Low-dose Rituximab in Adults with Autoimmune Haematological Disorders: A Review
Rituximab, a monoclonal anti-CD20 antibody, has proven to be an excellent therapeutic agent for haematological diseases. Low-dose rituximab (LDR; 100 mg weekly for 4 weeks) has emerged as a promising alternative to the standard dosing (375 mg/m²) for autoimmune haematological disorders, offering comparable efficacy with reduced adverse events and costs. In this review, the authors examine LDR’s role in immune thrombocytopenia, thrombotic thrombocytopenic purpura, and autoimmune haemolytic anaemia (AIHA) in the adult population. For immune thrombocytopenia, studies demonstrate that LDR achieves high overall response rates in both patients with relapsed/refractory disease and those who are newly diagnosed, confirming its non-inferiority to standard dosing. Combination therapies including thrombopoietin agonists further enhance efficacy. Single-dose rituximab shows comparable efficacy to LDR, but reduces indirect costs derived from hospital visits. In thrombotic thrombocytopenic purpura, LDR combined with plasma exchange plus steroids reduces relapse rates, in-hospital stays, and procedures, though prospective data remains limited. For AIHA, LDR combined with steroids or other agents (such as the immunomodulatory drug, bortezomib) induces high response rates, particularly in warm AIHA. Overall, LDR exhibits favourable safety (with minimal infectious complications) and cost-effectiveness across these disorders. While current evidence supports LDR as a front-line or salvage therapy, further research is needed to optimise dosing, identify ideal candidates, and validate combination strategies in randomised trials.
- Abstract
- 10.1182/blood.v122.21.3423.3423
- Nov 15, 2013
- Blood
Thirteen Year Retrospective Analysis Of Adult Patients With Autoimmune Hemolytic Anemia At The Cleveland Clinic: Responses To Therapy
- Abstract
- 10.1182/blood.v124.21.4009.4009
- Dec 6, 2014
- Blood
Long Term Outcomes of Warm and Cold Antibody-Mediated Autoimmune Hemolytic Anemia Treated with Rituximab from a 13 Year Retrospective Cohort
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20
- 10.1097/hs9.0000000000000618
- Jul 13, 2021
- HemaSphere
Autoimmune Hematologic Disorders in Two Patients After mRNA COVID-19 Vaccine
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19
- 10.1016/j.blre.2017.01.001
- Jan 4, 2017
- Blood Reviews
The role of splenectomy in autoimmune hematological disorders: Outdated or still worth considering?
- Abstract
4
- 10.1182/blood-2018-99-113677
- Nov 29, 2018
- Blood
Efficacy of Adjuvant Low Dose Rituximab and Plasma Exchange for Acquired TTP with Severe ADAMTS13 Deficiency — Results of the ART Study
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5
- 10.1002/ajh.22049
- May 31, 2011
- American Journal of Hematology
Positive HIV ELISA test, autoimmune hemolytic anemia, and generalized lymphadenopathy: A unifying diagnosis
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4
- 10.1159/000535927
- Mar 19, 2024
- Transfusion Medicine and Hemotherapy
Introduction: Autoimmune hemolytic anemia (AIHA) occurs in 0.7–5.6% of patients undergoing hematopoietic stem cell transplantation, especially from unrelated or haploidentical donor or after lympho-depleted transplant; the majority of cases are represented by warm AIHA, occurring in a full donor chimerism setting. Standard treatments (corticosteroids, intravenous immunoglobulin, splenectomy, rituximab, cyclophosphamide, plasma exchange) lead to lower response rates than those reported in primary AIHA. Daratumumab use has been proposed in many autoimmune conditions (immune thrombocytopenic purpura, aplastic anemia, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, multiple sclerosis), but only few reports have been published on its use for post-HSCT AIHA, mainly in pediatric patients. Case Presentation: We report the successful use of daratumumab in a 68-year-old patient, suffering from post-HSCT AIHA. Five months after Rh-mismatched HSCT, the patient was diagnosed with anti-D AIHA. After first-line treatment (oral prednisone, rituximab, and plasma exchange) failure, being still transfusion-dependent with symptomatic anemia, he underwent treatment with daratumumab, achieving both clinical and laboratory responses. Discussion: Daratumumab may represent a safe and effective alternative to conventional immunosuppressive therapy, and it deserves further investigations.
- Abstract
1
- 10.1182/blood.v118.21.4752.4752
- Nov 18, 2011
- Blood
Gaps in Access to Patient Care for Patients with Rare Hematological Disorders
- Abstract
1
- 10.1182/blood.v114.22.4027.4027
- Nov 20, 2009
- Blood
Low-Dose Rituximab in Idiopathic Autoimmune Hemolytic Anemia.
- Research Article
209
- 10.1111/j.1365-2141.2008.07054.x
- Mar 18, 2008
- British Journal of Haematology
Current treatment regimens for haematological autoimmune diseases are relatively non-selective and are often associated with considerable toxicity. Recently, it has become clear that B cells play a key role in both the development and perpetuation of autoimmunity, suggesting that B-cell depletion could be a valuable treatment approach for patients with autoimmune diseases. This article reviews data supporting the use of rituximab--an anti-CD20 monoclonal antibody that specifically depletes B cells--in four key autoimmune haematological disorders: idiopathic thrombocytopenic purpura (ITP); autoimmune haemolytic anaemia (AIHA); acquired haemophilia; and thrombotic thrombocytopenic purpura (TTP). Although treatment of ITP, AIHA, acquired haemophilia and TTP with rituximab is still relatively uncommon, results from case series and small phase II trials indicate that patients of all ages can respond to rituximab, irrespective of the number or type of prior treatments that they have received. Moreover, patients with these diseases receiving rituximab experienced predominantly mild adverse events, with only a few serious adverse events reported. These data suggest that rituximab provides an effective and well-tolerated alternative treatment option for patients with ITP, AIHA, acquired haemophilia and TTP, many of whom have limited treatment choices.
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1
- 10.6452/kjms.198906.0350
- Jun 1, 1989
- The Kaohsiung Journal of Medical Sciences
Combined cold- and warm-antibody autoimmune hemolytic anemia--review of the literature and a case report.
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- 10.1182/blood-2025-6652
- Nov 3, 2025
- Blood
Thrombotic thrombocytopenic purpura with features of evans syndrome in a case of lupus vasculitis
- Abstract
5
- 10.1182/blood.v126.23.3457.3457
- Dec 3, 2015
- Blood
Bortezomib Yields High Response Rates in Antibody-Mediated Autoimmune Hematological Diseases Refractory to Conventional Immunosuppression
- Research Article
102
- 10.1111/j.1538-7836.2005.01350.x
- Nov 1, 2005
- Journal of Thrombosis and Haemostasis
Thrombotic thrombocytopenic purpura and its diagnosis
- Supplementary Content
1
- 10.2450/2013.0301-12
- May 17, 2013
- Blood transfusion = Trasfusione del sangue
Haemostasis and immunity: cross-talking systems?