Background SLE in our area presents hematologic manifestations in approximately 70%of cases. Some of them are very rare, there are no large series whose analysis could provide relevant information.
Objectives To study the characteristics of patients with Hemophagocytic Syndrome (HS) in a large sample of SLE patients.
Methods SLE patients from RELESSER database were studied. We analysed the SLE manifestations present at 12domains (mucocutaneous, renal, musculoskeletal, constitutional, hematologic, vascular, cardiac, respiratory, neuropsychiatric, gastrointestinal, ophthalmic and serological) before, during and after HS diagnosis and until the last available assessment. We studied activity (SELENA- SLEDAI) and damage (SLICC/ACR DI) indices in each of those moments.We evaluated the treatment received, HS recurrences and the deaths by this entity.
Results 3656 patients from 45Rheumatology Units across Spain were studied.7patients with SLE and HS were identified. 71.4%were women, with a mean age (±SD) at the diagnosis of SH of 35.1 (±17.1) years. In 5of the 7 cases the HS occurred 115.5(±162.9 months after the diagnosis of SLE. In the remaining 2 cases the diagnosis of both entities was simultaneous. The main triggers of HS were infections, followed by SLE activity flares. At the time of HS diagnosis, they had high SLE activity with a mean SLEDAI score of 13.1 (±11.3) and 1.4 (±2.3) SDI scores. Clinically, 100% of the patients presented fever and alterations of the liver profile, 85.8%cytopenias and 71.5% dermatological manifestations. Respiratory manifestations and hemolytic anaemia were present in 57.2% of the cases. Lymph nodes and coagulopathy in 42.9%. Hepatomegaly was detected in 28.6%, as well as neuropsychiatric, digestive and renal manifestations. Splenomegaly was detected in 14.3%. The mean haemoglobin level was 8.6 (±1.1) g/dl, platelets 85 585 (±83,390), ferritin 7410 (±6,470) n/ml and triglycerides 404.7 (±235.6) mg/dl.All patients underwent a bone marrow study.All patients were admitted. They required an average of 2.2 (±1.5) treatment lines, using 2.8 (±1.7) drugs. One patient died during the HS episode and another 2 patients had 2 and 3 recurrences respectively.
The following table shows the characteristics of each patient.
Conclusion HS is a rare life-threatening manifestation (<0.5%). It must be suspected in patients with persistent fever who do not respond to antibiotics, cytopenias and evidence of multiorgan involvement.
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