Primary central nervous system lymphoma in a renal transplant recipient with Bardet-Biedl syndrome

Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder. End-stage renal failure has been reported as the most frequent cause of death in this disorder. There are few reports of kidney transplantation in these patients. Renal transplant patients are known to be at increased risk for the development of malignancies. Although a few patients with BBS have been described to develop malignant disease, there was no previous association with lymphoma. We report a 20-year-old patient in whom primary central nervous system lymphoma was diagnosed 20 months after renal transplantation.     

Renal Syndromes in the Acquired Immunodeficiency Syndrome (AIDS): Lessons Learned from Analysis Over 5 Years

Renal syndromes associated with the Acquired immunodeficiency syndrome include: potentially reversible acute renal failure, AIDS associated nephropathy which leads to end stage renal disease, and AIDS developing in patients who are being treated by maintenance hemodialysis. The longitudinal study of 95 patients with AIDS and various forms of renal syndrome at two urban institutions indicates that both acute and chronic renal failure is increasing yearly. While some patients with acute renal failure recover renal function and survive for prolonged period, the mortality of dialyzed patients with irreversible renal failure continues to be unsatisfactory. There is a great need for collecting data from high risk areas to analyze the results of maintenance dialysis therapy in patients with AIDS, to assess the economic impact of uremia therapy, and for long-term planning of available resources.     

Acquired immunodeficiency syndrome and the kidney

Initial autopsy studies concerned primarily with the systemic manifestations of the acquired immunodeficiency syndrome (AIDS) did not indicate that significant renal problems were likely to occur in AIDS patients. However, several recent studies have suggested that important renal and electrolyte disorders develop frequently in at least some groups of AIDS patients. In this report, we review current information concerning such disorders and describe our study of the frequency and types of renal lesions in the first 50 AIDS patients undergoing autopsy at this institution. We conclude that a number of renal lesions and electrolyte abnormalities occur in AIDS patients, although the frequency and nature of these problems vary considerably from center to center. Studies from several centers, including our own, indicate that AIDS patients are particularly likely to develop tubulointerstitial lesions (such as nephrocalcinosis and interstitial nephritis) and electrolyte disorders. Additional studies from specific centers in New York City, Miami, Detroit, and Los Angeles indicate that AIDS patients can also develop glomerular lesions, including a variant of focal and segmental glomerulosclerosis (FSGS) associated with heavy proteinuria and rapidly progressive renal failure. Although FSGS is not commonly observed in all centers, AIDS patients with this lesion appear to have a distinctive combination of clinical and pathological features, suggesting that they have a specific "human immunodeficiency virus (HIV)-associated" nephropathy. Preliminary evidence suggests that this lesion may be related to direct renal HIV infection, although confirmation of this possibility is needed. The approach to the AIDS patient with renal disease should involve correction of reversible disorders and consideration of dialysis as necessary.     

Hepatitis C virus-associated glomerular disease in patients with human immunodeficiency virus coinfection.

Chronic infection with hepatitis C virus (HCV) has been linked to the development of glomerular disease. HCV infection is highly prevalent among intravenous drug users, a population that is also at risk for HIV coinfection. This study reports the clinical-pathologic features and outcome of HCV-associated glomerular disease (HCV-GD) in 14 patients with HIV coinfection. All were intravenous drug users and all but one were African-Americans. Renal presentations included renal insufficiency, microscopic hematuria with active urine sediment, hypertension, and nephrotic syndrome or nephrotic-range proteinuria without hypercholesterolemia. Hypocomplementemia and cryoglobulinemia were present in 46 and 33% of patients, respectively. The predominant renal biopsy findings were membranoproliferative glomerulonephritis type 1 or type 3 (Burkholder subtype) in 79% of patients and membranous glomerulopathy with atypical features in 21% (including overlap with collapsing glomerulopathy in one patient). The clinical course was characterized by rapid progression to renal failure requiring dialysis. The overall morbidity and mortality were high with median time of 5.8 mo to dialysis or death. Although most patients died in renal failure, cause of death was primarily attributable to long-term immunosuppression and advanced AIDS. Patients with AIDS had shorter survival than those without (median survival time of 6.1 mo versus 45.9 mo, log-rank test P = 0.02). Only two patients were alive with stable renal function at follow-up of 28.5 mo. In patients with HCV-GD, coinfection with HIV leads to an aggressive form of renal disease that can be easily confused with HIV-associated nephropathy. Although hypocomplementemia, cryoglobulinemia, and more prominent hypertension and microscopic hematuria may provide clues to the presence of HCV-GD, renal biopsy is essential to differentiate HCV-GD from HIV-associated nephropathy.     

