Transcript Anemia of Chronic Disease

NYU Department of Medicine
Grand Rounds
Clinical Vignette
Elizabeth Haskins, PGY 3
February 25, 2009
Medicine-Surgery Conference
Thursday, February 26th, at 5pm
Hepatocellular Carcinoma:
Current Treatment Options
Speakers:
Dr. Umut Sarpel
Assistant Professor of Surgery; Surgical Oncology
Dr. Thomas Diflo
Associate Professor of Surgery; Transplant Surgery
Farber Auditorium (Bellevue Atrium)
Food will be served
Chief Complaint
The patient is an 18 year old female who
presents to clinic for routine follow-up.
History of Present Illness
At 8 months of age, the patient developed
hypoglycemic coma.
Work-up at the time included a liver biopsy which
made the diagnosis of Glycogen Storage Disease,
type 1a
This was confirmed on mutation analysis.
She was managed with frequent feeds during the day
and nocturnal nasogastric feeds.
At the age of 16, uncooked corn starch replaced her
nocturnal feeds.
She had been doing well and one year prior to the
current visit, she was noted to have normal hematocrit,
iron studies and a normal abdominal ultrasound.
Additional History
Past Medical History:
– Glycogen Storage Disease, type 1a
Past Surgical History:
– None
Social History:
– Denies any toxic habits
Family History:
– No other known genetic disorders in her family
Allergies: No Known Drug Allergies
Medications: none
Physical Exam
General: well appearing female in no
acute distress
Vital Signs: T: 98 BP: 105/65 HR: 70
RR: 12
The remainder of the physical exam
was normal
Laboratory
Hemoglobin: 9.8 g/dL
Hematocrit: 29.4%
MCV: 80.2 fL
RDW: 15.3%
Iron: 20 mcg/dL
TIBC: 301 mcg/dL
Saturation: 6.6%
Ferritin: 67.2 ng/mL
Sedimentation Rate: 100 mm/hr
Working Diagnosis
Microcytic Anemia
– Likely a result of chronic glucose-6phosphatase deficiency
Hospital Course
An Abdominal Ultrasound was obtained,
revealing:
– Single, large hepatic adenoma
Oral iron was initiated, but her hematocrit
remained low.
Hospital Course
Follow-up MRI 3 months later
demonstrated enlargement of the adenoma:
Hospital Course
6 months later, the patient underwent
resection of the adenoma
6 weeks after her surgery, her labs were
as follows:
– Hematocrit 38%
– Iron 100 mcg/dL
– Ferritin 4 ng/mL
– Erythrocyte Sedimentation Rate 10 mm/hr
Final Diagnosis
Hepatic adenoma
Microcytic anemia