Objective: Acquired aplastic anemia is a rare disease characterized by the irreversible loss of bone marrow function and threatens life when not treated. Bone marrow transplantation (BMT) or immunosuppressive agents (IST) are used in its treatment. In this article, we aimed to evaluate our patients with acquired aplastic anemia in epidemiological, etiological, and treatment outcomes.
Case report: Nine patients who were diagnosed with acquired aplastic anemia over ten years were evaluated.
Methodology: The patients admitted to the Istanbul Medical Faculty Pediatric Hematology-Oncology Outpatient Clinic between 2000 and 2010 were diagnosed with acquired aplastic anemia (those who underwent BMT, IST, or both) were evaluated retrospectively on patient files and computer records.
Results: Nine patients were diagnosed with acquired aplastic anemia over ten years. 4 of them were girls, and 5 were boys. The average age was 10 (1–17 years). There was a history of hepatitis in 3 cases and a history of metamizole use in 1 case. As a treatment, six patients were treated with IST, and five patients were treated with BMT. ATG 40mg/kg/day 4 days, cyclosporin 10mg/kg/day (6 months), methylprednisolone 2mg/kg/day (2 months) and G-CSF 5μg/kg (2 months) as immunosuppressive therapy. Response to immunosuppressive therapy was received at an average of 3 months. Two of them were fully responsive. One patient was lost due to septic shock before the IST response was evaluated. BMT was performed in 5 cases, three of them were unresponsive to IST. In the follow-up, two cases are in remission, and three are lost due to sepsis. When evaluating our 5 cases with dead, two of them were very severe aplastic anemia, the symptoms of sepsis were present in their first admission, and they died before the treatment started. Two of them died due to the complication of BMT in the very early period. One case was admitted with perforated appendicitis while in remission after BMT and died due to septic shock.
Conclusion: Two primary treatment modalities are used to treat patients with severe aplastic anemia; IST and BMT. The first option is BMT with the matched sibling donor. If there is no suitable sibling, IST is started first, and a fully compatible donor is searched immediately. If there is no response to IST, an allogenic BMT must be applied in the presence of a suitable donor. Our mortality rate is high compared to the literature because of severe disease presentation; most of them were late admission to the hospital due to low socioeconomic level.
