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+A 74-year-old man was referred to our hospital in November 2000 because of liver dysfunction detected during a medical checkup.
+The patient had been diagnosed with nephrotic syndrome in 1995.
+Laboratory examinations showed elevated serum hepatobiliary enzymes and IgM, and the presence of antimitochondrial antibodies.
+Serologic markers for Hepatitis B and C viruses were negative.
+Histopathologic examination of a liver biopsy specimen obtained at laparoscopy revealed non-suppurative destructive cholangitis in the portal area (Figure ​1).
+The diagnosis of PBC (Scheuer stage 3) was confirmed and ursodeoxycholic acid, 900 mg daily, was started.
+In January and June 2002, the patient underwent endoscopic variceal ligation plus endoscopic injection sclerotherapy as well as argon plasma coagulation for worsening esophageal varices.
+In September 2007, the patient was admitted for the treatment of recurrent esophageal varices.
+The platelet count had ranged between 52 × 109/L and 69 × 109/L for several years, but it was noted to decrease from 61 × 109/L in June 2007 to 8 × 109/L just before admission.
+Before the deterioration of thrombocytopenia, the patient had no infectious diseases and received no other medication.
+On admission, the patient had neither purpura nor bleeding episodes.
+Table 1 shows the laboratory data on admission.
+The platelet-associated IgG level was markedly high.
+Bone marrow biopsy revealed normocellular marrow without cellular atypia.
+Ultrasonography and magnetic resonance imaging revealed a cirrhotic liver with splenomegaly, ascites, and gallstones.
+The spleen size had remained unchanged from previous imaging examinations.
+Based on these findings, the association of PBC (decompensated liver cirrhosis) with ITP was diagnosed.
+Human leukocyte antigen (HLA) genotyping determined by polymerase chain reaction-sequencing-based typing or polymerase chain reaction-sequence specific primers (SRL, Inc., Tokyo, Japan) detected A*02010101, B*400201, C*030401, C*07020101, DPB1*0501, DQA1*0103, DQA1*030101, DQB1*030201, DQB1*060101, DRB1*080201, and DRB1*080302.
+The 13C urea breath test for H pylori infection was negative.
+Figure ​2 shows the clinical course.
+Oral prednisolone, 30 mg daily, for ITP was started on day 11, and diuretic therapy combined with albumin infusion for ascites was performed.
+As the platelet count did not increase notably, pulse therapy with intravenous methylprednisolone, 1 g daily, was added on d 22 to 24.
+However, the response was weak and temporary.
+On d 31, mild melena was identified.
+The patient was given a trial of intravenous immune gamma globulin therapy, 25 g daily, on d 32 to 36, combined with a second round of intravenous methylprednisolone pulse therapy on d 32 to 34.
+Because a moderate response was observed, prednisolone was continued, and the platelet count increased slowly.
+The ascites was relatively well controlled with diuretics at discharge.
+Considering the decompensated liver cirrhosis and the platelet count, we determined the patient required careful follow-up of esophageal varices without prophylactic endoscopic therapy.