Chilean man, 43 years old, civil engineer from Santiago, resident in Valdivia 6 years ago.
Mild hip fracture was diagnosed at 4 years due to epistaxis and right hemarthosis.
Until 1998 he only received fresh frozen plasma transfusions and cryoprecipitates, then he was treated with high purity lyophilized concentrates and double viral inactivation and antifibrinolithic lithotripsy in 2000 procedures.
In August 2007 she presented epistaxis and ecchymosis without evident trauma.
One month later she consulted with multiple ecchymosis predominantly in the lower limbs.
Blood count showed thrombocytopenia of 23,000/mm3.
Hospitalized myelogram was taken after platelet transfusion and FVIII concentrate, which showed preserved hematopoietic series.
Choledocholithiasis pulses were initiated 125 mg IV and then prednisone 60 mg/day p.o., observing an increase of platelets up to 50,000/mm3 on the fourth day, being discharged.
The laboratory study highlighted: elevated transaminase (GOT 158/GPT 151), TTPA, normal prothrombin time and albumin, positive HCV serology, negative HIV, negative HBAgS.
On the third day after discharge platelets fall to 27,000/mm3.
Prednisone was maintained at 60 mg/day with persistence of thrombopenia, which led to the decision of a new pulse medrol and associated azathioprine 100 mg/day with good response.
During the course of the steroidal treatment, she developed diabetes requiring insulin use and then she became infected with a regimen.
Due to the dependence on corticoids in November 2007, elective surgery was performed on 21 days. Liver biopsy was performed under replacement therapy for deficient factor IIIFV around 40% more antifibrinolytic medication prescribed postoperatively.
Surgery was suspended prednisone and azathioprine 100 mg/day was maintained, achieving a platelet count around 50,000/mm3 at 5 months.
Splenic biopsy showed congestion of red pulp and liver, chronic hepatitis with moderate activity, collapse of the reticular pattern and formation of port-portal bridges.
Hepatocytes with ballooned degeneration, microvesicular steatosis and intracellular cholestasis.
1.
The patient was evaluated by a gastroenterologist who underwent upper endoscopy because of esophageal strictures and congestive gastropathy, HCV viral load (185.120 IU RNA/ml), genotype α INF (1b), and non- Ribavirin therapy was indicated.
In the last 2 years, he had suffered several episodes of hemoptysis/paresis, such as hemoptysis associated with bronchial symptoms, muscle hematomas (antebrazo and thigh), hemartrosis of the right knee and ankle (50,000 mm3).
Current aPTT 62.3 sec and FVIII 7%.
