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24 Refractory lupus cytopenias
  1. Eloisa Bonfá
  1. University of São Paulo Medical School, Brazil


The aim of this presentation is to provide concise information regarding diagnosis and management of immune mediated hematological manifestations of systemic lupus erythematosus (SLE), specifically autoimmune hemolytic anemia (AIHA), immune mediated thrombocytopenia and immune mediated leukopenia.1–5

Studies on hematological manifestations of SLE are scarce and with low quality of evidence, therefore most existent guidelines are supported by hematological data from non-SLE patients. Of note, SLE patients have some peculiarities that must be taken into consideration when choosing the best treatment approach for their hematological conditions.

The key points of each cytopenia are:

  1. Autoimmune hemolytic anemia (AHAI) in SLE is usually mediated by warm antibodies (IgG). First-line treatment is glucocorticoid, but there is no consensus to guide second-line therapy. Rituximab and immunosuppressive drugs can be used in refractory cases.

  2. Immune thrombocytopenia is an important prognostic indicator of survival in SLE patients. Treatment is usually indicated for patients with a platelet count <30 × 109/mm3. First-line therapy remains glucocorticoids (dexamethasone is the hematologists 1st choice), but sustained response after glucocorticoid discontinuation in SLE patients is unlikely to happen. Rituximab and immunosuppressive drugs can be used in refractory cases. Thrombopoietin agonists and splenectomy should be avoided due to high risk of infection and thrombosis

  3. Neutropenia may be severe and can lead to morbidity and mortality from increased susceptibility to infection. Severe neutropenia can be successfully treated with granulocyte colony-stimulating factor. Lymphopenia is most often related to drug toxicity and disease activity. Severe lymphopenia may require the use of prophylactic therapy to prevent opportunistic infections. For all cytopenias an extensive evaluation of other causes, particularly drugs and infection should be performed.

Learning Objectives

  • Identify and manage immune mediated anemia in lupus

  • Discuss diagnosis and treatment options for thrombocytopenia in lupus

  • Explain the diagnosis and management of leukopenia in lupus


  1. Velo-García A, Castro SG, Isenberg DA. The diagnosis and management of the haematologic manifestations of lupus. J Autoimmun 2016;74:139–60.

  2. Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis 2019;78(6):736–45.

  3. Pons-Estel BA, Bonfa E, Soriano ER, et al. First Latin American clinical practice guidelines for the treatment of systemic lupus erythematosus: Latin American Group for the Study of Lupus (GLADEL, Grupo Latino Americano de Estudio del Lupus)-Pan-American League of Associations of Rheumatology (PANLAR). Ann Rheum Dis 2018;77(11):1549–57.

  4. Jäger U, Barcellini W, Broome CM, et al. Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting. Blood Rev 2020;41:100648.

  5. Neunert CE, Cooper N. Evidence-based management of immune thrombocytopenia: ASH guideline update. Hematology/the Education Program of the American Society of Hematology American Society of Hematology Education Program2018; 2018(1):568–75.

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