Pure Red Cell Aplasia in a Patient with Cri-Du-Chat
Jing Ai
Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA
Valeria Visconte
Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA
Heesun J. Rogers
Department of Clinical Pathology, Cleveland Clinic, Cleveland, OH, USA
Mikkael A. Sekeres
Leukemia Program, Department of Hematologic Oncology and Blood Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA
Ramon V. Tiu *
Department of Translational Hematology and Oncology Research, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA and Leukemia Program, Department of Hematologic Oncology and Blood Disorders, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA
*Author to whom correspondence should be addressed.
Abstract
Background: The Cri-Du-Chat syndrome also known as 5p- syndrome is a rare genetic autosomal disorder with the characteristic deletion of the short arm (p) of chromosome 5. To date, hematologic manifestations characteristic of BM failure have not been linked to this syndrome.
Aims: To evaluate the safety and efficacy of cyclosporine to treat pure red cell aplasia in the clinical setting of 5p- syndrome.
Presentation of Case: We report here a patient with 5p- syndrome who subsequently developed idiopathic pure red cell aplasia. He was treated with cyclosporine 1mg/kg twice a day, and achieved a complete remission, with no toxicities.
Conclusion: This case suggests that cyclosporine can be used safely and effectively in such clinical scenario.
Keywords: Cri-Du-chat syndrome, pure red cell aplasia, cyclosporine