Precursor B-cell lymphoblastic leukemia as a cause of a bilateral nephromegaly

Nephromegaly and non-oliguric acute renal failure is an unusual manifestation of lymphoblastic infiltration of the kidneys. We report the clinical history of a female child where a precursor B-cell lymphoblastic proliferation was diagnosed at the age of 21 months by a surgical renal biopsy for an unexplained bilateral nephromegaly. Lymphoblastic infiltration should be suspected in any patient presenting with unexplained renal failure and enlarged kidneys. The importance of renal biopsy to identify the etiology of renal failure and nephromegaly is emphasized.     

Renal biopsy diagnosis of acute lymphocytic leukemia.

Hyperuricemia, due to inborn errors of metabolism, dehydration, or tumor lysis, may cause renal insufficiency. Hyperuricemia from tumor lysis syndrome in malignancy is usually associated with electrolyte disturbances such as hyperkalemia, hyperphosphatemia or hyper or hypocalcemia. Tumor infiltration into the kidneys can occur, yet this accounts for renal insufficiency in only 1% of patients. This infiltration of tumor cells into the kidneys is usually associated with evidence of malignancy elsewhere as identified by physical exam, radiographic studies, and examination of the peripheral smear or bone marrow. We report an unusual presentation of a child with acute lymphocytic leukemia presenting with acute renal failure, nephromegaly and hyperuricemia without electrolyte disturbances or systemic evidence of tumor elsewhere. We stress the importance of kidney biopsy in order to identify the etiology of the renal failure and hyperuricemia.     

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.     

Primary renal lymphoma and xanthogranulomatous pyelonephritis in childhood

Primary renal non-Hodgkin's lymphoma is very rare in childhood. A six-year-old boy presented with bilateral non-obstructive multinodular nephromegaly and renal failure. Percutaneous needle biopsy showed large-cell lymphoma. The patient was started on chemotherapy. A right nephrectomy was done when systemic hypertension developed in the presence of a non-functional right kidney. Histopathologic examination revealed focal lymphomatous infiltration and xanthogranulomatous pyelonephritis which is an atypical form of chronic renal infection. The case is discussed in relation to previons reports.     

Diagnosis and characterization of non-Hodgkin's lymphoma in a patient with acute renal failure

This case illustrates the relatively rare occurrence of malignant lymphoma presenting as acute renal failure due to renal parenchymal infiltration. To our knowledge, it is the first report in which the phenotype of a non-Hodgkin's lymphoma was established using renal biopsy tissue. The dangers of treatment of renal lymphoma in the patient whose disease has not been adequately characterized have been noted by Coggins.     

Acute renal failure secondary to small cell lung cancer with tumor infiltration of the kidneys

Acute renal failure secondary to tumor infiltration of the kidneys is uncommon and largely described in patients with lymphoma or leukemia. We report a 64-year-old man previously diagnosed with limited stage small cell lung cancer who presented with acute renal failure (ARF). Renal imaging showed bilateral enlargement with features suggestive of an infiltrative process. A kidney biopsy established the diagnosis of metastatic small cell lung cancer with diffuse renal parenchymal infiltration. This case emphasizes the rare potential for cancers to metastasize to the kidneys, which can result in ARF. Early recognition of this cause of ARF is crucial, in particular, when the tumor is amenable to chemotherapy or irradiation.     

Acute Renal Failure Secondary to Small Cell Lung Cancer with Tumor Infiltration of the Kidneys

Acute renal failure secondary to tumor infiltration of the kidneys is uncommon and largely described in patients with lymphoma or leukemia. We report a 64-year-old man previously diagnosed with limited stage small cell lung cancer who presented with acute renal failure (ARF). Renal imaging showed bilateral enlargement with features suggestive of an infiltrative process. A kidney biopsy established the diagnosis of metastatic small cell lung cancer with diffuse renal parenchymal infiltration. This case emphasizes the rare potential for cancers to metastasize to the kidneys, which can result in ARF. Early recognition of this cause of ARF is crucial, in particular, when the tumor is amenable to chemotherapy or irradiation.     

Rapidly progressive glomerulonephritis as a presenting feature of chronic lymphocytic leukemia

We report a 64-year-old man who was referred with gross hematuria and acute renal failure. Initial laboratory data showed marked lymphocytosis. A diagnosis of chronic lymphocytic leukemia was made by bone marrow biopsy. In the mean time, kidney biopsy showed crescentic glomerulonephritis and interstitial infiltration with leukemic cells. To our knowledge, there are only three previous reports of rapidly progressive glomerulonephritis that occurred simultaneously with chronic lymphocytic leukemia.     

