Early onset of hemophagocytic syndrome following allogeneic bone marrow transplantation

We report a 40-year-old man who presented with acute onset of hemophagocyti c syndrome (HPS) after allogeneic bone marrow transplantation (alloBMT) for acute myelogenous leukemia. On day 8 after alloBMT, the patient suddenly m anifested high-grade fever, transfusion-resistant severe anemia, and thromb ocytopenia. Neither veno-occlusive disease nor thrombotic microangiopathy w as documented. The level of ferritin in serum was elevated to 1192 ng/mL. A bone marrow aspiration test on day 16 showed a markedly increased number o f activated macrophages showing massive hemophagocytosis. Serum levels of i nterferon-gamma, soluble interleukin-2 receptor, interleukin-6, tumor necro sis factor-alpha, and macrophage colony-stimulating factor (M-CSF) were ele vated. From these findings, we determined his transfusion-resistant cytopen ias to be attributable to HPS. No viruses (including cytomegalovirus, Epste in-Barr virus, human herpes-virus-6, parvovirus B19, and adenovirus B11) we re detected in serum or urine by polymerase chain reaction amplification. W e speculate that in addition to the administration of M-CSF, hypercytokinem ia during the early phase post-alloBMT might have contributed to the onset of HPS in this patient. Methylprednisolone pulse therapy was very effective for the treatment of the HPS. This case reveals that HPS could develop aft er alloBMT, even when engraftment of hematopoietic cells is not confirmed. Int J Hematol. 2000;72:243-246. (C) 2000 The Japanese Society of Hematology .