MEDizzy - A 23-year-old man presents with diffuse bruising. He

Feed Library Learning Discuss Leaderboard Journal

Combined hemotology IV

A 23-year-old man presents with diffuse bruising. He otherwise feels well. He takes no medications, does not use dietary supplements, and does not use illicit drugs. His past medical history is negative for any prior illnesses. He is a college student and works as a barista in a coffee shop. A blood count reveals an absolute neutrophil count of 780/µL, hematocrit of 18% and platelet count of 21,000/µL. Bone marrow biopsy reveals hypocellularity with a fatty marrow. Chromosome studies of peripheral blood and bone marrow cells are performed which exclude Fanconi’s anemia and myelodysplastic syndrome. The patient has a fully histocompatible brother. Which of the following is the best therapy?

ExplanationThis patient has aplastic anemia. In the absence of drugs or toxins that cause bone marrow suppression, it is most likely that he has immune-mediated injury. Growth factors are not effective in the setting of a hypoplastic marrow. Transfusion should be avoided unless emergently needed to prevent the development of alloantibodies. Glucocorticoids have no efficacy in aplastic anemia. Immunosuppression with antithymocyte globulin and cyclosporine is a therapy with proven efficacy for this autoimmune disease with a response rate of up to 70%. Relapses are common and myelodysplastic syndrome or leukemia may occur in approximately 15% of treated patients. Immunosuppression is the treatment of choice for patients without suitable bone marrow transplant donors. Bone marrow transplantation is the best current therapy for young patients with matched sibling donors. Allogeneic bone marrow transplants from matched siblings result in long term survival in >80% of patients, with better results in children than adults.