A case of renal failure due to primary cholesterol atheroembolism

We report a patient presenting rapid deterioration of renal function due to primary cholesterol atheroembolism. The patient was 75-year-old hypertensive male and was admitted to a hospital because of rt. hemiplegia which developed 2 weeks earlier. On admission, his blood pressure was 200/100 mmHg and serum creatinine level was 2.9 mg/dl with urinalysis 1+ both for protein and hematuria. 2 weeks later, an angiotensin converting enzyme inhibitor (ACE inhibitor, delapril 15 mg/day) was given to control high blood pressure. Immediately after this medication, his renal failure rapidly progressed with a fall in blood pressure (110/60 mmHg) and oliguria (100 ml/day). Although he was transferred to our hospital and was treated with hemodialysis, he died of an attack of acute myocardial infarction in a week. At post-mortem examination, microscopic findings of the kidney disclosed numerous occlusions of medium-sized artery by cholesterol emboli. These emboli were also observed in other organs, but not so prominent as in the kidney. The coronary arteries exhibited severe sclerosis. In this presented case, acute deterioration of renal function was caused by ACE-inhibitor, although which was administered in a volume depleted condition. Therefore, further study would be necessary whether or not ACE-inhibitors predispose the patients with this disease to acute renal failure.     

Reversible Renal Failure Due to Bilateral Renal Sarcoma in a Patient with Acute Myeloid Leukemia

A 68-year-old male presented with acute myeloid leukemia, renal failure, hypokalemia, and enlarged kidneys on renal ultrasound. Renal biopsy revealed massive leukemic infiltration of the kidney. After systemic chemotherapy, the patient developed tumor lysis syndrome followed by a phase of proximal tubule dysfunction presenting as polyuria and diverse electrolyte abnormalities. In time, renal function returned to normal, as did kidney size. This report shows that renal failure, enlargement of the kidneys, and tubule dysfunction in the course of AML infiltrating the kidneys can be reversed by treatment of the hematological disease.     

Clinical features and natural history in adults with IgA nephropathy

Two different clinical syndromes might be observed at presentation in most patients with IgA nephropathy (IgAN): (1) an acute reversible episode of macroscopic hematuria or (2) asymptomatic urinary abnormalities. Patients in these groups differ by genetic markers, the severity of their histologic lesions, and the rate of progression to renal insufficiency. Macroscopic hematuria is more common in children, and its frequency decreases with increasing age. In our experience, most patients presenting in adulthood with macroscopic hematuria did not have proteinuria or microscopic hematuria prior to the episode of macroscopic hematuria, suggesting the onset of disease was indeed in adulthood. IgAN is not a benign disease. About 20% of patients reach end-stage renal failure after 20 years of clinical disease. Features generally associated with a poor prognosis include older age at onset, no history of recurrent macroscopic hematuria, hypertension, and consistent proteinuria. In some studies, men progressed more rapidly than women. Using the regression of Cox in the present study, the magnitude of proteinuria was the only clinical parameter that independently predicted progressive renal impairment.     

53例急性间质性肾炎临床病理分析

目的 分析急性间质性肾炎（acute interstitial nephr-itis,AIN）的病因、临床表现及肾脏病理改变,探讨联合糖皮质激素治疗AIN的效果及预后.方法 回顾性分析我院自2004年1月至2012年12月经肾活检确诊的53例AIN患者的病因、临床表现、肾脏病理改变及联合糖皮质激素治疗的疗效等.结果 肾活检病例7 965例,确诊为AIN的53例（占0.67%）,其中11例（占20.75%）合并基础肾小球疾病.分析53例AIN的病因,药物相关性急性间质性肾炎（DAIN）32例（占60.38%）,其中抗生素导致的AIN 14例,占DAIN的43.75%.感染导致的AIN 10例（占18.87%）.主要的临床表现包括急性肾衰竭49例（占92.45%）、镜下血尿33例（占62.26%）、贫血31例（占58.49%）、蛋白尿31例（占58.49%）、白细胞尿17例（占32.08%）、发热14例（占26.42%）,少尿14例（占26.42%）、皮疹4例（占7.55%）、关节痛3例（占5.66%）、嗜酸细胞增高2例（占3.77%）.病理表现示肾小管间质水肿伴炎症细胞浸润,肾小管损伤轻重不等,合并有基础肾小球疾病者有肾小球损伤.共有16例（占30.19%）患者行血液透析治疗,除1例患者在肾活检结果出来前死亡外,其余52例患者均予糖皮质激素治疗.治疗好转出院时32例（占60.38%）患者肾功能恢复正常,16例行血液透析治疗的患者10例肾功能好转摆脱透析,5例维持性血液透析治疗,1例死亡.结论 AIN常见的病因为药物和感染,主要临床表现为非少尿性急性肾衰竭、血尿、贫血、蛋白尿等,联合糖皮质激素治疗效果较好.     

