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Abstract
Introduction
Hemophagocytic syndrome (HS) is a rare but potentially fatal condition, which results from an excessive and ineffective immune response with continuous activation of mononuclear phagocyte system.
Objective
To make a retrospective assessment of patients with HS reported in the last 8 years in the Hospital de Clínicas to asses prognostic and therapeutic factors.
Material and methods
Sixteen patients with primary (n=1) or secondary (n=15) HS were included. Patients were classified in four categories according to the etiology: lymphoma, infection, combined or unknown. Every patient received specific treatment of the underlying cause, as well as dexamethasone alone or combined with etoposide.
Results
Clinical signs and symptoms of our patients did not differ from the literature. Hemophagocytosis in bone marrow aspirate or biopsy was present in 15 cases (93.75%). The underlying diseases were lymphoma (46.7%; n=7), infection (33.3%; n=5) or combined (13.3%; n=2). The median survival of patients was of 79 days (CI95%: 29-not reached): 31 days for those with lymphoma, 160 days for those with combined etiology and not reached for those with infectious underlying cause (p=0.8). None of the variables assessed at the moment of diagnosis was predictive of survival, whereas hemoglobin decrease (p=0.019), LDH elevation (p=0.029) and ferritin incease (p=0.035) at 15th day correlated significantly with mortality. There was a non statistically significant survival benefit in those who received corticosteroids without etoposide. To achieve a complete response at week four (RC4) was significantly associated with improved survival (p=0.00016). There was a trend towards reduction of mortality in those who received prompt treatment, without reaching statistical signification (p=0.32).
Conclusions
The achievement of RC4 and the values of ferritin, LDH and hemoglobin at day 15th correlated significantly with the risk of death. New studies with more patients are needed to confirm the relevance of these findings.
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