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

We report a case of reversible acute renal failure after cefotaxime treatment in a patient affected by non-Hodgkin lymphoma. Renal biopsy showed necrotizing vasculitis associated with eosinophil-rich interstitial inflammatory infiltrates and patchy infiltrates of CD20+ lymphoid cells. High serum p-ANCA titers were also detected. Drug withdrawal was closely related with recovery of renal function and disappearance of ANCA. Acute renal failure therefore represented a consequence of ANCA-mediated renal vasculitis and acute interstitial nephritis related to cefotaxime treatment.
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PMID:Cephotaxime-associated allergic interstitial nephritis and MPO-ANCA positive vasculitis. 1080 69

Wegener's disease (WD) which is mostly a systemic illness rarely presents as isolated, monoorganic, limited disease. Limited pharyngolaryngeal WD is thus a very rare occurrence. We report the case of a 29 years old man who developed a pharyngolaryngeal tumor with clinically benign evolution, histologically showing granulomatous inflammation and small vessel vasculitis, with no signs of: tuberculosis, sarcoidosis, fungal disease, Hodgkin's disease or foreign body aspiration. p-ANCA's were positive. He was considered a limited form of WD and treated with moderate doses of corticoids and cotrimoxazole. One month later, the lesion diminished significantly. The finding of a pharyngolaryngeal tumor with granulomatous inflammation and vasculitis, in the context of p-ANCA positivity and without any evidence for another systemic granulomatous disease, suggested the diagnosis of limited WD. The response to treatment favoured this presumption. Limited pharyngolaryngeal WD is a rare disease, with a potential for life-threatening (even fatal) complications. It should be recognized early and treated promptly. Remissions can be achieved (even without the use of cyclophosphamide).
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PMID:Limited Wegener's disease presenting as pharyngolaryngeal tumor. 1552 90

Skin vasculitis may be associated with infections and autoimmune diseases. Furthermore, vasculitis may appear as a paraneoplastic symptom. A 19-year-old male patient was examined with swollen joints and papules presented on lower extremitis. Laboratory results showed high erythrocyte sedimentation ratio, positive C reactive protein, increased number of leukocytes and high circulating immune complex level. Histopatology of skin biopsy specimen proved vasculitis. ANCA was not present. No other changes could be observed besides skin involvement. Symptoms disappeared on 0.5-mg/bwkg methylprednisolon therapy. Few weeks later, enlarged cervical lymph nodes developed besides fever and weight loss. Biopsy indicated the presence of mixed cell type Hodgkin lymphoma. Appropriate examinations revealed clinical stage III/B with favorable prognosis (IPS=2). After eight cycles of ABVD therapy complete remission was achieved as confirmed by FDG-PET. As a consequence of the treatment of Hodgkin lymphoma, vasculitis also disappeared. The present case report calls to attention the importance of careful examinations to exclude other diseases, especially malignancies that may remain at the background of cutaneous vasculitis. Treatment of the primary disease also results in the improvement of secondary skin vasculitis.
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PMID:Cutaneous vasculitis as an initiating paraneoplastic symptom in Hodgkin lymphoma. 1809 65

A 74-year-old woman presented with multiple brain infarctions, an inflammatory reaction, and a high serum titer (414 U/ml) of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) with no hematological abnormalities. After the inflammation and ANCA titers were resolved with steroid therapy for suspected microscopic polyangiitis, hemophagocytic syndrome developed. Biopsies revealed non-Hodgkin's intravascular lymphoma (IVL). The flare of IVL with negative serum ANCA suggested that the initial high serum MPO-ANCA had not originated from tumor cells.
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PMID:A patient with intravascular lymphoma presenting with cerebral infarction and a high serum MPO-ANCA level. 2093 Dec 73

A considerable minority of patients with ANCA-associated small-vessel vasculitis are refractory to conventional therapy or experience dose-limiting side effects. Novel therapeutic approaches include rituximab, a genetically engineered chimeric murine-human monoclonal antibody that binds to CD20, which is expressed on human B cells. It was approved in 1997 for the treatment of CD20-positive B-cell non-Hodgkin lymphoma and in 2006 for the treatment of rheumatoid arthritis. The multiple mechanisms proposed for rituximab-mediated B-cell depletion are discussed in this paper. Cumulative data from several open studies on the treatment of microscopic ANCA-associated polyangiitis suggest that in the vast majority of cases rituximab has a beneficial effect. Two randomized controlled trials confirmed these promising results, suggesting that rituximab might be considered as an option for first-line therapy of induction of remission of ANCA-associated vasculitis, and providing an additional tool for treating patients with disease relapse after previous therapy. While rituximab is very effective in the depletion of B cells, current research suggests it could also influence other immune system cells and reestablish immune homeostasis and tolerance. The safety profile of rituximab reveals that most reactions are infusion-related and that the incidence of serious side effects is low. Systemic infection remains a major concern and may result in death. A small number of cases of progressive multifocal leukoencephalopathy reported in patients receiving rituximab in off-label use (albeit none with ANCA-associated vasculitis) highlights the importance of pharmacovigilance.
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PMID:[The role of rituximab in the treatment of ANCA-associated systemic vasculitis]. 2202 61

Rituximab is a monoclonal antibody to the CD20 antigen on B-cells that was initially designed and approved for the treatment of non-Hodgkin's B-cell lymphoma in 1997. In the last 15years, it has emerged as a potent immunosuppressant for many immune-mediated diseases, beginning initially with rheumatoid arthritis, and now extending into several other fields, including clinical nephrology. Based on recent large clinical trials, it is FDA-approved for the treatment of ANCA-associated vasculitis and continues to be studied in off-label usage for many glomerular diseases, including membranous nephropathy, lupus nephritis, and mixed cryoglobulinemia. It has been used as a treatment in nephrotic syndrome in children and adults, including both minimal change disease and focal segmental glomerulosclerosis. Given its efficacy, tolerability and safety profile in comparison to more conventional treatment regimens, RTX is rapidly emerging as a critical treatment modality in glomerular disease.
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PMID:Rituximab-based novel strategies for the treatment of immune-mediated glomerular diseases. 2300 Jun 33

Midline Destructive Lesions (MDL) are well known to cause nasal problems. There is a long differential diagnosis of such lesions. However, in the pediatric population, the 2 main diseases to be aware of are Non-Hodgkin's T-cell lymphoma and granulomatosis with polyangiitis (previously known as Wegener's granulomatosis). The authors present the report of a 15-year-old boy who presented with epiphora, chemosis, and limitation of left abduction. CT scan of his orbits suggested a destructive lesion of the ethmoid sinuses. His laboratory investigations revealed a positive ANCA. The patient underwent endoscopic sinus surgery, and this was characteristic for granulomatosis with polyangiitis. He was treated with systemic steroids and then maintained on cyclophosphamide, which controlled his disease activity. This case highlights the need for ophthalmologists to have a high index of suspicion for MDL and concomitant orbital disease.
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PMID:Orbital presentation of a nasal midline destructive lesion in a young boy. 2479 18