legionnaires' disease. A case report

Although the clinical and biochemical features of renal involvement in Legionnaires' disease have been well described, only a few case reports detailed the histological changes. A patient with Legionnaires' disease who developed acute renal failure is described; a biopsy specimen revealed mesangial proliferative glomerulonephritis. The renal morphological changes in Legionnaires' disease are reviewed.     

Enterococcal endocarditis associated with crescentic glomerulonephritis

Glomerulonephritis secondary to infective endocarditis (IE) is an uncommon diagnosis and is usually associated with cardiac valvular infection by blood-culture-positive bacteria. We report a case of necrotizing glomerulonephritis associated with culture-positive endocarditis caused by Enterococcus faecalis. The patient presented with renal abnormalities and was further investigated by renal biopsy. He had immune complex-mediated necrotizing and crescentic glomerulonephritis with mesengial and capillary deposition of immunoglobulin M (Ig M), Ig G, and complement 3 (C3). He was treated with antibiotics, including ampicillin and gentamicin. In addition, steroid and cyclophosphamide were administered. The patient died of renal failure 48 days after hospital admission. In conclusion, glomerulonephritis caused by Enterococcus faecalis endocarditis is an immune-complex-mediated disease characterized by necrotizing and crescentic glomerular lesions that can be fatal despite aggressive antimicrobial and immunosuppressive therapy.     

Glomerulonephritis, B monoclonal small lymphocytic lymphoma and mixed cryoglobulinemia

A novel association in the same patient with small lymphocytic lymphoma, type II cryoglobulinemia and glomerulonephritis is reported. This case is also characterized by a quite unusual sequence of glomerular alterations. A first renal biopsy showed severe endocapillary proliferative glomerulonephritis due to monocytic infiltration without any evidence of deposition of immune reactants. The immune deposits associated with type II cryoglobulinemia were only observed at a second renal biopsy performed five months later. This case shows that mononuclear cells can be responsible in and of themselves for severe glomerular damage, without deposition of immune material, and suggests that monocytic infiltration might be the first stage of type II cryoglobulinemia associated glomerulonephritis.     

Pulmonary hemorrhage and necrotizing glomerulonephritis without glomerular immune deposits: report of two cases

Two children with a syndrome of pulmonary hemorrhage and necrotizing, nonimmune glomerulonephritis are reported. A boy and girl, both of East Indian descent, developed recurrent lung infiltrates from the age of 3 months and 2 years, respectively. Both subsequently presented with pulmonary hemorrhage, fever, arthritis, hematuria, and nephrotic range proteinuria at 1.5 and 6 years of age, respectively. Renal biopsy in each case showed acute, severe, focal and segmental, necrotizing and crescentic glomerulonephritis without immune deposits. Subsequent renal biopsies revealed severe glomerular and tubulointerstitial scarring. No vasculitis or granulomas were seen on renal, skin, or lung biopsies. Antineutrophil cytoplasmic antibodies (ANCA) were not detected in sera taken 1.5 years in the boy and 3.5 years in the girl after the onset of renal disease. The boy was treated with prednisone, azathioprine, and plasmapheresis, but developed progressive renal impairment and commenced dialysis within 4 years. Subsequent to renal transplantation, he developed an immunoblastic lymphoma and died. The girl was treated initially with prednisone and later with cyclophosphamide. After 4 years, she had a normal glomerular filtration rate (GFR). While necrotizing nonimmune glomerulonephritis associated with pulmonary hemorrhage is rare, and cases are characteristically difficult to classify because of many overlapping features, it is possible that these children had a unique illness.     

Acute renal failure due to obstruction in Burkitt lymphoma

 Acute renal failure in Burkitt lymphoma is commonly the result of tumor lysis syndrome. We present a 15-year-old boy who developed hypertension, seizures, and acute renal failure due to extrinsic compression of the bladder and ureters by a large retrovesical Burkitt lymphoma. The causes of acute renal failure in Burkitt lymphoma and the incidence of acute urinary obstruction in this disease are reviewed. Received: 18 May 1998 / Revised: 30 June 1998 / Accepted: 1 July 1998     

Acute tubular necrosis caused by gross hematuria in a patient with focal and segmental necrotizing glomerulonephritis.

We describe a patient with gross hematuria, severe renal failure and symptoms suggestive of systemic vasculitis. Renal biopsy showed very focal and segmental necrotizing glomerulonephritis without crescents. A few C3 deposits were seen by immunofluorescence. The tubular lesions, on the contrary, were very severe, consisting of tubular cell necrosis and ruptured tubular basement membrane, associated with large numbers of intraluminal erythrocytes and erythrocytic casts. After gross hematuria had cleared, renal function slowly recovered. Because of biopsy findings and clinical course, acute renal failure in this patient was considered to be due to the tubular lesions caused by gross hematuria.     

Acute renal failure in an adult patient with Henoch-Schoenlein purpura after episode of macroscopic hematuria

A case of severe prolonged acute renal failure with a histological picture of acute tubulointerstitial lesions in an adult patient with Henoch-Schoenlein purpura after an episode of macroscopic hematuria is described. The macroscopic hematuria lasted only for 5 days and the renal biopsy was performed 50 days after the end of the macroscopic hematuria. Restoration of renal function was not complete six months after the beginning of improvement. Fewer than 65 cases of acute renal failure due to tubulointerstitial nephritis in patients with glomerulonephritis and after episode of macroscopic hematuria have been described in the international literature. Only one of these patients was suffering from Henoch-Schoenlein purpura.