Renal failure in patients with AIDS-related complex

The acquired immune deficiency syndrome (AIDS) and AIDS-related complex (ARC) can be associated with nephropathy and renal failure. As the number of AIDS and ARC patients continues to increase dramatically, urologists will be called upon more frequently to help evaluate these patients for reversible causes of renal failure and to obtain tissue for pathologic diagnosis. Eight patients with ARC and renal failure were recently evaluated. Renal biopsies in 4 patients revealed focal and segmental glomerulosclerosis while 2 others showed proliferative and necrotizing glomerulonephritis with crescents. The implications of this study are that ARC patients with renal failure should be fully evaluated and be considered for long-term dialysis. However, the results of renal biopsies in these patients are unlikely to affect patient treatment.     

Sulfadiazine-associated obstructive nephropathy occurring in a patient with the acquired immunodeficiency syndrome

A 45-year-old man with the Acquired Immunodeficiency Syndrome (AIDS) and CNS toxoplasmosis presented with acute renal failure, hematuria, and renal colic shortly after starting treatment with sulfadiazine. Ultrasound examination of his kidneys was suggestive of intraparenchymal crystallization of sulfadiazine. His renal failure and ultrasound findings rapidly resolved with alkaline hydration. On rechallenge with sulfadiazine, he again developed renal insufficiency and ultrasonic findings consistent with stones. The use of sulfadiazine in the treatment of CNS toxoplasmosis in AIDS patients should be monitored carefully with the recognition that this form of crystalline-induced acute renal failure can occur in a dehydrated patient.     

Early renal involvement in acute lymphoblastic leukemia and nonHodgkin's lymphoma in children

Clinical manifestations of kidney disease, particularly renal failure, caused by malignant infiltration in patients with acute lymphoblastic leukemia or nonHodgkin's lymphoma have been described rarely. We report 1 case of acute lymphoblastic leukemia and 3 cases of nonHodgkin's lymphoma in which renal disease was the only or one of the presenting manifestations of malignancy. Of these patients 2 had rapidly progressive renal failure with nephromegaly, 1 presented with bilateral abdominal masses caused by severe nephromegaly and with microscopic hematuria, and 1 had microscopic hematuria without nephromegaly. In all 4 patients kidney biopsy revealed malignant infiltration. In the 2 patients who presented with renal failure kidney function promptly returned to normal after chemotherapy and irradiation of the kidneys. Prompt and correct diagnosis of nephropathy, when it is the only or one of the presenting signs of acute lymphoblastic leukemia or nonHodgkin's lymphoma, is necessary to expedite initiation of specific antitumor therapy.     

Head and neck lymphoma in patients with the acquired immune deficiency syndrome.

A marked increase has recently been noted in the incidence of lymphoma in patients with AIDS. These lymphomas are generally high-grade, of B-cell origin, and often involve extranodal sites. Reported here are twenty patients with AIDS in whom symptoms and physical findings developed related to the head and neck region as a result of lymphoma. The tumor was observed in a variety of sites, including the nasopharynx, orbit, submandibular triangle, anterior and posterior cervical triangles, supraclavicular fossa, and the hypopharynx. Sixteen tumors were large cell nonHodgkin's B-cell lymphomas, three were small cell nonHodgkin's B-cell lymphomas, and one was Hodgkin's disease, mixed cellularity. All were treated with combination chemotherapy. A high degree of suspicion for lymphoma is required in treating any patient with AIDS who has a rapidly enlarging mass in the head and neck. If needle aspiration is nondiagnostic, excisional biopsy should be performed after a complete head and neck evaluation. Although the development of lymphoma associated with AIDS portends a grave prognosis, prompt diagnosis will allow an improved chance of remission of the lymphoma.     

