Practical issues in using eculizumab for children with atypical haemolytic uraemic syndrome in the acute phase: A review of four patients

Terano, Chikako; Ishikura, Kenji; Hamada, Riku; Yoshida, Yasuhiro; Wataru, Kubota; Okuda, Yusuke; Shinozuka, Shunsuke; Harada, Ryuichi; Iyoda, Sunao; Fujimura, Yoshihiro; Hamasaki, Yuko; Hataya, Hiroshi; Honda, Masashi

doi:10.1111/nep.13054

Cited by 7 publications

(3 citation statements)

References 22 publications

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“…Eculizumab demonstrated a high efficacy in patients with hemoglobinopathy ( Table 1 ). Despite its efficacy, infusion reactions have been reported and eculizumab has been shown to increase the risk of infection by encapsulated organisms, leading ideally to a meningococcal vaccination at least 2 weeks prior the initiation of therapy [ 44 ].…”

Section: Discussionmentioning

confidence: 99%

Delayed Severe Hemolytic Transfusion Reaction During Pregnancy in a Woman with β-Thalassemia Intermediate: Successful Outcome After Eculizumab Administration

et al. 2021

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Patient: Female, 39-year-old Final Diagnosis: Hemolytic transfusion reaction Symptoms: Hemolysis • severe anemia Medication: — Clinical Procedure: — Specialty: Hematology Objective: Rare co-existance of disease or pathology Background: Delayed hemolytic transfusion reactions (DHTR) are life-threatening complications mostly triggered by red blood cell (RBC) transfusions in patients with hemoglobinopathy. Case Report: We present a case of DHTR and hyperhemolysis syndrome in a 39-year-old pregnant woman with a history of β-thalassemia intermediate in whom the hemoglobin (Hb) level fell to 27 g/L after transfusion of 2 units of crossmatch-compatible packed RBCs. No allo- or auto-antibody formation was detected. Administration of intravenous immunoglobulins and methylprednisolone followed by anti-CD20 rituximab was tried, but was unsuccessful. Infusions of eculizumab (900 mg twice at a 7-day interval) followed by another course of intravenous immunoglobulins (2 g/kg/day for 5 days) and combined with repeated erythropoietin injections (darbepoetin alpha 300 µg/week) finally allowed biological and clinical improvement. Blood counts remained controlled until delivery. Despite signs of intrauterine growth retardation, she gave birth by cesarean section at 31 weeks of pregnancy to a 1.15-kg infant. Conclusions: Eculizumab seems to be of benefit in DHTR associated with hyperhemolysis and should be used early in the treatment of this pathology. Despite premature birth, our case report showed an acceptable outcome for the infant when eculizumab treatment was used during pregnancy.

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Section: Discussionmentioning

confidence: 99%

Delayed Severe Hemolytic Transfusion Reaction During Pregnancy in a Woman with β-Thalassemia Intermediate: Successful Outcome After Eculizumab Administration

et al. 2021

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“…In this regard, prophylaxis with various antibiotics has been performed to prevent infection in infants with aHUS undergoing eculizumab therapy before meningococcal vaccination [8][9][10][11][12][13]. There are the following three reports on infants who are at least 2 months old and qualified for eculizumab therapy [1]: amoxicillin prophylaxis was implemented until 2 weeks after vaccination (MCV4-TT; Nimenrix ® ) in a 3-month-old infant [8]; in a 4-month-old infant, prophylactic administration of cefdinir was started at the beginning of eculizumab therapy, and vaccination (MCV4-DT; Menactra ® ) was performed 2 weeks later (6 months after birth) and 17 weeks later (9 months after birth) [9]; and prophylactic antibiotics such as ampicillin were given to five infants aged 3-6 months until at least 2 weeks after vaccination [10]. Based on the findings from previous reports [8][9][10][11][12][13], prophylactic antibiotics can be necessary at least until vaccination, although there still remains controversy.…”

Section: Discussionmentioning

confidence: 99%

“…However, no standard of care for preventing meningococcal infection before immunization with meningococcal vaccine has been established. Previous studies have shown that prophylactic antibiotics were administered to infants with aHUS before or in parallel with eculizumab therapy [8][9][10][11][12][13]. Here we report our recent experience in two blood relative infants, a 4-month-old and a 5-month-old, who developed aHUS and were managed with prophylactic amoxicillin (20 mg/kg/day) [14] to prevent meningococcal infection from the beginning of eculizumab therapy to immunization with meningococcal vaccine.…”

Section: Introductionmentioning

confidence: 99%

Prophylactic amoxicillin for the prevention of meningococcal infection in infants with atypical hemolytic uremic syndrome under treatment with eculizumab: a report of two cases

Tanaka¹,

Fujita²,

Hibino³

2020

CEN Case Rep

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Eculizumab, a humanized monoclonal antibody to complement C5, is a therapeutic drug for atypical hemolytic-uremic syndrome (aHUS) that inhibits the terminal pathway of complement. Patients on eculizumab therapy may become more susceptible to infection with capsule-forming bacteria, including meningococci. Therefore, meningococcal vaccination is required for patients who are on eculizumab therapy. However, the means to prevent meningococcal infection in infants who cannot be vaccinated with the available meningococcal vaccine have not yet been established internationally. In two infants with aHUS at 4-5 months after birth, prophylactic oral amoxicillin was administered, and meningococcal infection was not detected during the period between the initiation of eculizumab therapy and the administration of meningococcal vaccine. Neither adverse events related to amoxicillin nor thrombotic microangiopathy occurred during the treatment. Thus, oral administration of amoxicillin may be effective for preventing meningococcal infection under treatment with eculizumab in infants who have not received meningococcal vaccination.

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Pediatric Neuromyelitis Optica Spectrum Disorders

Gombolay

Chitnis

2018

Curr Treat Options Neurol

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The aquaporin-4 antibody (AQP4 ab) is specific for NMOSD; however, recently another antibody, the myelin oligodendrocyte glycoprotein (MOG ab) has been found in a subset of AQP4 ab-negative patients including in children. Most treatment studies are reported in adults, but retrospective studies on efficacy and safety of mycophenolate mofetil, azathioprine, and rituximab in pediatric NMOSD are available. While some pediatric NMOSD-specific treatment studies are available, more research is needed in the mechanisms of early onset and specific treatment options in children, including whether different treatment considerations are needed for AQP4 ab as opposed to MOG ab-positive disease.

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Practical issues in using eculizumab for children with atypical haemolytic uraemic syndrome in the acute phase: A review of four patients

Cited by 7 publications

References 22 publications

Delayed Severe Hemolytic Transfusion Reaction During Pregnancy in a Woman with β-Thalassemia Intermediate: Successful Outcome After Eculizumab Administration

Delayed Severe Hemolytic Transfusion Reaction During Pregnancy in a Woman with β-Thalassemia Intermediate: Successful Outcome After Eculizumab Administration

Prophylactic amoxicillin for the prevention of meningococcal infection in infants with atypical hemolytic uremic syndrome under treatment with eculizumab: a report of two cases

Pediatric Neuromyelitis Optica Spectrum Disorders

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