18416479 Visualization

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