Minimal change disease associated with newly diagnosed mantle cell lymphoma

Mantle cell lymphoma (MCL) is a rare but aggressive form of non-Hodgkin’s lymphoma. Involvement of the kidney is an infrequent occurrence in patients with MCL and can be the result of direct infiltration or paraneoplastic glomerulopathy. Proliferative glomerulonephritis, membranoproliferative glomerulonephritis and focal segmental glomerulosclerosis have previously been reported in association with MCL. We report a 55-year-old woman who developed nephrotic syndrome due to biopsy proven minimal change disease (MCD) in association with MCL. Proteinuria decreased with prednisolone treatment and MCD remains in remission without any immunosuppressant after the treatment of the underlying MCL.     

Minimal change nephrotic syndrome associated with Hodgkin's lymphoma

This report documents the occurrence of minimal-change nephropathy in four patients with Hodgkin's disease. In two cases the onset of the nephrotic syndrome antedated the recognition of lymphoma by 4 and 7 years respectively, while in the other two the nephrotic syndrome manifested 5.5 years and 13 months respectively after lymphoma. In all cases, the nephrotic syndrome resolved when therapy was effective in treating active Hodgkin's disease. The significance of minimal-change nephropathy in association with Hodgkin's disease is discussed. It may be that abnormalities in T-cell function lead to minimal-change nephropathy in some patients with certain forms of Hodgkin's disease.     

Minimal change glomerulonephritis associated with hydatid disease

A 63-year-old man presented to our department with dyspnea and peripheral edema. A cystic mass in the right upper abdomen, consistent with echinococcal disease was discovered. Proteinuria was also present, and a nephrotic syndrome was diagnosed. The kidney biopsy revealed minimal change glomerulonephritis. Treatment with the antiechinococcal drug albendazole induced complete remission of the nephrotic syndrome, suggesting an etiopathogenic role for a hydatid antigen in the development of an immune-mediated glomerulonephritis.     

Minimal change glomerulopathy associated with nonsteroidal antiinflammatory drugs

Of 55 patients with adult onset minimal change glomerulopathy (MCG) studied at our center between 1971 and 1986, five (9%) had an association with the use of nonsteroidal antiinflammatory drugs (NSAIDs). All of the patients were female, and their mean age at the time of diagnosis was 57.4 +/- 11 (SD) (range 47 to 71) years. They had received NSAIDs for an average of 6.9 +/- 6.4 (range, 3 to 18) months before developing proteinuria. The presenting 24-hour urine protein was 11.3 +/- 10.2 (range, 2.1 to 24) g and all patients were hypoalbuminemic (serum albumin less than 3.5 g/dL) with edema. Two patients presented with acute renal insufficiency (serum creatinine greater than or equal to 1.3 mg/dL). Histologically, three patients had MCG associated with interstitial nephritis, and two had no evidence of interstitial disease. All five patients achieved a complete remission after discontinuing the NSAIDs. The remission occurred within 15 days for 80% of patients. A mean follow-up of 6.4 +/- 3.9 (range, 1.3 to 10.5) years was obtained on the patients. At the time of last follow-up, all patients had remained in complete remission, and all patients had normal renal function. Our experience confirms that MCG may present with or without interstitial nephritis. Once the NSAID is discontinued, a complete remission can be expected.     

Minimal change disease in systemic lupus erythematosus

We report the clinical and pathologic findings in 7 patients with systemic lupus erythematosus and minimal change disease. All 7 patients presented with full nephrotic syndrome including peripheral edema, nephrotic range proteinuria (mean 9.6 g/day), and hypoalbuminemia (mean 1.8 g/dl). In all cases, renal biopsy revealed diffuse foot process effacement in the absence of significant peripheral capillary wall immune deposits, findings consistent with minimal-change disease. In addition, 5 cases displayed mesangial electron-dense deposits, with or without associated mesangial proliferation, consistent with underlying lupus nephritis class II. In all cases, steroid therapy induced a rapid remission of nephrotic syndrome. Minimal change disease is an underrecognized and readily reversible form of nephrotic syndrome in systemic lupus erythematosus. Because it may occur superimposed on mild mesangial proliferative lupus nephritis, this entity may be misinterpreted as an atypical presentation of lupus nephritis class II. Proper recognition of this entity requires careful integration of the renal biopsy immunofluorescence and electron microscopic findings.     