Urological management of indinavir-associated acute renal failure in HIV-positive patients

Introduction Indinavir, a protease inhibitor that is commonly used to treat HIV infection, may cause crystal formation within the renal tubules when urine pH is above 3.5. Crystallization in the urine may lead to intrarenal crystal deposition and acute renal failure (ARF). Aim To establish the beneficial urological management of acute renal failure caused by indinavir treatment of HIV/AIDS patients. Patients––methods Five HIV positive patients (four men, one woman) with a mean age of 32 years (range 28–36 years) were referred to our Department of Urology from an AIDS outpatient Clinic, because of the development of postrenal acute renal failure with continuously elevated creatinine and urea plasma levels after indinavir therapy. Among the initial therapeutic maneuvers, indinavir administration was interrupted for 1 week while bilateral double-J ureteral stents were inserted in all the HIV/AIDS patients, during the first 24–72 h to secure upper-tract drainage. Concurrently urine has been acidified by oral administration of the amino acid l-methionine and oral fluid intake was increased. Results All the patients responded well to the treatment and their renal function was effortlessly restored to normal within a few days. Conclusion HIV-positive patients receiving indinavir therapy might be complicated by acute renal failure, mainly due to intrarenal crystal deposition (tubules) or urolithiasis (postrenal obstruction). This adverse effect may simply manage by the discontinuation of indinavir administration, urine acidification, as well as the possible early insertion of bilateral double-J ureteral stents.     

Abdominal Burkitt's lymphoma: role of early aggressive surgery

Current surgical teaching advocates debulking of the abdominal Burkitt's lymphoma to decrease tumor burden and improve survival. The records of 16 children who had Burkitt's lymphoma were reviewed. Eleven of these children presented with abdominal tumors. Five of these 11 patients are long-term survivors and six have died. Three of the tumors were primarily resected; two were in patients who became long-term survivors. One resection was complicated by acute renal failure, leading to the early demise of the patient. Four tumors were debulked; only one of these was in a long-term survivor. Acute renal failure also complicated the hospital course in one of these children. Finally, four patients underwent initial incisional biopsies and plans were made for subsequent resection of any residual tumor 6 weeks later. Two of these children survived and two have died. The results of this pilot study do not confirm that there is a clear advantage to aggressive operative cytoreduction. Although this approach decreases the total tumor burden, it may also impose severe metabolic complications and postpone the administration of chemotherapy. These results do suggest the need for a multi-institutional review of the surgical management of abdominal Burkitt's lymphoma.     

HIV/AIDS-related hyponatremia: an old but still serious problem

Hyponatremia is the most common electrolyte disorder in hospitals. Many medical illnesses, including congestive heart failure, liver failure, renal failure and pneumonia, may be associated with hyponatremia. In addition, hyponatremia in patients with the acquired immunodeficiency syndrome (AIDS) and AIDS-related complex (ARC) was first reported in 1993. The evidence suggests that severe hyponatremia is associated with increased morbidity and mortality in human immunodeficiency virus (HIV)/AIDS patients; however, the incidence of hyponatremic syndrome in HIV/AIDS patients remains very high in clinical practice, as almost 40% of HIV/AIDS inpatients in Xinjiang, a developing region of China, are hyponatremic. A method for identifying the pathogenesis and therapeutic treatments for hyponatremia in HIV/AIDS patients is needed. This review focuses on the clinical and pathophysiological aspects of hyponatremia and highlights the causes, presentation and treatment recommendations for hyponatremic patients with HIV/AIDS.     

