05-06-2008, 09:49 AM
298.
A 31-year-old female is referred to your clinic for an evaluation of anemia. She describes a
2-month history of fatigue. She denies abdominal pain but notes that her abdomen has become
slightly more distended in recent weeks. Past medical history is otherwise unremarkable. The
patient's parents are alive, and she has three healthy siblings. Physical examination is
significant for pale conjunctiva and a palpable spleen 4 cm below the left costal margin.
Hematocrit is 31% and bilirubin is normal. The reticulocyte percentage is low. Haptoglobin and
lactic dehydrogenase (LDH) are normal. A peripheral blood smear shows numerous
teardrop-shaped red cells, nucleated red cells, and occasional myelocytes. A bone marrow
aspirate is unsuccessful, but a biopsy shows a hypercellular marrow with trilineage hyperplasia
and findings consistent with the presumed diagnosis of chronic idiopathic myelofibrosis. You
transfuse her to a hematocrit of 40%. What is the most appropriate next management step?
A. Administer erythropoietin.
B. Follow up in 6 months.
C. Institute combined-modality chemotherapy.
D. Perform HLA matching of her siblings.
E. Perform a splenectomy.
A 31-year-old female is referred to your clinic for an evaluation of anemia. She describes a
2-month history of fatigue. She denies abdominal pain but notes that her abdomen has become
slightly more distended in recent weeks. Past medical history is otherwise unremarkable. The
patient's parents are alive, and she has three healthy siblings. Physical examination is
significant for pale conjunctiva and a palpable spleen 4 cm below the left costal margin.
Hematocrit is 31% and bilirubin is normal. The reticulocyte percentage is low. Haptoglobin and
lactic dehydrogenase (LDH) are normal. A peripheral blood smear shows numerous
teardrop-shaped red cells, nucleated red cells, and occasional myelocytes. A bone marrow
aspirate is unsuccessful, but a biopsy shows a hypercellular marrow with trilineage hyperplasia
and findings consistent with the presumed diagnosis of chronic idiopathic myelofibrosis. You
transfuse her to a hematocrit of 40%. What is the most appropriate next management step?
A. Administer erythropoietin.
B. Follow up in 6 months.
C. Institute combined-modality chemotherapy.
D. Perform HLA matching of her siblings.
E. Perform a splenectomy.