Fulminant, CMV-associated, haemophagocytic syndrome following unrelated bone marrow transplantation

Abstract

We report a case of haemophagocytic syndrome (HPS) occurring after allogeneic bone marrow transplantation (BMT) for acute promyelocytic leukaemia (APL) in a patient in fourth complete remission (CR). Anti-cytomegalovirus (CMV) antibody (Ab) was negative in this patient before BMT. BMT was performed from an HLA-identical unrelated donor who was positive for CMV Ab. After bone marrow engraftment and haematological recovery, severe acute graft-versus-host disease (GVHD) developed. This patient was treated with methylprednisolone in addition to cyclosporin A (CsA). Acute GVHD showed partial improvement, but CMV antigenaemia was observed. Despite administration of gancyclovir and immunoglobulin, CMV antigenaemia showed no improvement and HPS developed. As no other infections or malignancies were observed, we suspect that CMV infection was the trigger for development of HPS.

Hemophagocytic syndrome: a rare complication of allogeneic nonmyeloablative hematopoietic stem cell transplantation

“…Many of these reports have shown an association with viral infections, such as adenovirus 7 and cytomegalovirus, 8,9 but there has been at least one case report in which a causative viral infection was not documented 10 as in our cases. However, NST is an intensely immunosuppressive procedure, and the possibility that other infectious agents might have caused this complication cannot be ignored.…”

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

“…[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.…”

“…Previous studies of HLH after SCT have not included many patients who received an etoposide-containing condition regimen. 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.…”

Origin of macrophages involved in the development of allogeneic hematopoietic stem cell transplantation-associated hemophagocytic syndrome: observations on a patient with juvenile myelomonocytic leukemia

“…1 Hemophagocytic syndrome (HPS; also known as hemophagocytic lymphohistiocytosis), which is characterized by the inappropriate activation of macrophages due to uncontrolled hypercytokinemia, 2 is known to occur early (during weeks 2-6) after allo-HSCT as a rare complication. [3][4][5][6] Previously, two studies used genotyping or microsatellite polymorphism analysis to show that 98-99% of the whole bone marrow nucleated cells in patients with allo-HSCT-HPS are of donor origin. 4,6 This suggests that the activated macrophages involved in the development of HPS are of donor origin.…”