A case of mucosa associated lymphoid tissue (MALT) lymphoma

We examined a 49-year-old man, who was referred to our hospital for resection of abnormal findings on the X-ray. Before hospital admission, he was performed TBLB and aspiration biopsy in other hospital, but the results was not decided diagnosis. Because the possibility of the lung cancer was not completely nagated, we performed the tumor resection (partial pulmonary resection) in use of thoracoscopy. The tumor was diagnosed as a MALT (mucosa associated lymphoid tissue) lymphoma at histologically and immunohistologically (CD 79), and resectable lymph node did not detected malignant cell. He has had no evidence of recurrence for 5 months after the operation.     

Renal lymphoma associated with Castleman's disease.

We report the second case in the literature of a renal lymphoma associated with multicentric Castleman's disease.     

Minimal change nephropathy in a 7-year-old boy with Rosai-Dorfman disease

Rosai-Dorfman (R-D) disease is a benign lympho-histiocytosis of the lymphoid system. Immune derangement due to cytokine over-expression (tumor necrosis factor (TNF), interleukin (IL)-1b and IL-6) has been considered the cause of R-D disease. We present a 7-year-old boy with R-D disease who developed minimal change nephropathy (MCN) during the progression of R-D disease. The patient was resistant to oral prednisolone; and the remission of both R-D disease and MCN was achieved with oral cyclophosphamide (2 mg/kg, 12 weeks). MCN, the most common cause of nephrotic syndrome in childhood, is generally accepted to emerge by way of cytokine derangement. Correlation between R-D disease activity and the development and remission of nephrotic syndrome in our case suggested that nephrotic syndrome had been induced through some R-D disease-related immune mechanisms.     

Gastric MALT lymphoma: a rarity

Association of Helicobacter pylori (H. Pylori) infection with gastric 'Mucosa Associated Lymphoid Tissue' (MALT) lymphomas (MALTomas) is well established. In this study the frequency and characteristics of gastric MALToma over a period of 18 years was evaluated. During this period 24 patients with gastric MALToma were diagnosed; out of them, 13 (54.2%) had active H. Pylori infection. The mean and median age was 49.7 and 53.5 years. The male: female ratio was 17:7. The common presenting complaints were epigastric pain (n=10) and dyspepsia (n=9). Endoscopic findings revealed mild gastric hyperemia (n=16), superficial erosions (n=4) and superficial ulcers (n=4). It was concluded that the prevalence of gastric MALToma was very low in contrast to a high H. pylori gastritis in the Pakistani population.     

Thyroid MALT lymphoma in patients with a compressive goiter

BACKGROUND: Mucosa-associated lymphoid tissue (MALT) lymphomas account for less than 1% of all primary thyroid malignancies. They typically arise as neoplastic transformations within areas of autoimmune thyroiditis. Although they tend to have an indolent course, these lymphomas present a diagnostic challenge requiring a high level of suspicion in patients at increased risk of development. METHODS: We retrospectively reviewed 3 cases of primary MALT thyroid lymphoma discovered in patients with a compressive goiter. This represents the experience at a single institution from 1996 to 2005. Hospital and clinic records were reviewed to identify the workup, treatment, and outcome. RESULTS: One of 3 patients underwent preoperative fine-needle aspiration (FNA). All patients underwent total thyroidectomy to relieve compressive symptoms, and the final pathology revealed MALT lymphoma. Two patients subsequently underwent radiation therapy. All patients are alive without evidence of disease recurrence. CONCLUSIONS: These cases are notable for the unexpected presentation of MALT lymphoma within a compressive goiter.     

Minimal change nephropathy and pemphigus vulgaris associated with penicillamine treatment of rheumatoid arthritis

A 51-year-old man developed pemphigus vulgaris, minimal change nephropathy and acute tubular necrosis after 11 months of treatment with penicillamine for rheumatoid arthritis associated with features of Reiter's syndrome. Penicillamine is a polyclonal B lymphocyte activator in animals and precipitates a variety of autoimmune reactions in man. The pathogenesis of minimal change nephropathy is obscure, but our observations support the possibility that autoimmune mechanisms play a role.     

