title: Severe thalassaemia intermedia with multiple fractures: role of transfusion therapy.
abstract: Thalassaemia intermedia includes thalassaemias with clinical severity intermediate between asymptomatic thalassaemia minor and transfusion dependent thalassaemia major. By definition patients of thalassaemia intermedia maintain a haemoglobin level of 7-10 g/dl and do not, or only occasionally, require blood transfusion. An eight-year-old girl who was a known case of thalassaemia intermedia and had been occasionally transfused presented with fever, pain and swelling over the wrists, ankles and above the right knee joint. Radiographs showed medullary widening, cortical thinning and; multiple, recent and old, partially healed fractures of metadiaphseal regions of long bones. Her fractures have been immobilized by means of back slabs. In view of her recurrent fractures and growth retardation we advised a regular transfusion-chelation regimen to our patient to suppress her ineffective dyserythropoiesis. The treatment is expected to prevent further bone fragility and fractures, as well as improve her life quality.
authors: Department of Pathology, PAF Hospital, Mianwali.
authors: Saqib Qayyum
authors: SQ
authors: Ahmad
authors: Mudassar
authors: M
authors: Iqbal
authors: Madiha Saeed
authors: MS
authors: Wahla
authors: Aimel Munir
authors: AM
authors: Tarrar
mesh_terms: D000900:Anti-Bacterial Agents / Q000008:administration & dosage; D058633:Antipyretics / Q000008:administration & dosage; D001803:Blood Transfusion; D015913:Chelation Therapy; D002648:Child; D005260:Female; D050723:Fractures, Bone / Q000150:complications* / Q000628:therapy; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D012720:Severity of Illness Index; D013789:Thalassemia / Q000097:blood / Q000628:therapy*; D016896:Treatment Outcome; D014815:Vitamins / Q000008:administration & dosage
chemical_list: D000900:Anti-Bacterial Agents; D058633:Antipyretics; D007502:Iron Chelating Agents; D014815:Vitamins