Clinical features and treatment outcome of non-Hodgkin’s lymphoma-associated hemophagocytic syndrome: report of 32 cases
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    Abstract:

    Objective To investigate the clinical features, treatment efficiency and prognosis of non-Hodgkin’s lymphoma-associated hemophagocytic syndrome (LAHS). Methods Clinical data of 32 LAHS patients treated in the Department of Hematology in Ruijin Hospital from January 2005 to December 2013 were retrospectively collected. Kaplan-Meier survival analysis was used to estimate survival functions. Cox regression model was performed to evaluate 14 factors affecting survival. Results Among the 32 patients, 7(22%) were of high risk (4-5 points) according to the international prognostic index (IPI). T-cell or natural killer (NK)/T cell subtypes were the most predominant pathological subtypes. Of the 23 patients who were treated with the regimens containing etoposide, 8 patients (35%) achieved complete remission (CR) and 6 patients (26%) partial remission (PR), with an overall response rate (ORR) of 61%. Of the 9 patients who were treated with the regimens without etoposide, only 1 patient (11%) achieved CR and none PR (0%). The regimens with etoposide were more efficient than those without etoposide (Chi square=4.874, P=0.036). The median overall survival (OS) of the cohort was 122 days (95%CI=79.0165.0). Multivariate analysis showed that CR non-achievement (P=0.001) and subnormal hypofibrinogenemia (<1.5g/L, P=0.031) were the predictive parameters for total survival rate. Conclusion LAHS is usually accompanied with multi-organ dysfunctions, and has rapid progression and poor prognosis. Regimens containing etoposide significantly improve the treatment outcome of those patients.

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  • Online: November 24,2015
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