Cases from the Osler Medical Service at Johns Hopkins University. Zieve syndrome

Presenting features

A 47-year-old African American man was admitted to the Osler Medical Service with a chief complaint of light-headedness. He was a heavy drinker and consumed 2 pints of fortified wine every day. On the day of admission, he had been at home consuming alcohol when he stood up, became lightheaded, and immediately had to sit down. He denied any loss of consciousness, dyspnea, hematemesis, coffee-ground emesis, lower abdominal pain, bright red blood per rectum, or melena, but he recalled a burning epigastric discomfort. The patient's medical history was notable only for a 20-year history of hypertension and recently diagnosed type 2 diabetes mellitus. He was not taking any medications. He had a normal hematocrit of 43% 6 months prior to admission.Physical examination showed a supine heart rate of 115 beats per minute and blood pressure of 165/90 mm Hg, without orthostatic changes. There was scleral icterus and a jaundiced palate, but no other stigmata of end-stage liver disease. His abdominal examination was unremarkable; there were no masses, tenderness, or hepatosplenomegaly. Rectal examination revealed guaiac-negative stool. The chest radiograph and electrocardiogram were unremarkable.On admission, the laboratory examination was notable for the following values: hematocrit, 22.8% with a mean corpuscular volume of 86.7 fL and a red cell distribution width of 25.7%; absolute reticulocyte count, 177,500/mm³; total bilirubin, 4.2 mg/dL; direct bilirubin, 2.3 mg/dL; albumin, 3.3 g/dL; lactate dehydrogenase, 481 U/L; aspartate aminotransferase, 106 U/L; and alanine aminotransferase, 44 U/L. The prothrombin and activated partial prothrombin times were normal. There was no evidence of iron, vitamin B₁₂, or folate deficiency. The patient's haptoglobin level was severely depressed (<6 mg/dL) with a negative direct Coomb test and normal glucose-6-phosphate dehydrogenase activity. A peripheral blood smear was compatible with hemolysis and demonstrated normocytic, normochromic erythrocytes with moderate poikilocytosis, as well as rare spherocytes and target cells Figure 1).On the second day of hospitalization, a lipid panel revealed hypercholesterolemia with a total cholesterol level of 300 mg/dL. Due to the patient's complaint of burning epigastric pain and long history of alcohol consumption, esophagogastroduodenoscopy was performed and revealed grade 1 nonbleeding esophageal varices.What is the diagnosis?