title: Hemophagocytic syndrome after hematopoietic stem cell transplantation: a prospective observational study.
abstract: The aim of this prospective observational study was to evaluate the incidence of hemophagocytic syndrome (HPS) after hematopoietic stem cell transplantation (HSCT). Between July 2006 and December 2007, all patients who received a HSCT in our institution were included in this study. All the following criteria were needed for the diagnosis of HPS: sustained fever over 7 days; cytopenia (neutropenia and/or thrombocytopenia); presence of more than 3% mature macrophages in bone marrow; hyperferritinaemia (>1,000 ng/mL). During this study, 171 patients received a HSCT (68 allogeneic and 103 autologous). The median age was 32 years (3-62). We observed six cases of HPS (6/68; 8.8%) after allogeneic stem cell transplantation (ASCT): one case of EBV-related HPS, two cases of CMV-related HPS, and three cases with no evidence of bacterial, fungal or viral infections. We observed only one case of CMV-related HPS (1/103; 0.9%) after autologous stem cell transplantation. Four patients died despite aggressive supportive care. To our knowledge, this is the first prospective observational study conducted with the aim to evaluate the incidence of HPS after HSCT. This study provides a relatively high incidence of HPS after ASCT. When sustained fever with progressive cytopenia and hyperferritinaemia are observed, HPS should be suspected, and bone marrow aspirate considered. The rapid diagnosis of HPS and the early initiation of an appropriate treatment are essential for patient management.
authors.affiliation: Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia. aabdelkefi@yahoo.fr.
authors.forename: Abderrahman
authors.initials: A
authors.lastname: Abdelkefi
authors.affiliation: Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
authors.forename: Wassim Ben
authors.initials: WB
authors.lastname: Jamil
authors.affiliation: Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
authors.forename: Lamia
authors.initials: L
authors.lastname: Torjman
authors.affiliation: Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
authors.forename: Saloua
authors.initials: S
authors.lastname: Ladeb
authors.affiliation: Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
authors.forename: Habib
authors.initials: H
authors.lastname: Ksouri
authors.affiliation: Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
authors.forename: Amel
authors.initials: A
authors.lastname: Lakhal
authors.affiliation: Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
authors.forename: Assia Ben
authors.initials: AB
authors.lastname: Hassen
authors.affiliation: Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
authors.forename: Abdeladhim Ben
authors.initials: AB
authors.lastname: Abdeladhim
authors.affiliation: Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
authors.forename: Tarek Ben
authors.initials: TB
authors.lastname: Othman
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005293:Ferritins / Q000097:blood; D005500:Follow-Up Studies; D006086:Graft vs Host Disease / Q000276:immunology / Q000517:prevention & control; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000276:immunology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D014184:Transplantation, Homologous / Q000276:immunology
chemical_list: D005293:Ferritins