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Renal thromboembolism during treatment with recombinant activated factor VII (rFVIIa) in a child with hemophilia B with factor IX inhibitors.

Milošević D, Bilić E, Batinić D, Poropat M, Štern-Padovan R, Galić S, Turudić D - BMC Pediatr (2014)

Bottom Line: We present unique case of patient with hemophilia B and high titer inhibitors to coagulation FIX, who developed severe renal damage due to thromboembolic event during rFVIIa therapy, associated with unsuspected renovascular anomalies.Caution is necessary if hematuria B requires administration of rFVIIa.US color doppler renal imaging before and after drug administration should be sufficient as an early warning.

View Article: PubMed Central - PubMed

ABSTRACT

Background: Serious thromboembolic events connected with rFVIIa therapy in hemophilia patients are rare. Only three cases are reported in children, all of them with hemophilia A.

Case presentation: We present unique case of patient with hemophilia B and high titer inhibitors to coagulation FIX, who developed severe renal damage due to thromboembolic event during rFVIIa therapy, associated with unsuspected renovascular anomalies.

Conclusion: Caution is necessary if hematuria B requires administration of rFVIIa. US color doppler renal imaging before and after drug administration should be sufficient as an early warning.

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Related in: MedlinePlus

Multi-slice computer tomography (MSCT) renal angiography of both kidneys in anteroposterior position shows severe left kidney damage (arrow) with 3 independent unobstructed arteries; two of them starting regularly, the third beginning caudally at the approximate position of the lower pole of the left kidney. (R = right kidney).
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Fig3: Multi-slice computer tomography (MSCT) renal angiography of both kidneys in anteroposterior position shows severe left kidney damage (arrow) with 3 independent unobstructed arteries; two of them starting regularly, the third beginning caudally at the approximate position of the lower pole of the left kidney. (R = right kidney).

Mentions: A seven-year-old Croatian boy with hemophilia B with high-titer inhibitors to coagulation FIX was admitted at our institution with severe hematuria. The parents denied trauma, any medication or infection. He was previously treated with rFVIIa, mostly for bleeding affecting limb joints. Clinical, diagnostic and medication follow-up is shown in Figure 1. Painless hematuria was treated during the first three days with only symptomatic therapy consisting of intravenous hyperhidration and bed rest. On the fourth and fifth day, a fall in hemoglobin level was noticed and single daily dose of 285 μg/kg rFVIIa was administered intravenously in a 10–20 minute interval on both consecutive days. Despite the therapy, a life-threatening condition developed on the sixth day with rapid fall of red blood cells count (RBC) accompanied with massive hematuria. The total rFVIIa dose was subsequently increased by administration every three hours, four times in total with each amount of 105 μg/kg. The treatment successfully stabilized RBC count and reduced hematuria. As hematuria, although reduced, continued, for the following two days the child received additional rFVIIa (once daily 285 μg/kg). On the fourth day of rFVIIa therapy the patient first time complained of left lumbar colic pain, and visible blood clots in urine appeared. The rFVIIa therapy was discontinued. Only hyperhydration and occasional spasmolytic therapy were continued. From the eleventh day, hematuria was only microscopic. In the course of the disease several ultrasound (US) examinations were performed. Initially, normal US showed, coincidently with renal colics, enlarged left kidney with hyperechogenic inhomogenous parenchyma with partial loss of corticomedulary differentiation and dilated pelvicaliceal system with hyperechogenic inhomogenous content compatible with clots. Only a vascular bed over the left kidney without visualization of the parenchyma with practically afunctional renographic curve was found on 99mTc-DTPA (Diethylene Triamine Pentacaetic Acid) renal scintigraphy (Figure 2A). In the first minutes of 99mTc-MAG3 (Mercaptoacetyltriglycine) scintigraphy, the left kidney was very pale becoming increasingly better visualized later (Figure 2B). Renographic curve showed obstruction over the third phase of the renogram. MSCT (multi-slice computer tomography) renal angiography revealed severe left kidney damage with 3 independent unobstructed arteries; two of them starting regularly, the third beginning caudally at the approximate position of the lower pole of the left kidney. The same kidney had 2 veins who communicated with each other, the first had circumaortal course with vascular convolutes and the second (accessory) showed retroaortal course supplying the lower pole of the kidney (Figure 3). Nine months later renal scintigraphy was repeated. The finding was normal.Figure 1


Renal thromboembolism during treatment with recombinant activated factor VII (rFVIIa) in a child with hemophilia B with factor IX inhibitors.

