Internal medicine
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A 46-year-old man with Klinefelter syndrome (KS) presented with obliterative phlebitis of the lower legs with a deteriorated renal function, and elevated serum alkaline phosphatase and ataxia levels. Examinations demonstrated tubulointerstitial nephritis, obliterative phlebitis and lymphadenopathy with IgG4+ plasma cell infiltrate and sclerosing cholangitis. ⋯ IgG4-related disease (IgG4-RD) with KS was finally diagnosed, and high dose prednisolone with intravenous cyclophosphamide was initiated and thereafter the patient demonstrated a prompt improvement. This is the first known case demonstrating overlapping IgG4-RD with lupus-like serological and neurological features in a patient with KS, thus highlighting the pathogenic role with the genomic background for IgG4-RD and SLE.
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Pulmonary cryptococcosis develops not only in immunocompromised patients but also in immunocompetent patients. However, lymph node involvement is relatively rare in immunocompetent patients. ⋯ The patient was resistant to fluconazole and voriconazole monotherapy; thus, his lung lesions significantly worsened. He eventually responded well to a combination therapy of amphotericin B and flucytosine, which was administered according to the treatment strategy for disseminated diseases.
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Case Reports
A Case of Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Involving the Lung and Brain: A Rare Case Study.
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a primary intestinal T-cell lymphoma and other organ involvement is very rare. A rare case of MEITL involving the lung and brain is herein reported. ⋯ Moreover, the pathological findings of lung specimens taken by bronchoscopy were consistent with those of the small intestine. It is therefore important to include the possibility of MEITL in the differential diagnosis of cancer patients.
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A 61-year-old Japanese man with the pure spinal form of cerebrotendinous xanthomatosis developed dysesthesia of the lower limbs and gait disturbance at 57 years of age. At 61 years old, he was unable to walk without support. A neurological examination showed spasticity and sensory disturbance in the lower limbs. ⋯ R474W, and a novel p. R262C variant. Combination therapy with chenodeoxycholic acid and 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase decreased his serum cholestanol level.
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We encountered a patient with multiple myeloma treated with autologous hematopoietic stem cell transplantation (HSCT) who developed repeated episodes of enteritis but regressed spontaneously. An endoscopic examination revealed no abnormalities, but biopsy specimens showed massive infiltration of CD4+ and Epstein-Barr encoding region (EBER+) abnormal lymphocytes in which a high copy number of Epstein Barr virus (EBV) genomes was detected by quantitative polymerase chain reaction (qPCR). EBV infection was exclusively detected in CD4+ T-cells, leading to a diagnosis of EBV-positive CD4+ T-cell lymphoproliferative disorder (LPD). This case suggests that an immediate biopsy and examinations, including qPCR for EBV DNA, should be considered for patients with recurrent enteritis after autologous HSCT, regardless of endoscopic findings.