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Background: Hematological manifestations are common in systemic lupus erythematosus (SLE), and are thought to result from immune-mediated peripheral cell destruction or bone marrow failure(1,2).
Objectives: To assess the clinical characteristics and outcomes of severe hematological disease in a large cohort of lupus patients.
Methods: Retrospective analysis of the “Attikon” lupus cohort (over 800 patients total) (3) for the identification of patients with a history of severe hematological manifestation. The latter were defined as: thrombocytopenia with a platelet count <30.000/mm3, hemolytic anemia with an hemoglobin <8 g/dL, neutropenia with less than 500 neutrophils/mm3, history of thrombotic microangiopathy (TMA) or macrophage activation syndrome (MAS). Treatments and long-term outcomes (relapses, mortality) were recorded.
Results: Among 300 patients with hematologic manifestations, 40 patients had severe disease. Most of them were women (75%). Mean age at SLE diagnosis was 41,1 years and mean disease duration at diagnosis of cytopenia was 3.8 years. Hematologic manifestation preceded SLE diagnosis in 13 patients (32,5%).
Autoimmune thrombocytopenia was the most common (57,5%), followed by hemolytic anemia (17.5%), TMA (12.5%) and Evans syndrome (7,5%). All patients received glucocorticoids (GC). Rituximab (15%) and cyclophosphamide (12,5%) were the most frequently used GC-sparing agents during the first episode. Nine patients (22,5%) received intravenous immunoglobulin (IVIg). Relapse occurred in 23 patients (57,5%), most of which (N=17, 73,9%) were treated with GC alone or in combination with IVIg or plasma exchange at initial presentation, without the use of GC-sparing agents. Most of the flares (60,8%) were severe. No deaths were observed.
Conclusion: Hematological disease in SLE is treatment-responsive. GC remain the mainstay of treatment, but the high relapse rates underscore the need for more efficient GC-sparing agents.
REFERENCES:
[1]Fayyaz A, Igoe A, Kurien BT, Danda D, James JA, Stafford HA, Scofield RH. Haematological manifestations of lupus. Lupus Sci Med. 2015 Mar 3;2(1):e000078.
[2]Hepburn AL, Narat S, Mason JC. The management of peripheral blood cytopenias in systemic lupus erythematosus. Rheumatology (Oxford). 2010 Dec;49(12):2243-54
[3]Nikolopoulos D, Kostopoulou M, Pieta A, Karageorgas T, Tseronis D, Chavatza K, Flouda S, Rapsomaniki P, Banos A, Kremasmenou E, Tzavara V, Katsimbri P, Fanouriakis A, Boumpas DT. Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the ‘Attikon’ cohort. Lupus. 2020 Apr;29(5):514-522.
Characteristics of patients with hematological manifestations
| Age at diagnosis, mean (years) | 41,1 |
| Women N(%) | 30(75) |
| Mean disease duration at cytopenia diagnosis (years) | 3,8 |
| Thrombocytopenia, N=23, (mean platelet count,/mm3) | 11682 |
| Hemolytic anemia,N=7 (mean Hb,g/dL) | 6,75 |
| Neutropenia N=1,(mean neutrophil count) | 450 |
| Evans syndrome, N=3 (mean Hb, g, mean platelet count) | 7 /46600 |
| TMA,N=5, (mean platelet count) | 8750 |
| MAS, N=1 | |
| Immunosuppressive treatment at initial presentation | Azathioprine (N,%) 4(10) Corticosteroids (N,%) 40 (100) Cyclophosphamide (N,%) 5(12,5) IgIV (N,%) 9 (22,5) Mycophenolate mofetil (N,%) 2(5) Rituximab (N,%) 6(15) |
| Relapse N(%) | 23(57,5) |
| Severe relapse (N,%) | 14(60,8) |
| Non responder to corticosteroids/IgIV (N,%) | 1(2,5) |
Disclosure of Interests: None declared