Selective engraftment of the granulocyte compartment after allogeneic bone marrow transplantation in a patient with severe aplastic anemia
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Date
1996
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Abstract
Purpose: We present a patient with severe aplastic anemia who had partial engraftment with full chimerism after allogeneic bone marrow transplantation from an HLA identical sibling.
Patients and Methods: A 3-year-old girl with severe aplastic anemia (SAA) received a bone marrow transplantation (BMT) from an HLA identical brother 9 months after her diagnosis. Before BMT she was red blood cell transfusion dependent, had an absolute neutrophil count (ANC) of 1,000-1,500 X 10(9)/l and a platelet count of 15-19,000 X 10(9)/l. She was conditioned with 800 cGy total body irradiation (TBI) and cyclophosphamide and received 3 X 10(8) nucleated cells/kg.
Results: She reached an ANC of 1500 X 10(9)/l on day + 35 but her reticulocyte and platelet counts did not recover. A bone mar row aspirate and biopsy post BMT showed hypoplasia with marked decrease in megakaryocyte and red blood cell precursors. The granulocyte compartment showed a left shift with predominance of promyelocytes and myelocytes. The karyotype showed full chimerism (46,XY) with no 46,XX metaphases.
Conclusion: This case illustrates the possibility of a bone marrow microenvironment defect as the cause of SAA.
Patients and Methods: A 3-year-old girl with severe aplastic anemia (SAA) received a bone marrow transplantation (BMT) from an HLA identical brother 9 months after her diagnosis. Before BMT she was red blood cell transfusion dependent, had an absolute neutrophil count (ANC) of 1,000-1,500 X 10(9)/l and a platelet count of 15-19,000 X 10(9)/l. She was conditioned with 800 cGy total body irradiation (TBI) and cyclophosphamide and received 3 X 10(8) nucleated cells/kg.
Results: She reached an ANC of 1500 X 10(9)/l on day + 35 but her reticulocyte and platelet counts did not recover. A bone mar row aspirate and biopsy post BMT showed hypoplasia with marked decrease in megakaryocyte and red blood cell precursors. The granulocyte compartment showed a left shift with predominance of promyelocytes and myelocytes. The karyotype showed full chimerism (46,XY) with no 46,XX metaphases.
Conclusion: This case illustrates the possibility of a bone marrow microenvironment defect as the cause of SAA.
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aplastic anemia, bone marrow transplantaion, engraftment failure