Systemic sclerosis and gastric MALT lymphoma

CASE-REPORT: A 76-year-old woman was admitted for evaluation of esophagitis complicating limited cutaneous systemic sclerosis. Endoscopy showed persistent grade III esophagitis and an erythematous antral lesion found upon biopsy to be a lymphoma of mucosa-associated lymphoid tissue (MALT). Tests were positive for Helicobacter pylori. Moderate mucosal infiltration by endoscopic ultrasonography was the only finding from staging investigations. Six months after treatment to eradicate H. pylori, a repeat endoscopy showed no evidence of the lesion. DISCUSSION: MALT lymphomas are usually confined to the gastric wall. Gastric MALT lymphomas have a well-established association with H. pylori infection. The clinical manifestations are nonspecific. Endoscopy usually shows a focus of erythema or, less often, erosion. The diagnosis rests on histological examination of a biopsy, which shows infiltration by monoclonal cells whose immunophenotype is characteristic of marginal-zone B cells. Eradication of H. pylori is the mainstay of treatment.     

Minimal change disease with IgM+ immunofluorescence: a subtype of nephrotic syndrome

Immunoglobulin (Ig) M nephropathy is defined by electron-dense mesangial deposits and mesangial IgM visible by immunofluorescence (IF) without other histopathologic and immunofluorescent microscopic abnormalities. Certain patients have only immuno-positive (IgM+) IF. Children presenting with steroid-dependent or steroid-resistant nephrotic syndrome have a high prevalence of IgM+ IF with or without electron-dense deposits. We reviewed the clinical course of children with steroid-dependent or steroid-resistant nephrotic syndrome who underwent renal biopsy at Texas Children‘s Hospital from 1989 to 2006 to further characterize IgM+ IF in children with nephrotic syndrome. Of the 55 children with steroid-resistant or -dependent minimal change disease (MCD), 23 had IgM+ IF. Of these 23 children, 61% had microscopic hematuria at presentation, 48% (11/23) were steroid-dependent, and 48% (11/23) steroid-resistant (one underwent biopsy prior to steroid therapy). We compared the efficacy of adjuvant treatment with cyclophosphamide and cyclosporine: 18% initially treated with cyclophosphamide obtained remission, while 55% had no response; 83% obtained subsequent remission with cyclosporine. Of those initially treated with cyclosporine, 88% obtained complete or partial remission. IgM+ IF may be surrogate marker for the severity of MCD. Based on our results, children with MCD and IgM+ IF have a better response to cyclosporine than cyclophosphamide.     

桥本病合并甲状腺淋巴瘤的诊治体会(附七例报告)

目的　探讨桥本病和甲状腺淋巴瘤的可能关系。方法　收集我院 1993年～ 2 0 0 2年手术治疗桥本病 2 3例的资料 ,从术前诊断、手术方式、病理等方面加以分析。结果　桥本病 2 3例中合并淋巴瘤 7例。结论　对疑似恶变病例应建议做病理检查。桥本病与淋巴瘤相关 ,被认为是癌前病变。有自身免疫疾病者易患淋巴瘤。     

Minimal change nephrotic syndrome associated with immune dysregulation,polyendocrinopathy, enteropathy,X-linked syndrome

Several studies have suggested that T cell-producing permeability factors might lead to proteinuria in minimal change nephrotic syndrome (MCNS). However, it is still unclear whether T-cell abnormalities cause MCNS. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare disorder of the immune regulation system, which leads to severe autoimmune phenomena including autoimmune enteropathy, atopic dermatitis with high levels of serum immunoglobulin E (IgE), type 1 diabetes mellitus (T1DM), and severe infection such as sepsis, which frequently result in death within the first 2 years of life. This disease is caused by mutations in the FOXP3 gene that result in the defective development of regulatory T (Treg) cells. This report describes a 5-year-old boy with IPEX syndrome with a 3 bp deletion in the FOXP3 gene (c.748–750delAAG, p.250K.del) and a paucity of CD4⁺ CD25⁺ FOXP3⁺ T cells. The boy’s condition was complicated by MCNS in addition to many IPEX-related manifestations, such as atopic dermatitis, T1DM, enteropathy, sepsis and hemolytic anemia. This is the first report of IPEX syndrome complicated by MCNS, and our findings imply that Treg cell dysfunction may be crucial for the development of MCNS.