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

2018 
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
    • Correction
    • Source
    • Cite
    • Save
    • Machine Reading By IdeaReader
    2
    References
    0
    Citations
    NaN
    KQI
    []