MR imaging of bone marrow in glycogen storage disease type IB in children and young adults

OBJECTIVE. Patients with glycogen storage disease type IB have neutropenia and neutrophil dysfunction that predispose them to frequent infections, for which they are given granulocyte colony-stimulating factor. Because neutro penia is a consequence of defects in myeloid maturation, the bone marrow as pirations show hypercellularity due to myeloid hyperplasia. This study eval uated MR imaging of bone marrow in glycogen storage disease type IB with an d without granulocyte colony-stimulating factor. CONCLUSION. As confirmed by the histologic results in bone marrow aspiratio ns, abnormal findings on MR images of bone marrow in patients with glycogen storage disease type IB indicate an increased myelopoietic activity, which is augmented by treatment with granulocyte colony-stimulating factor.