Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Glomerulonephritis rarely appears associated with Hodgkin's disease or non-Hodgkin's lymphoma (NHL). We present a patient with a relapse of a non-Hodgkin's lymphoma which first presented as nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS). This case report discusses the unusual association of non-Hodgkin's lymphoma and focal segmental glomerulosclerosis, as well as the crucial role of positron emission tomography in detecting the relapsing lymphoma.
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PMID:Focal segmental glomerulosclerosis due to a relapsing non-Hodgkin's lymphoma diagnosed by positron emission tomography. 1791 50

A circulating permeability factor is present in some patients with minimal change nephrotic syndrome (MCNS) or focal segmental glomerulosclerosis. Nephrotic syndrome occurs in less than 1% of patients with Hodgkin disease. A substance derived from T lymphocytes may be responsible for proteinuria in these patients, but a circulating permeability factor was not shown. Serum permeability activity (P(alb)) of a young man who presented with MCNS was tested over 11 years. He first was treated with oral prednisone, then cyclosporine (CsA; 4 mg/kg/d). Two years after the initial diagnosis, during CsA-induced remission of nephrotic syndrome, Hodgkin disease was diagnosed and he underwent systemic chemotherapy with doxorubicin hydrochloride, bleomycin, vinblastine, and dacarbazine and radiation therapy. P(alb) was 0.67 before CsA therapy. Although CsA treatment decreased proteinuria to protein less than 100 mg/d, P(alb) did not change. P(alb) decreased to 0.19 within 2 weeks of initiation of chemotherapy for Hodgkin disease and has remained at less than 0.17 for the last 9 years. The patient, in remission from Hodgkin disease, has normal renal function and no detectable proteinuria. This is the first demonstration of the presence of P(alb) in a patient with MCNS and subsequent Hodgkin disease. It also is the first report that aggressive chemotherapy abolishes P(alb). Although the potential causal relationship between nephrotic syndrome and Hodgkin disease in this patient is not clear, the immediate decrease in P(alb) during treatment suggests that aggressive chemotherapy may be an effective treatment for patients with high P(alb) in steroid-resistant MCNS or focal segmental glomerulosclerosis.
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PMID:Serum permeability activity in steroid-resistant minimal change nephrotic syndrome is abolished by treatment of Hodgkin disease. 1795 96

Amyloidosis is a systemic disorder characterized by the extracellular tissue deposition of insoluble, toxic aggregates in bundles of beta-sheet fibrillar proteins. These deposits are typically identified on the bases of their apple-green birrefringence under a polarized light microscope after staining with Congo red, and by the presence of rigid, nonbranching fibrils 8 to 10 nm in diameter on electron microscopy. The type of amyloid fibril unit can be further defined by immunohistology or by immunoelectron microscopy. It has been described at least 25 different human protein precursors of amyloid fibrils, which will describe its corresponding amyloid disease. The most common types of amyloidosis are AL (primary) and AA (secondary) types; the former, is the most frequent and is due to deposition of proteins derived from immunoglobulin light chain fragments, occurring alone or in association with multiple myeloma. The later (AA), is caused by deposition of fibrils composed of fragments of the acute phase reactant serum amyloid A (SAA) and complicates chronic diseases with ongoing or recurring inflammation, namely; rheumatoid arthritis (RA), juvenile chronic polyarthritis, ankylosing spondylitis, familial periodic fever syndromes (Familial Mediterranean Fever), chronic infections and furthermore, some neoplasms (mainly renal cell carcinoma and Hodgkin's disease). Despite its less frequent association, some benign neoplasms can subsequently complicate to AA amyloidosis, therefore, an early diagnose and successful treatment may lead indeed, to regression of the amyloid disease. Herein, we present two cases of AA amyloidosis, both of them caused by 2 different benign neoplasms: 1. A 34 year-old woman, after chronic oral contraceptive use, developed an hepatic adenoma (fig. 1) which finally lead to AA amyloidosis with primary kidney presentation (pure nephrotic syndrome) (table 1). Post-surgical complications yield to acute renal failure from which unfortunately could not be recovered. After being on hemodialysis therapy during 10 months she received a first renal allograft without any complication. 2. A 20 year old woman, was diagnosed of AA amyloidosis after a renal biopsy (fig. 2) because of nephrotic syndrome (table 1). Further investigation lead to the finding of a hialyne-vascular type Castleman's disease located in the retroperitoneum (fig. 2). Despite surgical resection and medical treatment (colchicine) she developed progressive renal failure requiring initialization of hemodialysis therapy. After 6 years being on hemodialysis, she received a first renal allograft which is currently functioning after one year of follow- up. Although other chronic inflammatory diseases complicate more frequently to AA amyloidosis, benign tumors have to be taken into account as a potential ethiological cause for secondary amyloidosis.
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PMID:[Systemic AA amyloidosis induced by benign neoplasms]. 1833 38

