Refractory warm IgM‐mediated autoimmune hemolytic anemia associated with <scp>C</scp>hurg–<scp>S</scp>trauss syndrome responsive to eculizumab and rituximab

Chao, Mark; Hong, Jison; Kunder, Christian A.; Lester, Laura; Schrier, Stanley L.; Majeti, Ravindra

doi:10.1002/ajh.23791

Cited by 11 publications

(7 citation statements)

References 15 publications

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“…Furthermore, routine direct antiglobulin tests detected RBC-bound C3 and IgG in 90% and 24% of cases in their study, respectively. Patients who develop IgM-mediated warm AIHA commonly have an underlying disease that causes several immune abnormalities, and some of the underlying diseases associated with IgM-mediated warm AIHA are Sjögren's syndrome (4), severe combined immunodeficiency (5), idiopathic thrombocytopenic purpura (6), and eosinophilic granulomatosis with polyangiitis (7).…”

Section: Discussionmentioning

confidence: 99%

“…In previous case reports, treatment with steroids and/or transfusion was mainly performed in patients with IgM-mediated warm AIHA. However, in several cases, high-dose intravenous immunoglobulin and plasma exchange therapies were provided to the patients, or rituximab was administered (7,11). Recently, a randomized and double-blind controlled trial evaluating the safety and efficacy of rituximab for warm AIHA in adults (the RAIHA study) showed that the administration of rituximab combined with steroids for newly developed warm AIHA in adults significantly improved the complete remission and mortality rates compared to placebo (12).…”

Section: Discussionmentioning

confidence: 99%

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IgM-mediated Warm Autoimmune Hemolytic Anemia: An Autopsy Report

Ito¹,

Tojo²,

Takashiro³

et al. 2019

Intern. Med.

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A 79-year-old man with Sjögren's syndrome and systemic lupus erythematosus developed acute impaired consciousness and hemolytic anemia. The patient's red blood cells agglutinated spontaneously at 25-37℃. The treatment of red blood cells with 2-mercaptoethanol resulted in the loss of spontaneous agglutination. A diagnosis of IgM-mediated warm autoimmune hemolytic anemia was made. The patient received steroid pulse and plasma exchange therapies. Rituximab was also administered. However, the patient died from multiple organ failure at six days from the symptom onset. The clinical progress of the patient and autopsy findings suggested that complement activation might have been associated with the pathology.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

IgM-mediated Warm Autoimmune Hemolytic Anemia: An Autopsy Report

Ito¹,

Tojo²,

Takashiro³

et al. 2019

Intern. Med.

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“…Data on the use of eculizumab in AIHA is limited. There have been, to date, two case reports on the use of eculizumab in IgM-mediated cold agglutinin disease and one additional case report on the use of anticomplement therapy in IgM-mediated wAIHA [ 7 – 9 ]. Responses varied from 18 to 43 months, and duration of response varied from 1 to 36 months.…”

Section: Discussionmentioning

confidence: 99%

Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy

Caplan

2016

Case Reports in Hematology

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Warm autoimmune hemolytic anemia (wAIHA) is the most common form of AIHA, with corticosteroids in first-line treatment resulting in a 60–80% response rate. Atypical wAIHA and IgG plus complement mediated disease have a higher treatment failure rate and higher recurrence rate. We report a case of severe wAIHA secondary to Waldenström macroglobulinemia with life threatening intravascular hemolysis refractory to prednisone, rituximab, splenectomy, and plasmapheresis. A four-week treatment of eculizumab in this heavily pretreated patient resulted in a sustained increase in hemoglobin and transfusion independence, suggesting a role for complement inhibition in refractory wAIHA.

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“…A single administration of eculizumab 600 mg IV, a humanized monoclonal antibody against terminal complement C5, lead to a 3 g/dl increase in haemoglobin levels, without further need to treatment or transfusion. Subsequently, RTX was successfully administered as maintenance therapy [43]. In 64-year-old female patient with AIHA, secondary diffuse large B-cell non-Hodgkin lymphoma, was given C1 esterase inhibitor (C1-INH) to enhance the efficacy of the RBC transfusion.…”

Section: New Therapiesmentioning

confidence: 99%

The diagnosis and management of the haematologic manifestations of lupus

Velo-García

Castro

Isenberg

2016

Journal of Autoimmunity

122

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Haematological manifestations in systemic lupus erythematosus (SLE) are frequently observed. They are diverse and range from mild to severe. Therefore, different treatment approaches are needed from simply keeping vigilant to significant immunosuppression. Most treatment evidence is based on case-reports or small retrospective studies, as few randomized controlled trials have been performed. The development of biological therapy has opened new possible ways to treat the most severe cases but further clinical trials are necessary. In this review we consider the most common and characteristic haematological manifestations of SLE patients, focusing on their pathogenesis and management.

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Refractory warm IgM‐mediated autoimmune hemolytic anemia associated with Churg–Strauss syndrome responsive to eculizumab and rituximab

Cited by 11 publications

References 15 publications

IgM-mediated Warm Autoimmune Hemolytic Anemia: An Autopsy Report

IgM-mediated Warm Autoimmune Hemolytic Anemia: An Autopsy Report

Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy

The diagnosis and management of the haematologic manifestations of lupus

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