Abstract
A four-year-old female child who was initially diagnosed with myelodysplastic syndrome was admitted in the bone marrow work up. A fully matched HLA allogeneic bone marrow transplantation was performed. Post-transplant the patient received multiple platelets transfusions to manage extreme thrombocytopenia, but without any positive effects. The platelet count was zero/μl and the life of the patient was seriously in danger. At our center, we define the refractory for a patient as an increase of platelet count of less than 10 k after the transfusion of at least two fully functioned platelet units containing a sufficient number of platelets (>2 × 1011per unite). As a further therapeutic attempt, the young girl received Prednisolone 4 mg/kg/day for 4 days and IVI g 1 g/kg/day for 2 days. However, the medications did not render any response and the count of platelet was still zero and the life of this child was still threatened as she developed rapidly and increasingly petechial exanthema with a massive hemorrhagic in the skin. The HLA-workup showed that the patient developed multiple HLA antibodies against HLA class I antigens (B8. B55. B27. B18. B56. B7. B47. B13. A69. B54. B41. B37. B46. B35. B75. Cw10. B67. B76. Cw1. B53. B64. A68. Cw8. Cw9. B65. Cw14. A68. A34. Cw7. A66. A43). The screening for platelet specific antibodies against human platelet antigens (HPA) was negative. Using our in–house established platelet HLA-matched program, corresponding HLA-matched platelet units donated by 4 selected donors was given over several sessions. The child responded excellent to most of the given HLA-matched platelets. After the second transfusion, the platelets count raised to 51 × 10[c]/μl. Generally, we consider an increase in platelet count of more than 30 k per transfused unit as a sufficient transfusion. After the clinical symptoms and platelet count were stabilized the patient was discharged with a platelet count of 40 × 10[c]/μl. This case highlights the importance and the benefits of platelet matched HLA-program in rescuing the life, decreasing the mortality rate as well as the stay in the hospital and the treatment cost.