Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024312 (lymphopenia)
 4,859 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present a case of paraneoplastic pemphigus associated with Castleman's disease. Our patient had stomatitis and vulvar erosions followed by a cutaneous polymorphous eruption. Investigations for neoplasia disclosed a Castleman's tumour suggesting the diagnosis of paraneoplastic pemphigus. The diagnosis was supported by immunoblotting using an extract of cow tongue although keratinocytes extracts did not identify relevant target antigens. One year after surgical excision of the tumour the patient remained unwell with persistent buccal erosions and lymphopenia. This case is unusual because of the length of its evolution before the discovery of the Castleman's tumour. It is only the second occasion that the association between paraneoplastic pemphigus and Castleman's tumour has been reported. Our case emphasizes the usefulness of immunoblotting on cow tongue in suspected cases of paraneoplastic pemphigus.
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PMID:Paraneoplastic pemphigus associated with Castleman's tumour. 1084

We report a case of solitary intracranial childhood Castleman's disease (CD) presenting with a sudden onset of partial seizures due to a meningeal and cortical mass lesion. The patient was a previously healthy 8-year-old girl who developed a new onset of simple partial seizures with motor signs. On physical examination, she was neurologically intact. Other findings included low-grade fever, mild microcytic anemia and lymphopenia. Magnetic resonance imaging (MRI) of the brain revealed a left posterior parietal, partly mineralized, contrast-enhancing meningeal mass with cortical invasion and adjacent white matter edema. A complete surgical resection of the dural-based component and a subtotal resection of the adherent, invasive cortical lesion were performed. Pathohistology and flow cytometry of the dural-based lesion disclosed a hyaline-vascular type of CD with striking proliferation of polyclonal B lymphocytes, scattered plasma cells and extensive multifocal cortical mineralization. At the 6-month follow-up, the patient was seizure free on antiepileptics and had returned to normal daily activities. MRI showed no residual lesion, and a workup for systemic disease was negative.
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PMID:Castleman's disease in a child presenting with a partly mineralized solitary meningeal mass. 1268 65