Efficacy of eculizumab in refractory life‐threatening warm autoimmune hemolytic anemia associated with chronic myelomonocytic leukemia

Gauchy, Anne-Cécile; Hentzien, Maxime; Wynckel, Alain; Marcellus, Victoire de; Rodier, Cyrielle; Delmer, Alain; Quinquenel, Anne

doi:10.1002/ccr3.3250

Cited by 8 publications

(5 citation statements)

References 13 publications

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“…ITP-CMML patients have high relapse rates and respond to all treatments commonly used for idiopathic ITP, except for IVIG. Reports of AIHA and PRCA complicating CMML are much rarer [ 52 , 53 , 66 ], with some concern about possible evolution to acute leukemia after immunosuppressive treatments. Myeloproliferative neoplasms (MPN) may be rarely complicated by AICy, with isolated case reports of AIHA and ITP in Philadelphia (Ph)-negative MPN cases [ 55 , 60 ].…”

Section: Autoimmune Complications In Myeloid Neoplasmsmentioning

confidence: 99%

Autoimmune Complications in Hematologic Neoplasms

Barcellini

Giannotta

Fattizzo

2021

Cancers

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Autoimmune cytopenias (AICy) and autoimmune diseases (AID) can complicate both lymphoid and myeloid neoplasms, and often represent a diagnostic and therapeutic challenge. While autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) are well known, other rarer AICy (autoimmune neutropenia, aplastic anemia, and pure red cell aplasia) and AID (systemic lupus erythematosus, rheumatoid arthritis, vasculitis, thyroiditis, and others) are poorly recognized. This review analyses the available literature of the last 30 years regarding the occurrence of AICy/AID in different onco-hematologic conditions. The latter include chronic lymphocytic leukemia (CLL), lymphomas, multiple myeloma, myelodysplastic syndromes (MDS), chronic myelomonocytic leukemia (CMML), myeloproliferative neoplasms, and acute leukemias. On the whole, AICy are observed in up to 10% of CLL and with higher frequencies in certain subtypes of non-Hodgkin lymphoma, whilst they occur in less than 1% of low-risk MDS and CMML. AID are described in up to 30% of myeloid and lymphoid patients, including immune-mediated hemostatic disorders (acquired hemophilia, thrombotic thrombocytopenic purpura, and anti-phospholipid syndrome) that may be severe and fatal. Additionally, AICy/AID are found in about 10% of patients receiving hematopoietic stem cell transplant or treatment with new checkpoint inhibitors. Besides the diagnostic difficulties, these AICy/AID may complicate the clinical management of already immunocompromised patients.

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Section: Autoimmune Complications In Myeloid Neoplasmsmentioning

confidence: 99%

Autoimmune Complications in Hematologic Neoplasms

Barcellini

Giannotta

Fattizzo

2021

Cancers

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“…In line with the pathophysiology of wAIHA, one may expect complement inhibition to be less significant in this case. Although only licensed for PNH and atypical haemolytic uraemic syndrome, there have been previous case reports on the successful off-licence use of eculizumab in wAIHA 14–19. There is evidence that complement plays a role in wAIHA, as up to 50% of the DATs in wAIHA are positive for complement fragments and some IgG subclasses are able to activate complement via the classical pathway 20 21…”

Section: Discussionmentioning

confidence: 99%

Severe refractory warm autoimmune haemolytic anaemia after the SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA) managed with emergency splenectomy and complement inhibition with eculizumab

Jackson

Harper²,

Webb³

et al. 2022

BMJ Case Rep

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A male in his teens with a history of liver transplant for biliary atresia (aged 2 years) and autoimmune haemolytic anaemia (AIHA, aged 6 years) presented with jaundice, dark urine, fatigue and chest discomfort that began 48 hours after the first dose of SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA). Investigations revealed a warm AIHA picture. Over 4 weeks the patient developed life-threatening anaemia culminating in haemoglobin of 35 g/L (after transfusion), lactate dehydrogenase of 1293 units/L and bilirubin of 228 µmol/L, refractory to standard treatment with corticosteroids and rituximab. An emergency splenectomy was performed that slowed haemolysis but did not completely ameliorate it. Eculizumab, a terminal complement pathway inhibitor, was initiated to arrest intravascular haemolysis and showed a favourable response. AIHA is rare but described after the SARS-CoV-2 Pfizer-BioNTech vaccine. This case highlights the rare complication of AIHA, the use of emergency splenectomy for disease control, and the use of eculizumab.

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“…Eculizumab has shown efficacy in warm antibody AIHA and cold agglutinin disease. 113,114 In post-HSCT AIHA, eculizumab was effective in 1 of the 3 cases reported in the literature. 72 Several other complement inhibitors, such as pegcetacoplan and sutimlimab, are actively being investigated in complement-mediated hemolytic anemia.…”

Section: Treatmentmentioning

confidence: 97%

“…Four of the 10 patients enrolled overcame platelet transfusion refractoriness with one dose of eculizumab [113]. Eculizumab has shown efficacy in warm antibody autoimmune hemolytic anemia and cold agglutinin disease [114,115]. In post-HCT AIHA, eculizumab was effective in one of the three cases reported in literature [73].…”

Section: Treatmentmentioning

confidence: 99%

Post–hematopoietic stem cell transplantation immune-mediated anemia: a literature review and novel therapeutics

et al. 2022

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Anemia following allogeneic hematopoietic stem cell transplantation (HCT) can be immune- or non-immune mediated. Auto- or alloimmunity due to blood groups incompatibility remain an important cause if post-HCT immune-mediated anemia. ABO incompatibility is commonly encountered in HCT and may lead to serious clinical complications including acute hemolysis, pure red cell aplasia, and passenger lymphocyte syndrome. It remains controversial whether ABO incompatibility may affect HCT outcomes, such as relapse, non-relapse mortality, graft-versus-host disease and survival. Non-ABO incompatibility is less frequently encounterd but can have similar complications to ABO incompatibility, causing adverse clinical outcomes. It is crucial to identify the driving etiology of post-HCT anemia in order to prevent and treat this condition. This requires a comprehensive understanding of the mechanism of anemia in blood group incompatible HCT, and the temporal association between HCT and anemia. In this review, we summarized the literature on post-HCT immune-mediated anemia with a focus on ABO and non-ABO blood group incompatibility, described the underlying mechanism of anemia, and outlined preventive and treatment approaches.

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Efficacy of eculizumab in refractory life‐threatening warm autoimmune hemolytic anemia associated with chronic myelomonocytic leukemia

Abstract: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Cited by 8 publications

References 13 publications

Autoimmune Complications in Hematologic Neoplasms

Autoimmune Complications in Hematologic Neoplasms

Severe refractory warm autoimmune haemolytic anaemia after the SARS-CoV-2 Pfizer-BioNTech vaccine (BNT162b2 mRNA) managed with emergency splenectomy and complement inhibition with eculizumab

Post–hematopoietic stem cell transplantation immune-mediated anemia: a literature review and novel therapeutics

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