Paraneoplastic glomerulopathies are rare manifestations of neoplastic disease to be distinguished from iatrogenic renal damage. Solid tumors are preferentially associated with membranous nephropathy, whereas Hodgkin's lymphomas are associated with minimal change disease. The most common neoplasia associated with paraneoplastic glomerular disease are carcinomas of the lung and of the gastrointestinal tract. Nephrotic syndrome is the most frequent presentation of paraneoplastic glomerulopathy and the most critical glomerular disease regarding prognosis and patient care. Renal biopsy is recommended in patients with glomerular proteinuria or nephrotic syndrome and cancer, depending on life expectancy and therapeutic options. The primary treatment must be directed at the cancer in all cases. Symptomatic treatment of the nephrotic syndrome with diuretics and ACE inhibitors is justified. Prevention of nephrotic syndrome complications, i.e. thromboses and infections, should also be addressed and systematic regular renal follow-up is warranted. All treatments should be regularly reviewed to avoid toxicity, associated renal function loss or low albumin levels for patients receiving albumin-binding drugs. Epidemiologic studies have low evidence-based value. There is no widely accepted experimental model of the association of glomerulopathy and cancer. Thus, epidemiologic and mechanistic studies are needed to determine the true prevalence of paraneoplastic glomerulopathies and investigate new pathophysiologic approaches.
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PMID:Paraneoplastic glomerular diseases and malignancies. 1879 Jun 51

It is currently considered that idiopathic minimal change nephrotic syndrome is an immune-mediated glomerular disease. Its association with classical Hodgkin lymphoma minimal change nephrotic syndrome (cHL-MCNS) suggests a molecular link, which remains to be elucidated. We analyzed the expression of cmaf inducing protein (c-mip) in lymphomatous tissues and kidney biopsy samples of patients with cHL-MCNS (n = 8) and in lymphomatous tissues of patients with isolated cHL (n = 9). Because c-mip affects the regulatory loop involving Fyn, we investigated possible structural defects in this signaling pathway, using laser capture microdissection, reverse transcription polymerase chain reaction, and Western blotting. We found that c-mip was selectively expressed in Hodgkin and Reed-Sternberg (HRS) cells and podocytes of patients with cHL-MCNS but is undetectable in patients with isolated cHL. We demonstrated that c-mip was specifically involved in the negative regulation of early proximal signaling through its interaction with phosphoprotein associated with glycosphingolipid-enriched microdomains and Fyn. We showed that the up-regulation of c-mip in cHL-MCNS was associated with a possible Fyn defect in HRS cells and podocytes. Moreover, we showed that c-mip was up-regulated in Fyn-deficient podocytes. c-mip may be a useful marker of cHL-MCNS and its induction reflects the dysregulation of proximal signaling.
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PMID:Occurrence of minimal change nephrotic syndrome in classical Hodgkin lymphoma is closely related to the induction of c-mip in Hodgkin-Reed Sternberg cells and podocytes. 2020 Mar 55

