Snapshot A 54-year-old man presents to the emergency department smelling of whiskey and rambling about the bad service at "this restaraunt." As the patient is sobering up, blood work is ordered demonstrating a hemoglobin of 7 g/dL and a hematocrit of 21%. Iron studies are performed demonstrating an increase in free iron. On peripheral smear, one sees ringed-sideroblasts. A Prussian blue stain is performed and demonstrates the image to the right. Introduction Defect in erythropoiesis due to disorder in porphyrin pathway iron can enter mitochondria but cannot be incorporated into heme iron now stuck in mitochondria forming a ring around the nucleus (ringed-sideroblasts) less heme results in smaller RBCs (microcytic) see heme metabolism Causes include chronic alcoholism mitochondria toxin heme synthesis disrupted drugs (isoniazid) created B6 deficiency required cofactor of ALA-synthetase genetic XR mutation in ALA-synthetase other drugs chloramphenicol cytotoxic agents vitamin B6 antagonists lead exposure Presentation Anemia Physical exam findings seborrheic dermatitis glossitis angular chelitis peripheral neuropathy confusion seizures Evaluation Labs ↓ MCV and TIBC ↑ serum iron and ferritin Blood smear Pappenheimer bodies Bone marrow stain shows ringed sideroblasts (See above) Prussian blue stain is the most accurate test Treatment Sometimes responsive to pyridoxine (vitamin B6 supplements)