Internal medicine
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This report describes the case of a 29-year-old patient with chronic myeloid leukemia in the blast phase who underwent hematopoietic stem cell transplantation (HSCT) after living-donor liver transplantation. Donor selection, conditioning regimens, and graft-versus-host disease (GVHD) prophylaxis are discussed. ⋯ Maintenance therapy with ponatinib effectively maintained remission. This case highlights the complexities of managing HSCT after solid organ transplantation and suggests strategies for future cases.
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Lysinuric protein intolerance (LPI) is an autosomal recessive disease and pulmonary alveolar proteinosis is a respiratory complication. A Japanese man in his 30s, diagnosed with LPI in infancy, was diagnosed with interstitial lung disease (ILD) similar to fibrotic nonspecific interstitial pneumonia (f-NSIP) based on the findings of a transbronchial lung cryobiopsy (TBLC). ⋯ Corticosteroid pulse therapy was administered twice, but the patient died approximately one month after emergency hospitalization. This is the first report of a patient with LPI diagnosed with ILD similar to f-NSIP using a cryobiopsy and who was treated with nintedanib.
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Case Reports
Erdheim-Chester Disease with Pancreatic Enlargement Observed Using Contrast-enhanced Endoscopic Ultrasonography.
We herein report an unusual case of Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, and emphasize its unique presentation and diagnostic challenges. Our patient exhibited uncommon symptoms and significant organ involvement, particularly pancreatic enlargement that is not typically associated with ECD. ⋯ This is the first report to document pancreatic lesions in patients with ECD evaluated using CEH-EUS. EUS-FNA is valuable for diagnosing rare diseases, including ECD, with diffuse pancreatic enlargement.