Narrowing the differential — abnormal labs in a transplant recipient

60-year-old male with history of HCV cirrhosis s/p deceased donor liver transplant with duct-to-duct biliary anastomosis six months ago presents with jaundice, dark urine, pruritus, and pale stools. Exam is notable for icterus. Labs revealed a total bilirubin of 10 mg/dL, direct bilirubin 5 mg/dL, ALP 538 u/L, AST 36 u/L, and ALT 41 u/L. INR is 1.0. Labs from three weeks prior were notable for a normal bilirubin and mildly elevated ALP to 181 u/L which has been rising. Doppler ultrasound reveals coarsened hepatic echotexture, patent vessels, and intrahepatic biliary dilatation to 1cm.

What is the next best step?

Abnormal liver profile and autoimmune liver disease

A 39 year-old female was referred for an abnormal liver profile after she noted scleral icterus, fatigue, and pruritus. She denies alcohol, substance, herbal supplement, or recent antibiotic use. Labs demonstrate an alkaline phosphatase of 462 u/l, AST 277 u/l, ALT 281 u/l, total bilirubin 1.9 mg/dl, direct bilirubin 1.0 mg/dl, INR is normal. Viral serologies were notable for immunity to HAV and HBV and negative HCV antibody. Ultrasonography revealed hepatomegaly and coarsened echotexture. Her primary care provider obtained an ANA in evaluation of a silvery plaque which was elevated to >1:2560 titer. You order several studies which are remarkable for significantly elevated IgG level, positive anti-mitochondrial antibody, and positive anti-smooth muscle antibody.

Hepatology casts a wide NET

A 45-year-old female underwent colonoscopy for iron deficiency and chronic diarrhea with associated flushing and was found to have an erythematous region in her terminal ileum. Biopsies revealed a neuroendocrine tumor. She undergoes an abdominal MRI showing multiple hepatic lesions and a mass near the terminal ileum. She was referred to oncology and started on octreotide depot injections for symptom control. Liver biopsy confirmed metastatic well-differentiated neuroendocrine tumor with synaptophysin and chromogranin stains positive. An octreotide scan demonstrated multiple avid foci in the liver but no extrahepatic lesions. An echocardiogram showed normal LV and RV function without valvulopathies.  She now presents to Hepatology clinic for assessment.

What is the next best step?

A puzzling case of portal hypertension: a collision between hema- and hepatology

A 60-year-old male with a history of HBV exposure, HCV with spontaneous clearance, and myelofibrosis with transfusion-dependent pancytopenia presents with tense ascites and coffee ground emesis. On exam he has massive splenomegaly and a nontender abdomen. Labs are notable for a hemoglobin of 6 gm/dL, platelet count of 12 K/L, INR of 1.2, and normal liver profile. Fluid studies from paracentesis reveal a serum-ascites albumin gradient (SAAG) of 1.8 gm/dL and total protein of 1.7 gm/dL, consistent with portal hypertension. EGD shows multiple columns of non-bleeding large esophageal varices with stigmata of recent bleeding, gastric varices, and portal hypertensive gastropathy. A transjugular liver biopsy with pressure measurement shows a hepatic venous pressure gradient (HVPG) of 10 mmHg. Pathology reveals minimal fibrosis, CD34+ cells within the sinusoids, and reticulin stain shows diffuse nodules surrounded by a compressed reticulin network.

In addition to extramedullary hematopoiesis due to myelofibrosis and increased portal flow, what other entity is contributing to this patient’s portal hypertension?