Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquiredhematologic condition characterizedwithvariousclinical manifestations.

Aims: We report here the case of PNH revealed by portal thrombosis in a 28 yearold man to emphasize on such a complication and how to manage it.

Methods: Case report.

Results: A 28 year-old male with no medicalhistorywasadmitted to the emergency room for severe abdominal pain evolving for 3 days. On examination he was a febrile with marked pallor, scleralicterus, abdominal distension and diffuse epigastric tenderness associated with splenomegaly. Laboratory investigations showed pancytopenia with severe regenerative normocyticanemia. Serum bilirubin was increased with predominant indirect bilirubin component associated to elevated Lactate dehydrogenase (LDH) level. Direct antiglobulin test was negative. A computerized tomography (CT) scan wasperformedshowing acute right and left portal vein thrombosis. PNH was suspected confirmed with flow cytometry.
Anticoagulation therapy using low molecular weight heparin was started associated with folic acid and blood transfusion.
Treatment with monoclonal antibody Eculizumab could not be used because of its non availability.
A slight improvement was initially noted with relief of his abdominal pain and decrease of LDH but few days later he presented a sub-occlusive syndrome with dyspnea and acute hemolysis. He was admitted than in the intensive care unit where he dead because of massive pulmonary embolism.

Conclusions: PNH is a rare acquired hematologic disorder with various clinical manifestations. Thrombosis can be the first manifestation of this disease. Prognosis has significantly improved owing to Eculizumab.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/portal-vein-thrombosis-revealing-paroxysmal-nocturnal-hemoglobinuria/