A 76-year-old man developed minimal-change nephrotic syndrome (NS). After treatment with prednisolone failed to induce sustained remission, cyclosporin was added. The NS improved, and prednisolone and cyclosporin doses were gradually decreased. However, he had repeated relapses of the syndrome, and at each relapse, the drug doses were increased. After 5 years, the patient developed left inguinal lymphadenopathy. The histological diagnosis was mixed cellularity classical Hodgkin lymphoma. He received 6 courses of ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine), and mixed cellularity classical Hodgkin lymphoma and NS both showed complete response. Although the association between Hodgkin lymphoma and minimal-change NS is well known, the pathogenesis is unknown. To the best of our knowledge, this is the first case report of minimal-change NS associated with Hodgkin lymphoma in which Hodgkin-Reed-Sternberg cells were immunostained for tumor necrosis factor-alpha (TNF-alpha) clearly demonstrating that Hodgkin-Reed-Sternberg produced TNF-alpha and in which the plasma level of TNF-alpha normalized after improvement of Hodgkin lymphoma by chemotherapy. The production of TNF-alpha by Hodgkin-Reed-Sternberg cells might play a key role as a potential mediator of minimal-change NS.
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PMID:Minimal-change nephrotic syndrome preceding Hodgkin lymphoma by 5 years with expression of tumor necrosis factor alpha in Hodgkin-Reed-Sternberg cells. 2062 22

An association between nephrotic syndrome and extrarenal neoplasia was described for the first time in 1922. Since then a large number of cases have been published, few of them describing the link between Hodgkin disease (HD) and nephrotic syndrome (NS). It shows that the incidence of nephrotic syndrome in Hodgkin lymphoma is less than 1%. Till date, to the best of author's knowledge, there are about 50 pediatric cases published, no one among Italian children. In the present paper, the authors report 2 cases observed in their department in the 7 yrs period.
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PMID:Hodgkin lymphoma and nephrotic syndrome in childhood. 2087 97

The glomerular lesion of paraneoplastic nephrotic syndrome usually presents as membranous nephropathy, minimal change disease or membranoproliferative glomerulonephritis. We present six cases of paraneoplastic nephrotic syndrome. Four cases were associated with epithelial malignancies (lung, gastric and colon cancer), and two cases with lymphoproliferative malignancies (Hodgkin's lymphoma and chronic lymphocytic leukemia). On the basis of light microscopy, immunofluorescence study and electron microscopy, membranous nephropathy, minimal change disease, and membranoproliferative glomerulopathy were established. We concluded, that the search for malignancy is warranted in patients over the age of 55 presenting with nephrotic syndrome, particularly in cases of membranous nephropathy.
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PMID:Nephrotic syndrome and neoplasia: our experience and review of the literature. 2157 1

Low-grade Extranodal Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, a subtype of non-Hodgkin's Lymphoma, involving the kidney is a rare clinical entity. Association of Minimal change disease nephrotic range proteinuria with Hodgkin's lymphoma is well described, however is extremely uncommon with non-Hodgkin's lymphoma. We describe a patient who presented with nephrotic syndrome and a kidney biopsy revealed marginal zone lymphoma and diffuse epithelial foot process effacement. He showed dramatic response to a combination therapy with cyclophosphamide, corticosteroids, and Rituximab.
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PMID:Minimal change disease associated with MALT lymphoma. 2159 66

Minimal change disease is the most common nephrotic syndrome in children. Although the etiology of minimal change disease remains to be elucidated, it has been postulated that it is the result of a circulating T-cell factor that causes podocyte cytoskeleton disorganization leading to increased glomerular capillary permeability and/or changes in glomerular basement membrane heparan sulfate glycosaminoglycans resulting in proteinuria. Minimal change disease has been associated with allergies and Hodgkin disease. Consistent with these associations, a role for interleukin-13 with minimal change disease has been proposed. Furthermore, studies evaluating podocytes also have evolved. Recently, increased expression of CD80 (also termed B7-1) on podocytes was identified as a mechanism for proteinuria. CD80 is inhibited by binding to CTLA-4, which is expressed on regulatory T cells. Recently, we showed that urinary CD80 is increased in minimal change disease patients and limited studies have suggested that it is not commonly present in the urine of patients with other glomerular diseases. Interleukin-13 or microbial products via Toll-like receptors could be factors that induce CD80 expression on podocytes. CTLA-4 appears to regulate CD80 expression in podocytes, and to be altered in minimal change disease patients. These findings lead us to suggest that proteinuria in minimal change disease is caused by persistent CD80 expression in podocytes, possibly initiated by stimulation of these cells by antigens or cytokines.
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PMID:Minimal change disease: a CD80 podocytopathy? 2183 64


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