Prognostic factors of early death in a cohort of 162 adult haemophagocytic syndrome: impact of triggering disease and early treatment with etoposide

Abstract

Reactive haemophagocytic syndrome is a life-threatening disease for which factors influencing the outcome remain unclear. We sought to identify determinants of early mortality in patients with reactive haemophagocytic syndrome by conducting a non-interventional retrospective multicentre study in three tertiary care teaching hospitals over a 6-year period. The medical files of 162 patients fulfilling our diagnostic criteria of haemophagocytic syndrome were reviewed. Patients were classified according to 30-d outcome following diagnosis. Thirty-three patients (20·4%) died within 30 d. Clinical features at diagnosis associated with 30-d death in univariate analysis were older age (P = 0·004), underlying lymphoma (P = 0·04), lower platelet count (P = 0·001) and elevated aspartate aminotransferase and lactate dehydrogenase (P = 0·04 both). The use of etoposide as a first-line treatment tended to be associated with a better outcome (P = 0·079). In multivariate analyses, increasing age, decreasing platelet count, underlying lymphoma and no etoposide in the management were associated with a poorer prognosis (P = 0·03, 0·01, 0·003 and 0·04, respectively). These prognostic factors could help to identify those patients more severely affected by reactive haemophagocytic syndrome, who should benefit from aggressive supportive care, combined with specific treatment of the precipitating factor.

How I treat hemophagocytic lymphohistiocytosis in the adult patient

“…Case series of adults treated with a variety of regimens report a 30-day mortality of 20% to 44% and overall mortality of 50% to 75%. [25][26][27]31,74,75 Patients with HLH associated with malignancy suffer a worse prognosis.…”

Plasma Levels of Presepsin (Soluble CD14-subtype) as a Novel Prognostic Marker for Hemophagocytic Syndrome in Hematological Malignancies

“…In refractory cases, immunosuppressive agents such as steroids and calcineurin inhibitors (CI) and etoposide are used according to the HLH-2004 protocol. Despite this treatment, the mortality is reportedly high at 20-75% (7)(8)(9). Several previous studies showed that the following clinical and laboratory findings were risk factors associated with a very poor prognosis: underlying malignant lymphoma, increasing age, a low platelet count, management without etoposide (8), low levels of albumin (9) and low levels of fibrinogen (10).…”

Proliferation through activation: hemophagocytic lymphohistiocytosis in hematologic malignancy

“…Age .60 years was found to have a poorer overall prognosis. 81 One study found that male sex lead to poorer outcomes, although this has not …”

“…81, The treatment of HLH should be aimed at suppressing the immune system and treating the underlying disorder. The first international treatment protocol for HLH included an 8-week induction therapy with etoposide, high-dose dexamethasone, and intrathecal methotrexate for patients with central nervous system involvement followed by maintenance with cyclosporine; however, this regimen is used mainly in FHL.…”