Primary renal lymphoma presenting as acute renal failure

Diffuse bilateral infiltration of the kidneys by lymphoma cells is a rare but well-documented cause of acute renal failure (ARF). Only 51 such cases have been reported, 15 of which had ARF as the initial presentation of lymphoma. The clinical and pathologic features of these 15 cases and of two additional cases reported herein are reviewed. The diagnosis should be suspected in a patient with ARF, bilateral enlargement of the kidneys, minimal proteinuria, nonspecific findings on urinalysis, and absence of features of allergic tubulointerstitial nephritis. Renal imaging techniques may suggest the possibility of lymphomatous infiltration, but only renal biopsy or autopsy can provide a definitive diagnosis. Although modern chemotherapy and/or radiation therapy usually leads to a dramatic normalization of renal function, almost all patients eventually die of widespread recurrent lymphoma, despite the absence of clinical or pathologic involvement of the kidneys at the time of death.     

Splenic Marginal Lymphoma and Glomerulonephritis: Case Report and Review of the Literature

Malignant lymphomas can affect kidneys in several ways. They may precipitate acute renal failure by causing ureteral or renal vascular obstruction, or by direct renal parenchymal infiltration. Furthermore, they may insult renal function via paraneoplastic mechanisms such as hypercalcemia. Lymphomas only rarely can cause glomerulonephritis (GN). We report a case of a 72-year-old male who presented with mild renal function impairment, proteinuria, and microscopic hematuria, suggesting active glomerulonephritis, and pancytopenia of immune origin. A bone marrow biopsy led to a diagnosis of splenic marginal zone lymphoma. Although a kidney biopsy was not performed, glomerulonephritis was attributed to the lymphoma and splenic marginal zone lymphoma-related glomerulonephritis was the final diagnosis. The course of splenic marginal zone lymphoma is extremely indolent. The first manifestation in some patients can be immune cytopenia or other autoimmune phenomena. These patients may respond well to corticosteroids. Therefore, our patient was started on prednisolone resulting in a good hematologic response. Renal function also improved and proteinuria and hematuria disappeared, suggesting a lymphoma-related origin of the GN. Two years after full steroids withdrawal, the patient remained stable with a good renal function and daily protein excretion less than 300 mg. Lymphomas rarely are the cause of secondary glomerulonephritis; however, with a lack of an apparent cause, the clinician should be aware of them, particularly in the elderly with autoimmune manifestations.     

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.     

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 involvement in patients infected with HIV: experience at San Francisco General Hospital

A spectrum of renal abnormalities has been described in patients infected with the human immunodeficiency virus (HIV) with or without signs of the acquired immunodeficiency syndrome (AIDS). In particular, attention has been focused on a nephropathy characterized clinically by nephrotic proteinuria and rapidly advancing renal insufficiency, and histologically by focal and segmental glomerulosclerosis (FSGS). To evaluate the relationship between HIV infection and structural renal disease, we reviewed all consultations between January 1982 and March 1988 to the Division of Nephrology at San Francisco General Hospital (SFGH), a municipal hospital treating approximately one-third of AIDS cases in San Francisco. Seventy-three consultation requests were received during this period regarding patients with AIDS (48), AIDS-Related Complex (23), or asymptomatic HIV infection (2). Of these, 27 gave evidence of structural renal disease (Group I): 14 had chronic renal insufficiency, in 10 of whom nephrotic proteinuria was also present. However, progression of renal insufficiency to end-stage renal disease (ESRD) in this group did not follow the rapid course described for HIV-associated nephropathy. Renal tissue was examined in 11 Group I patients and showed FSGS in four and a variety of acute and chronic glomerular and tubulointerstitial changes in the others. In 46 Group II patients, consultation was requested for acute renal failure or fluid, electrolyte, and acid-base disturbances. We also reviewed 91 consecutive autopsies performed in patients dying with AIDS at SFGH between 1981 and 1986.(ABSTRACT TRUNCATED AT 250 WORDS)     

Acute renal failure due to non-Hodgkin lymphoma infiltration of the kidneys detected by ultrasonography and confirmed by positron emission tomography

Acute renal failure (ARF) as a consequence of non-Hodgkin lymphoma infiltration of the kidneys (LIK) is an uncommon complication of non-Hodgkin lymphoma. In literature, ARF due to LIK is reported in progressive disease. A case of non-oliguric acute renal failure secondary to relapse of large B cell non-Hodgkin lymphoma primarily localized in the mediastinum is reported. LIK of both kidneys was diagnosed by ultrasonography, computer tomography scan and 18-fluorodeoxyglucose positron emission tomography. No other causes for renal failure were found. The prognosis of renal involvement in relapsed non-Hodgkin lymphoma is poor, as is demonstrated by our case.     

