A Clinical Analysis of 52 Adult Patients With Hemophagocytic Syndrome: The Prognostic Significance of the Underlying Diseases

Abstract

We retrospectively analyzed 52 adult patients with hemophagocytic syndrome (HPS). The underlying diseases were heterogeneous, including malignant lymphoma (lymphoma-associated hemophagocytic syndrome [LAHS]) in 26 patients, systemic lupus erythematosus in 3 patients, viral infections in 7 patients, and bacteria] or fungal infections in 6 patients. More than 83% of patients received prednisolone as an initial treatment. Multiple-agent chemotherapies (cyclophosphamide, doxorubicin, and vincristine) were administered to 96% of LAHS patients after a histopathological diagnosis of lymphoma. HPSs were controllable and remissions were achieved except for those patients with LAHS, fulminant Epstein-Barr virus-associated HPS, and an immunosuppressive state. Twenty-one (81%) of the LAHS patients had uncontrollable HPS and died of multiple organ failure and disseminated intravascular coagulation. The median survival time of LAHS patients was 83 days. In contrast, 3 (12%) of the other HPS patients died of multiple organ failure within 44 days.The clinical manifestations and the laboratory findings of LAHS and the other HPSs were too variable to establish the prognosis based only on the findings at the onset of HPS. The prognostic factors of adult HPS were found to be the underlying diseases, notably malignant lymphoma and infections, accompanied by the immunosuppressive state.

Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis

“…The study results agree with those of Parikh et al's [19] finding that malignancy-related HLH patients had poor prognosis, with a median OS of 1.4 months. In addition, Takahashi et al [31] found that patients with lymphoma-associated hemophagocytic syndrome had worse survival than patients with non-lymphoma-associated hemophagocytic syndrome. Recently, Riviere et al [26] reported that patients with hematologic malignancies-associated HLH had a poorer early outcome (survival at 1 month) compared with HLH patients with underlying infections.…”

Pregnancy Induced Haemophagocytic Syndrome

“…Clinical severity ranges from complete recovery to rapid deterioration and death. HPS may be primary, as observed in familial HPS, X-linked lymphoproliferative syndrome and Chediak-Higashi syndrome or it may be secondary to malignancies (particularly lymphoma), infections (mainly EBV), autoimmune diseases or drugs [2]. Few reports of pregnancy induced HPS are available in the retrieved literature [3,4].…”

“…HPS may be primary related to genetic mutations or secondary to various infections, malignancies and rheumatic conditions [2]. These antigenic stimuli cause uncontrolled immune reaction with a cytokine storm and activated macrophages that can be fatal.…”

T-Cell/Histiocyte-Rich Large B-Cell Lymphoma Masquerading as Autoimmune Hepatitis with Clinical Features of Hemophagocytic Lymphohistiocytosis

“…The correct diagnosis of T/HRBCL subsequently was established by bone marrow biopsy after the patient presented with clinical features of HLH, the diagnostic criteria for which include at least five of the following eight findings: fever >38.5°C, splenomegaly, peripheral blood cytopenia, hypertriglyceridemia and/or hypofibrinogenemia, hemophagocytosis (in bone marrow, spleen, lymph node, or liver), low or absent NK cell activity, ferritin >500 ng/mL, and elevated sIL-2R (also termed soluble CD25) [1]. Hematological malignancies, particularly non-Hodgkin lymphoma, are the most common underlying disease in adults who present with the hemophagocytic syndrome [2,3]. Moreover, the patient manifested a level of sIL-2R >5000 U/ml and an sIL-2R/ferritin ratio (>2.0) that have been strongly associated with lymphoma [4].…”