case of unexplained jaundice & severe anaemia Posted By Dr.Ashok Chattoraj Date : 28-Aug-07
18 yr. old girl admitted with history of fever, mild rt. hypochonriac pain & progressive jaundice for 2 weeks. No history of vomiting or altered bowel function. Examn. revealed a fully conscious & well oriented girl with severe pallor & jaundice. Pulse- 92/min, B.P.- 110/74mm Hg. P/A- soft, non tender abdomen. Splenomegaly- 10 cm, hepatomegaly- 6 cms. No free fluid inside the abdomen. Chest, CVS- normal. Hb% 6gm. on admission which fell to 2.9gm%. TLC- 10,600/mm3, platelet count- 1,20,000. Serum bilirubin-12mg/dL which progressively rose to 26.6- 45.7- 66.9mg/dL, Direct-21.4, Indirect-45.5 Alk.Phos-192, ALT-122, AST- 102. peripheral smear - spherocytes+. Hb electrphoresis- ruled out Thallasemia. Ultrasound abdomen- gall stones with normal CBD & intra-hepatic ducts. 15 units of packed cells transfused but Hb. remained around 3.9gm%.Decision taken to perform splenectomy to check the anaemia. Post splenectomy & cholecystectomy pt. was conscious & feeling well. However her bilirubin rose to 90mg, PT-INR- 9.1 & her Blood urea to 300mg/dl. Pt. died 4 days following surgery. What is the diagnosis? What would have been the best treatment?