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.

Etoposide-containing conditioning regimen reduces the occurrence of hemophagocytic lymphohistiocytosis after SCT

“…8,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] Indeed, none of the patients in the report by Takagi et al received an etoposide-containing condition regimen. 6 These facts may also serve to support our speculation. Moreover, all three patients who developed complicated HLH despite the use an etoposide-containing condition regimen were alive at the time of analysis.…”

“…[6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] The frequency of HLH after SCT was reported as 4.1% in patients who had undergone autologous and allogeneic SCT 6 and was reported as 16.8% in patients who underwent CBT. 7 In the present study, the cumulative incidence of HLH was 4.3% in all patients and was 5.8% in patients who underwent CBT.…”

“…Although some studies have described HLH occurring after SCT, there are few data regarding risk factors for HLH after SCT. Takagi et al 7 reported that fewer infused CD34 þ cells are a significant risk factor for the development of HLH, and Aldelkefi et al 6 reported that HLH after SCT was observed in many patients with allogeneic SCT and AA. Unfortunately, multicenter analysis did not include data on CD34 þ cells in our study.…”

“…It is characterized by severe clinical manifestations and high mortality. [4][5][6][7] Despite current therapeutic approaches, outcomes remain poor. The present study analyzed HLH after SCT in patients in Hokkaido prefecture in Japan.…”

Virus-Associated Hemophagocytic Syndrome Caused by Pandemic Swine-Origin Influenza A (H1N1) in a Patient After Unrelated Bone Marrow Transplantation

“…Shigeki Saito, 2) Miki Kobayashi, 1,2) Akari Kodaira, 6) Shinichiro Shibata, 6) Isao Oda, 7) Tetsuya Yagi, 3) Hitoshi Kiyoi, 3) Tadashi Matsushita, 4) Tetsuhito Kojima, 5) and Tomoki Naoe 2) Keywords: virus-associated hemophagocytic syndrome, bone marrow transplantation, H1N1 influenza…”