RESUMO
Congenital dyserythropoietic anemias (CDA) is a heterogeneous class of anemia of varying degrees of ineffective erythropoiesis and secondary hemochromatosis. We reported a case of CDA and showed our approach to reaching a diagnosis, highlighting the importance of the typical morphological appearance of bone marrow erythroblasts to reach the diagnosis.
RESUMO
Leukocyte adhesion deficiency type I (LAD-I) is a rare autosomal recessive immunodeficiency syndrome leading to recurrent bacterial and fungal infections. Bone marrow transplantation offers the only cure. In this report, we describe the course and outcome of bone marrow transplant in a 4 month-old female infant with LAD-I at King Hussein Medical Center, Jordan. A successful matched HLA- related allogeneic bone marrow transplantation was performed. Engraftment was demonstrated on the 12th day. The patient developed Grade III grafts versus host disease (GVHD), veno-occlusive disease of the liver, and late onset hemorrhagic cystitis. She recovered with appropriate immune reconstitution.
