title: Severe infections in thalassaemic patients: prevalence and predisposing factors.
abstract: The incidence of infections among patients with thalassaemia and the role of risk factors for infection are uncertain. We studied the occurrence of infections necessitating hospitalisation in 92 homozygous beta-thalassaemia patients who had been followed longitudinally for decades, and investigated the role of potential risk factors for these infections. Pneumonia accounted for 26% of the infections and fever of unknown origin for 14%. Staphylococcus aureus was the major pathogen possibly related to injections associated with intensive chelation with deferoxamine. There was a significant increase in the rate of infection over time, notably after 15 years. Splenectomy correlated with the incidence of infection (P < 0.001) without being confounded by other variables and with highest frequencies of infections present after 10 years. A direct correlation between iron overload and infection was evident only before the initiation of iron-chelating treatment (P < 0.01). Following initiation of deferoxamine, paradoxically, the infection rate increased (P = 0.046). The combination of splenectomy and deferoxamine treatment was associated with the highest adjusted infection rate. Parathyroid dysfunction and glucose-6-phosphate dehydrogenase deficiency were significantly associated with infection (P = 0.02 and P = 0.04 respectively). The infection rate in thalassaemia is affected mainly by the duration of the disease and is increased by splenectomy and, in the long term, by treatment with deferoxamine.
authors.affiliation: Department of Clinical Microbiology and Infectious Diseases, Hadassah Medical Centre and School of Medicine, The Hebrew University, Ein-Kerem, Jerusalem, Israel. galia.rahav@sheba.health.gov.il
authors.forename: Galia
authors.initials: G
authors.lastname: Rahav
authors.forename: Vania
authors.initials: V
authors.lastname: Volach
authors.forename: Mervyn
authors.initials: M
authors.lastname: Shapiro
authors.forename: Deborah
authors.initials: D
authors.lastname: Rund
authors.forename: Eliezer A
authors.initials: EA
authors.lastname: Rachmilewitz
authors.forename: Ada
authors.initials: A
authors.lastname: Goldfarb
mesh_terms: D000293:Adolescent; D000328:Adult; D001424:Bacterial Infections / Q000209:etiology*; D002648:Child; D003676:Deferoxamine / Q000009:adverse effects / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000097:blood; D006760:Hospitalization; D006801:Humans; D019190:Iron Overload / Q000150:complications; D008137:Longitudinal Studies; D008297:Male; D009894:Opportunistic Infections / Q000209:etiology; D011184:Postoperative Period; D012307:Risk Factors; D017262:Siderophores / Q000009:adverse effects / Q000627:therapeutic use; D013156:Splenectomy / Q000009:adverse effects; D017086:beta-Thalassemia / Q000150:complications* / Q000188:drug therapy / Q000601:surgery
chemical_list: D017262:Siderophores; D005293:Ferritins; D003676:Deferoxamine