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Transplant Proc ; 49(7): 1596-1603, 2017 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-28651806

RESUMEN

BACKGROUND: Thrombotic microangiopathy (TMA) pathogenesis after living donor liver transplantation (LDLT) is thought to be caused by release of unusually large von Willebrand factor multimers (UL-vWFMs) resulting from sinusoidal endothelial cell damage and induction of platelet adhesion and aggregation. A decrease in a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs-13 (ADAMTS-13) that cleave UL-vWFMs might cause excessive UL-vWFMs activity and result in platelet thrombus formation. However, this phenomenon has not undergone a full pathologic assessment. PROCEDURES: A 60-year-old man was diagnosed with hepatitis C-related end-stage cirrhosis. His son was the donor, and he underwent LDLT. On postoperative day 44, his laboratory findings met most TMA diagnostic criteria, and he was diagnosed with TMA-like disorder (TMALD). Localization of CD42b as a platelet marker, vWF, and ADAMTS-13 in allograft tissue of this patient were evaluated using immunohistochemistry. RESULTS: CD42b expression was observed as platelet aggregates attached to hepatocytes or within the hepatocyte cytoplasm, a morphology called extravasated platelet aggregation (EPA). vWF expression was observed mainly as deposited compact clusters, and ADAMTS-13 expression resembled distinct dots throughout the liver tissue. CONCLUSION: These findings suggest that EPA indicated sinusoidal endothelial cell damage followed by detachment, and vWF deposition resulted from UL-vWFM oversynthesis. ADAMTS-13 might be consumed in the allograft tissue to cleave UL-vWFMs, but ADAMTS-13 levels might be insufficient to cleave all the deposited UL-vWFMs. We present the case of an LDLT recipient diagnosed with TMALD using blood tests, which showed the presence of TMA pathogenesis in the allograft.


Asunto(s)
Proteína ADAMTS13/metabolismo , Trasplante de Hígado/efectos adversos , Complicaciones Posoperatorias/metabolismo , Microangiopatías Trombóticas/metabolismo , Factor de von Willebrand/metabolismo , Aloinjertos/metabolismo , Biomarcadores/análisis , Plaquetas , Humanos , Hígado/metabolismo , Trasplante de Hígado/métodos , Donadores Vivos , Masculino , Persona de Mediana Edad , Agregación Plaquetaria , Complicaciones Posoperatorias/etiología , Microangiopatías Trombóticas/etiología
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