Ascites and sinusoidal thrombosis in paroxysmal nocturnal hemoglobinuria: diagnostic and therapeutic challenges
DOI:
https://doi.org/10.32818/reccmi.a10n2a18Keywords:
paroxysmal nocturnal hemoglobinuria, sinusoidal thrombosis, ascites, anticoagulationAbstract
Paroxysmal nocturnal hemoglobinuria (PNH) is a non-malignant clonal disorder of the hematopoietic stem cell, caused by an anchoring deficiency of complement regulatory proteins, which increases the risk of haemolysis, thrombosis and renal dysfunction. We present the case of a young man with a history of unrelated chronic anaemia, who presented with ascites secondary to sinusoidal thrombosis, diagnosed by liver biopsy. Subsequently, PNH subtype 2 was confirmed by flow cytometry. This case highlights the importance of considering PNH in the face of unexplained ascites, pancytopenia, and thrombosis in atypical locations, even with normal imaging tests.
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Copyright (c) 2025 Ximena Andrea Cano-Narváez, María Dolz-Rubert, Claudia Tironi-Martínez, Beatriz Tena-Rausell, Sergio Fabra-Juana
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