SAT0604 Secondary hemophagocytic syndrome: retrospective study according to the underlying disease

Autor: L.M. López, E. Uriarte, M.M. Yagüe, J.R. Furundarena, C.A. Egües Dubuc, J. Cancio, A. De Diego, J. Belzunegui, O. Maíz, J.A. Valero, J. Calvo Alén
Rok vydání: 2018
Předmět:
Zdroj: Saturday, 16 JUNE 2018.
DOI: 10.1136/annrheumdis-2018-eular.6184
Popis: Background Secondary Hemophagocytic Syndrome (SHS) is associated with Hematoncologic (HO), Autoimmune (AI) diseases [such as Systemic Lupus Erythematosus (SLE) or Adult’s Still Disease (ASD)] and in a lower frequency with Infections (Inf.) and Tumours (Tum.). Objectives Describe the demographic and underlying disorders during hospital admission of patients with SHS during the period December/2005-January/2018. Methods A retrospective search of patients diagnosed with SHS and bone marrow biopsy (B.M.O.) with hemophagocytosis was performed. Patients were grouped in: AI, HO, Inf, Tum. and SHS without cause (wc). The variables were: sex, age, diagnosis of the underlying disease, fever, organomegaly, laboratory findings, days of hospital stay, days from admission to B.M.O. and mortality. Results A total of 27 patients were found. table 1 shows the characteristics of the groups. AI xxdiseases found were: 5 SLE, 2 ASD, 1 Rheumatoid Arthritis and 1 Sclerosing Disease Related to IgG4. The HOs were: 4 Myelodysplastic Syndrome, 3 Non-Hogkins Lymphomas, 2 Acute Leukemias, 1 Extranodal Lymphoma of Natural Killer Cells (NKC) and 1 Gastric Plasmocytoma. Inf. were: 1 infection of Pneumocystis in the recent diagnosis of HIV and Gastroenteritis by Campylobacter yeyuni. One Tum. in a patient with Glioblastoma multiforme who received temozolomide. During the follow-up no recurrence of SHS was observed. The sCD25 and cytolytic activity of the NKC were not done. Conclusions Patients with SHS to HO disease had a high mortality and a longer hospital stay compared to the rest of the groups. Practically all patients met all diagnostic criteria, the most frequent were fever, pancytopenia and hyperferritinemia. Disclosure of Interest None declared
Databáze: OpenAIRE