PS1080 LYMPHOMA ASSOCIATED HEMOPHAGOCYTIC SYNDROME: A SINGLE‐CENTER EXPERIENCE

Abstract

Background:Aproximately 1% of the patients with haematological malignancies will develope an hemophagocytic syndrome. Some types of lymphoma are associated with higher risks of developing hemophagocytic syndrome. It is described a high rate of lymphoma associated hemophagocytic syndrome (LAHS) related‐mortality. Only few studies have addressed LAHS.Aims:To describe the clinical and epidemiological features and the prognosis of LAHS in a tertiary hospital from January 2008 to December 2018.Methods:We performed a ambispective observational study of the cases of hemophagocytic lymphohistiocytosis (HLH) diagnosed by bone marrow aspirate. The diagnosis was made according to the diagnostic criteria of the International Histiocyte Society (IHS‐2004). Lymphoma classification followed the World Health Organization (WHO) 2017 system. Epidemiological and clinical features of the patients diagnosed of LAHS were analysed. Statistics performed with SPSS 17.0.imageResults:48 patients were diagnosed with HLH in our center in this period. 16 cases (37%) were associated to lymphoma. The median age for patients diagnosed with LAHS was 63 (range: 13–80). The most common histological type was diffuse large B cell lymphoma, which occurred in 7 of 16 patients (44 %). Peripheral T cell lymphoma and Hodgkin lymphoma occurred both in 3 of 16 patients. 7 patients (44%) had other known risk factor for LAHS: viral infection, autoimmune disease and immunosuppression. The clinical and analytical features are summarized in Table 1. The histopathological analysis of the bone marrow did not identify hemophagocytosis in any cases.Most of the patients diagnosed with LAHS (9 of 14) were treated with the first‐line chemotherapy regimen for lymphoma. 3 patients were treated following the HLH‐2004 protocol. 2 patients died before receiving any treatment. The median overall survival time was 88 days (range: 0 days‐6 years). The three months mortality rate was 50%. 4 patients are still alive with 2, 3, 35 and 71 months after the diagnosis of LAHS (Fig 1).imageSummary/Conclusion:The most common associated lymphoma was diffuse large B cell lymphoma. 44% of the patients had other known risk factor for LAHS. The bone marrow aspirate is superior to the histopathological examination for the diagnosis of LAHS. The three months mortality rate was 50%. Further researches are needed to gain better understanding about this entity.