Hemophagocytic syndrome after hematopoietic stem cell transplantation: a prospective observational study.
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.
Abdelkefi
Abderrahman
A
Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia. aabdelkefi@yahoo.fr.
Jamil
Wassim Ben
WB
Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
Torjman
Lamia
L
Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
Ladeb
Saloua
S
Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
Ksouri
Habib
H
Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
Lakhal
Amel
A
Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
Hassen
Assia Ben
AB
Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
Abdeladhim
Abdeladhim Ben
AB
Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
Othman
Tarek Ben
TB
Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia.
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
D005293:Ferritins