Clinical Snapshot
Renal Hemorrhage in Severe Hemophilia A
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A 7-year-old male with severe hemophilia A and inhibitor (21 BU) presented to the emergency department with isolated macrohematuria. On ultrasound, the left kidney showed architecturally abnormal/inhomogenous parenchyma and diffuse hemorrhages (Figure a arrows, left image), mold-shaped, hyperechoic clot formation in the renal pelvis-calcyceal system (Figure b, arrowhead, right image), as well as a sharply delineated hyperechoic parenchymal wedge indicating secondary infarction (Figure b arrow, right image). Due to hemophilia with inhibitors, intravenous recombinant activated factor VII was administered as replacement therapy. As a result of this, hematuria was no longer detectable after 12 h, meaning that the patient could be discharged 2 days later. Hematuria in adolescents/adults with hemophilia is a common bleeding symptom, whereas intrarenal hemorrhage is rarer. Spontaneous renal hemorrhage/hematuria, as medical emergencies, require immediate replacement therapy with factor VIII or—if inhibitors are present—the administration of bypassing agents (recombinant activated factor VII, factor VIII inhibitor bypassing activity [FEIBA]), as well as close monitoring to prevent potential complications such as urinary tract obstruction or severe anemia.
PD Dr. med. Martin Olivieri, Dr. med. Victoria Lieftüchter, Pädiatrische Hämostaseologie, Dr. von Haunersches Kinderspital, LMU Klinikum, München, martin.olivieri@med.uni-muenchen.de
Dr. med. Marco Paolini, Klinik und Poliklinik für Radiologie, LMU Klinikum, München
Conflict of interest statement: The authors state that no conflict of interest exists.
Translated from the original German by Christine Rye.
Cite this as: Olivieri M, Lieftüchter V, Paolini M: Renal hemorrhage in severe hemophilia A. Dtsch Arztebl Int 2025; 122: 721. DOI: 10.3238/arztebl.m2025.0109
Photograph: PD Dr. Marco Paolini
