Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paraneoplastic pemphigus (PNP) is an autoantibody-mediated mucocutaneous blistering disease that often occurs with confirmed or occult malignancy. It is often accompanied by extensive eruption of respiratory mucosa and can lead to respiratory failure. The indications for anesthetic management in patients with PNP are rare due to the high risk of generating further eruption of respiratory mucosa during intubation, with potentially disastrous consequences. We report the anesthetic management in a 43-year-old woman with PNP scheduled for diagnostic splenectomy. The surgery was postponed due to persistent hoarseness, diffuse oral-pharyngeal mucositis, and laryngeal erosion. Methylprednisolone 40 mg 3 times daily was prescribed for controlling the mucosa lesions. Two weeks later, after the improvement of mucosal lesions, direct laryngoscopic intubation and the scheduled splenectomy were performed under general anesthesia. Pathology of the spleen showed low-grade B-cell lymphoma had induced PNP. Flexible fiberoptic bronchoscopic examination revealed neither bleeding nor bullae formation in the trachea either immediately after intubation or before extubation. The tracheal mucosa is highly susceptible to mechanical stimulation with direct laryngoscopic intubation. Cuff inflation and positive pressure ventilation, awareness of the disease characteristics of PNP, a sufficient course of steroid therapy beforehand, and adequate mucosal protection are essential to the uneventful anesthetic management of the patient with PNP involving the respiratory tract mucosa.
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PMID:General anesthetic management for diagnostic splenectomy in a patient with paraneoplastic pemphigus. 1451 89

Paraneoplastic pemphigus is a life-threatening autoimmune bullous disease associated with neoplasia, generally of lymphoid origin. Immunosuppressive therapy is often disappointing and there are only a few reports of patients surviving more than 2 years. These cases were generally associated with benign neoplasms. We report here the case of a patient with paraneoplastic pemphigus associated with non-Hodgkin B-cell lymphoma who had a surprisingly good response to systemic corticosteroids and remains free of lesions more than 3 years later despite progression of her neoplasm.
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PMID:Paraneoplastic pemphigus associated with non-Hodgkin B-cell lymphoma and good response to prednisone. 1604 Apr 8

Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome, is a rare disorder associated with underlying neoplasia. The common underlying neoplasms include non-Hodgkins lymphoma, chronic lymphocytic leukemia, and Castlemans disease. Though B-cell lymphoma is the most common underlying malignancy, only one case associated with splenic B-cell lymphoma has been recognized. The prognosis of PNP is very poor, and PNP-associated bronchiolitis obliterans (BO) is not uncommon. Herein, we report a 44-year-old woman who initially presented with multiple oral ulcers, conjunctivitis, and numerous cutaneous blisters. Serial workup established the diagnosis of PNP and revealed an underlying splenic B-cell lymphoma. Although the mucocutaneous lesions gradually healed after splenectomy and chemotherapy, deteriorating respiratory function developed 7 months later with pathologically proven BO. She finally succumbed to respiratory failure 12 months after presentation despite intensive respiratory care.
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PMID:Paraneoplastic Pemphigus and Bronchiolitis Obliterans in a Patient with Splenic B-cell Lymphoma. 1790 66

Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that presents as severe mucosal erosions and variable cutaneous lesions and is primarily associated with hematologically malignant or benign diseases. A 59-year-old Japanese woman presented with oral, ocular and vaginal mucosal erosions and erythema as well as blistering on her trunk and limbs. She developed bronchiolitis obliterans; lymphadenopathy in the cervical, subclavian, para-aortic and intraperitoneal regions; and splenomegaly. PNP with B-cell lymphoma was diagnosed. She was treated with two courses of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) for B-cell lymphoma, rituximab once every 3 weeks for five cycles, steroid pulse therapy, oral prednisolone, cyclosporin and high-dose i.v. immunoglobulin. The B-cell lymphoma was in remission after two courses of R-CHOP treatment. Although her skin erythema and blistering were also improved, the mucosal erosions and bronchiolitis obliterans gradually worsened. The patient died of bronchiolitis obliterans after 6 months of hospitalization. Because a cellular immune response is thought to be involved in the pathogenesis of PNP, cyclosporin therapy is expected to aid in suppressing the cellular response. In this case, however, the patient's mucosal lesions and bronchiolitis obliterans were not improved by regular administration of cyclosporin therapy.
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PMID:Paraneoplastic pemphigus associated with fatal bronchiolitis obliterans and intractable mucosal erosions: Treatment with cyclosporin in addition to steroid, rituximab and intravenous immunoglobulin. 2650 47