Cyclic neutropenia and concomitant IgA nephropathy: a case report

BMC Nephrology

Case Report

K. Kakleas¹  C. Kanaka-Gantenbein¹  T. Zaggogianni¹  H. Gakiopoulou²  A. Kapogiannis³  N. Stergiou³  C. Kapogiannis⁴ 

[1] First Department of Paediatrics, National and Kapodistrian University of Athens, Aghia Sophia Children’s Hospital, Athens, Greece;First Department of Pathology, Medical School, National and Kapodistrian University of Athens, Athens, Greece;Renal Unit, First Department of Paediatrics, National and Kapodistrian University of Athens, Aghia Sophia Children’s Hospital, Athens, Greece;Renal Unit, First Department of Paediatrics, National and Kapodistrian University of Athens, Aghia Sophia Children’s Hospital, Athens, Greece;Renal Unit, Great Ormond Street Hospital, London, UK;

关键词: Case report;    IgA nephropathy;    Cyclic neutropenia;    Angiotensin-converting-enzyme inhibitors;    Corticosteroids;   

DOI  :  10.1186/s12882-023-03179-1

 received in 2022-08-28, accepted in 2023-04-20,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundIgA nephropathy (IgAN) is universally recognized as one of the most common primary glomerular diseases in all ages. Cyclic neutropenia (CN) is a rare haematologic disorder that is associated with mutations of the ELANE gene. The co-occurrence of IgAN and CN is extremely rare. This is the first case report of a patient with IgAN and genetically confirmed CN.Case presentationWe report a case of a 10-year-old boy who presented with recurrent viral upper respiratory tract infections accompanied by several episodes of febrile neutropenia, haematuria, proteinuria and acute kidney injury. Upon first admission, his physical examination was unremarkable. His kidney function was impaired, whereas his urine microscopy showed evidence of macroscopic haematuria and proteinuria. Further workup showed elevated IgA. The renal histology was consistent with mesangial and endocapillary hypercellularity with mild crescentic lesions, while immunofluorescence microscopy showed IgA-positive staining, which was characteristic of IgAN. Moreover, genetic testing confirmed the clinical diagnosis of CN, therefore Granulocyte colony-stimulating factor (G-CSF) was initiated to stabilize the neutrophil count. Regarding proteinuria control, the patient was initially treated with an Angiotensin-converting-enzyme inhibitor for approximately 28 months. However, due to progressive proteinuria (> 1 g/24 h), Corticosteroids (CS) were added for a period of 6 months according to the revised 2021 KDIGO guidelines with favorable outcome.ConclusionsPatients with CN are more susceptible to recurrent viral infections, which can trigger IgAN attacks. In our case CS induced remarkable proteinuria remission. The use of G-CSF contributed to the resolution of severe neutropenic episodes, viral infections and concomitant AKI episodes, contributing to better prognosis of IgAN. Further studies are mandatory to determine whether there is a genetical predisposition for IgAN in children with CN.

【 授权许可】

CC BY   
© Crown 2023

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