原发性肾脏恶性淋巴瘤（附3例报告）

目的 探讨肾脏原发性恶性淋巴瘤的临床特点。方法 总结3例肾脏原发性恶性淋巴瘤患者临床资料，结合文献复习讨论其发病特点、影像学特征、治疗及预后。结果 3例患者中手术活检加化疗1例，手术切除加化疗1例，术前化疗加手术切除加术后化疗1例。3例均诊断为非何杰金淋巴瘤，1例死于肾衰，另2例分别存活38个月和8个月，仍在随访中。结论 肾脏原发性恶性淋巴瘤影像学征象与肾细胞癌相似，肾脏包膜或包膜下弥漫浸润被认为是原发性肾恶性淋巴瘤的特征性表现，通过经皮穿刺活检可明确诊断，治疗应根据组织学分型、分期及肿瘤大小，采取手术联合化疗及放疗。     

Human immunodeficiency virus infection in end-stage renal disease patients

Human immunodeficiency virus (HIV) infection in patients with end-stage renal disease (ESRD) offers many diagnostic and therapeutic challenges to nephrologists. Renal failure may be a direct consequence of viral infection (HIV-associated nephropathy), or intrinsic renal diseases may occur in previously infected individuals. Patients receiving renal replacement therapy (RRT) may acquire HIV infection from blood transfusions, renal allografts, sexual contacts, or needle sharing by drug addicts. In the early 1980s, the overall prognosis of patients with the acquired immunodeficiency syndrome (AIDS) was very poor, and survival of those with ESRD was dismal. Consequently many even questioned the value of providing maintenance dialysis to patients with AIDS. With advances in diagnostic techniques in serologic and viral markers of disease, and deployment of highly effective antiretroviral agents, the prognosis of HIV-infected patients has dramatically improved. Over the past two decades, experiences in the management of HIV patients with ESRD is accumulating. Both peritoneal dialysis and hemodialysis are effective modes of therapy and many centers are now beginning to perform renal transplantation in HIV-infected patients. This article deals with various aspects of HIV infection in patients with ESRD.     

Renal infiltrate by a plasmocytoïd chronic B lymphocytic leukaemia and renal failure: a rare occurrence in nephropathology. A case report and review of the literature

We report the case of a 55-year-old male with renal failure as the initial manifestation of interstitial and focal infiltration of the kidneys by a small B-cell lymphoma. Since three years, this patient had a history of CLL with plasmocytic differentiation and was left untreated owing to stade A Binet classification. After chemotherapy, the lymphocytosis and the adenopathies disappear and the renal function improve. Infiltration of the kidneys by non-Hodgkin small B-cell lymphoma, including chronic lymphocytic leukaemia (CLL), is usually asymptomatic, fortuitously discovered at the time of an X-ray examination or at autopsy. Association with renal failure is extremely rare. We review the reported cases of renal failure associated with lymphomatous infiltration (13 cases of CLL and five cases of lymphoplasmocytic lymphoma kappa or lambda IgM), with the following conclusions: in most cases, renal insufficiency appears in a few months and significantly disappears after chemotherapy; the renal infiltrate is usually focal in lymphoplasmocytic lymphoma and rather massive and diffuse in CLL; the neoplastic feature of a small B-cell lympho?d infiltrate may be difficult to determine: a poorly limited, monomorphous, CD20+ CD5+ lymphoid infiltrate is lymphomatous. In case of plasmocytic differentiation, it must be looked for kappa or lambda monotypy; the type of the lymphomatous infiltrate according to the WHO 2008 classification may be difficult to determine in a small sampling of renal tissue: the renal infiltrate must be compared, if possible, with a lymph node infiltrate. Owing to its bad prognosis, mantle cell lymphoma must be distinguished from other small B-cell lymphoma like CLL/small lymphocytic lymphoma, marginal zone lymphoma and lymphoplasmocytic lymphoma.