Step by step clinical diagnosis. A 59 year old man with episodes of jaundice, anemia and repeated transfusions

ABSTRACT

A 59 Businessman presented to private clinic in Mashhad with one-week history of jaundice, anemia, weakness and vertigo. He reported having had dark urine, loss of appetite and tiredness. This man was a proved Case of PNH since 9 years, and he had been on predniselone 30 mg every other day. When the diagnosis of PNH by Ham test and Sugar test was confirmed and a bone marrow aspiration showed no severe abnormality except Hyperplasic B.M. Because of severe hemolytic anemia he received several times blood transfusion. In many developing countries, one of the primary routes of HCV infection is post blood transfusion. In spite of transfusion of screened blood products. In screening the patient for viral Hepatitis, HBsAg and anti HBC markers were negative but HCV antibody by 3ed generation Elisa assay was positive. A quantitative HCV PCR assay was positive with more than 12 million Virus copies per ml, Liver function tests were high [ALT and SLT]. Ultrasonography of liver showed a fatty liver without symptoms of portal Hypertension and ascitis. Pegasys [Peg interferon alpha -2 alpha Roche] with 180 mcgr. per week started but because of declining platelets, Neutrophyls and Hb after 4 injections, the peg interferon dosage was decreased to 135 mcgr per week and after 20 weeks to 90 mcgr per week. In spite of decreasing peg-interferon dosage, the number of platelets and neutrophils were under normal limit. Because of hemolytic side effect of Ribaverine we did not, prescribe this drug. In another HCV-PCR assay, it was strongly positive and liver enzymes were high, and PT was 19 seconds despite of injection Vit K and infusion of [factors 8 and 9] FFP, Prothrombin time did not change. The patient in a trip to Paris in Necker hospital was evaluated again and a transjugular liver biopsy was done. The report of liver pathologist of Necker Hospital by Metavir scoring was A[1] F[2] and by Knodell scoring was 0+1+1+1. So peg interferon discontinued and a complete check up of patient after Peg interferon D.C, evaluated a good condition. Liver enzymes were at normal range, total Bilirubin, less than 3 mg, WBC at a normal range, and no hemorrhagic abnormality. The patient now is in Mashhad with a good condition in spite of HCV-PCR positive. He is on a monthly clinical and laboratory surveillance and probably second liver biopsy should be done after one-year interval