原发性小血管炎肾损害92例临床分析

目的探讨原发性小血管炎的临床表现和肾脏病理特点。方法回顾性分析92例原发性小血管炎的临床和病理资料。结果①临床呈多器官受累：为不规则发热、乏力、消瘦;浮肿、血尿、蛋白尿、肾功能减退;咳嗽、咯血、胸痛、肺部浸润;鼻塞伴有脓性分泌物;关节肿痛等。②光镜下,肾组织小血管壁节段性纤维素样坏死,多见新月体形成。免疫荧光示肾小球内无或仅有微量免疫球蛋白及补体c3沉积,3例伴有IgA沉积。③使用糖皮质激素联合细胞毒药物（环磷酰胺、来氟米特或霉酚酸酯）,治疗有效率76％。结论原发性小血管炎并不少见,患者以中老年为主,显微镜下型多血管炎居多,抗中性粒细胞胞浆抗体（ANCA）检测有助于早期诊断及判断复发,及时治疗可延缓肾脏病变。     

Hematopoietic Tumors Primarily Presenting in Bone

Hematologic neoplasms that primarily present in bone are rare; this article describes the most common examples of hematologic tumors primarily presenting in bone, including plasma cell myeloma, solitary plasmacytoma of bone, primary non-Hodgkin lymphoma of bone, acute lymphoblastic leukemia/lymphoma, and Langerhans cell histiocytosis. The macroscopic and microscopic features, differential diagnosis, diagnostic workup, and prognosis of all these different entities are discussed, with special emphasis on common differential diagnosis.     

Successful treatment of IgA nephropathy in association with low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue type

Kidney and the urogenital tract are among the various mucosal sites involved in mucosa-associated lymphoid tissue (MALT) lymphoma. We report a case with simultaneous onset of crescentic immunoglobulin (Ig) A nephropathy and gastrointestinal low-grade B-cell lymphoma of the MALT type with kidney infiltration. M-component of IgM lambda was detected in the serum, and the renal biopsy specimen showed monotypic lambda light chain staining in the lymphoma cells but not the glomeruli. The heavy proteinuria and impaired creatinine clearance returned to normal, and microscopic hematuria disappeared 20 months after treatment with chlorambucil as single-agent chemotherapy. This coincided with a complete resolution of the gastric and renal lymphoma infiltration. The close association of both the onset and successful outcome of the two entities thus support their possible causal relationship, and we discuss the possibility of an association of the disturbance of the MALT by the lymphoma cells with the pathogenesis of IgA nephropathy.     

Rapidly progressive glomerulonephritis in a patient with rheumatoid arthritis during treatment with high-dosage D-penicillamine

A patient with advanced rheumatoid arthritis and severe clinical manifestations of rheumatoid vasculitis died of acute renal failure after 30 months of treatment with high-dosage D-penicillamine. She had had no signs of adverse drug reactions until the terminal illness. Although streptococcal pharyngitis was diagnosed late in her disease, penicillamine-induced immune complex glomerular damage is considered more likely than poststreptococcal glomerulonephritis, because her microscopic hematuria preceded diagnosis of pharyngitis. Postmortem examination disclosed findings suggestive of rapidly progressive glomerulonephritis of immune complex pathogenesis. The short period of microscopic hematuria and the rapidity of development of renal failure before death emphasize the need for frequent monitoring of renal function and prompt discontinuation of D-penicillamine treatment upon detection of otherwise unexplained hematuria. There is urgent need for early immunological evaluation, renal biopsy, and vigorous therapeutic measures.     

Acute tubulo-interstitiel nephritis associated with diffuse large B-cell lymphoma presenting as acute renal failure

Renal infiltration in malignant lymphomas may involve the interstitium but rarely causes acute renal failure. In this report, we describe a 59-year-old woman presenting with an acute renal failure due to bilateral diffuse large B-cell non-Hodgkin's infiltration of the kidneys.     

Acute Tubulo-Interstitiel Nephritis Associated with Diffuse Large B-Cell Lymphoma Presenting as Acute Renal Failure

Renal infiltration in malignant lymphomas may involve the interstitium but rarely causes acute renal failure. In this report, we describe a 59-year-old woman presenting with an acute renal failure due to bilateral diffuse large B-cell non-Hodgkin's infiltration of the kidneys.     

Treatment of IgA nephropathy

IgA nephropathy (IgAN) is an important cause of progressive kidney disease with 25-30% of patients developing end-stage renal disease within 20 years of diagnosis. There is still no treatment to modify mesangial IgA deposition and available treatments are those extrapolated from the management of other patterns of chronic glomerulonephritis. There remains no consensus on the use of immunosuppressive agents for treatment of progressive IgAN and this is compounded by the relative lack in IgAN of randomized controlled trials relevant to current clinical practice. Patients with recurrent macroscopic hematuria or isolated microscopic hematuria and proteinuria <1 g/24 h require no specific treatment. Those with nephrotic syndrome and minimal change on renal biopsy should be managed as for minimal change nephropathy. There is no evidence to support the use of corticosteroids for nephrotic IgAN outside this group of patients. Patients presenting with acute renal failure require evaluation to distinguish acute tubular necrosis, which requires supportive therapy only, from crescentic IgAN, for which treatment with cyclophosphamide and corticosteroids in a regimen similar to that for renal small vessel vasculitis is indicated in the absence of significant chronic histologic injury. Patients at greatest risk of progressive renal impairment are those with hypertension, proteinuria >1 g/24 h, and reduced glomerular filtration rate at diagnosis. All such patients should be treated to a blood pressure of 125/75 mm Hg with dual blockade of the renin-angiotensin system with angiotensin-converting enzyme inhibition and angiotensin receptor blockade. At present, there is insufficient evidence for the additional use of immunosuppressive agents, antiplatelet agents, or anticoagulants.     

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.