Milošević D, Bilić E, Batinić D, Poropat M, Štern-Padovan R, Galić S, Turudić D - BMC Pediatr (2014)

Multi-slice computer tomography (MSCT) renal angiography of both kidneys in anteroposterior position shows severe left kidney damage (arrow) with 3 independent unobstructed arteries; two of them starting regularly, the third beginning caudally at the approximate position of the lower pole of the left kidney. (R = right kidney).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4279815&req=5

Fig3: Multi-slice computer tomography (MSCT) renal angiography of both kidneys in anteroposterior position shows severe left kidney damage (arrow) with 3 independent unobstructed arteries; two of them starting regularly, the third beginning caudally at the approximate position of the lower pole of the left kidney. (R = right kidney).
Mentions: A seven-year-old Croatian boy with hemophilia B with high-titer inhibitors to coagulation FIX was admitted at our institution with severe hematuria. The parents denied trauma, any medication or infection. He was previously treated with rFVIIa, mostly for bleeding affecting limb joints. Clinical, diagnostic and medication follow-up is shown in Figure 1. Painless hematuria was treated during the first three days with only symptomatic therapy consisting of intravenous hyperhidration and bed rest. On the fourth and fifth day, a fall in hemoglobin level was noticed and single daily dose of 285 μg/kg rFVIIa was administered intravenously in a 10–20 minute interval on both consecutive days. Despite the therapy, a life-threatening condition developed on the sixth day with rapid fall of red blood cells count (RBC) accompanied with massive hematuria. The total rFVIIa dose was subsequently increased by administration every three hours, four times in total with each amount of 105 μg/kg. The treatment successfully stabilized RBC count and reduced hematuria. As hematuria, although reduced, continued, for the following two days the child received additional rFVIIa (once daily 285 μg/kg). On the fourth day of rFVIIa therapy the patient first time complained of left lumbar colic pain, and visible blood clots in urine appeared. The rFVIIa therapy was discontinued. Only hyperhydration and occasional spasmolytic therapy were continued. From the eleventh day, hematuria was only microscopic. In the course of the disease several ultrasound (US) examinations were performed. Initially, normal US showed, coincidently with renal colics, enlarged left kidney with hyperechogenic inhomogenous parenchyma with partial loss of corticomedulary differentiation and dilated pelvicaliceal system with hyperechogenic inhomogenous content compatible with clots. Only a vascular bed over the left kidney without visualization of the parenchyma with practically afunctional renographic curve was found on 99mTc-DTPA (Diethylene Triamine Pentacaetic Acid) renal scintigraphy (Figure 2A). In the first minutes of 99mTc-MAG3 (Mercaptoacetyltriglycine) scintigraphy, the left kidney was very pale becoming increasingly better visualized later (Figure 2B). Renographic curve showed obstruction over the third phase of the renogram. MSCT (multi-slice computer tomography) renal angiography revealed severe left kidney damage with 3 independent unobstructed arteries; two of them starting regularly, the third beginning caudally at the approximate position of the lower pole of the left kidney. The same kidney had 2 veins who communicated with each other, the first had circumaortal course with vascular convolutes and the second (accessory) showed retroaortal course supplying the lower pole of the kidney (Figure 3). Nine months later renal scintigraphy was repeated. The finding was normal.Figure 1

Bottom Line: We present unique case of patient with hemophilia B and high titer inhibitors to coagulation FIX, who developed severe renal damage due to thromboembolic event during rFVIIa therapy, associated with unsuspected renovascular anomalies.Caution is necessary if hematuria B requires administration of rFVIIa.US color doppler renal imaging before and after drug administration should be sufficient as an early warning.

View Article: PubMed Central - PubMed

ABSTRACT

Background: Serious thromboembolic events connected with rFVIIa therapy in hemophilia patients are rare. Only three cases are reported in children, all of them with hemophilia A.

Case presentation: We present unique case of patient with hemophilia B and high titer inhibitors to coagulation FIX, who developed severe renal damage due to thromboembolic event during rFVIIa therapy, associated with unsuspected renovascular anomalies.

Conclusion: Caution is necessary if hematuria B requires administration of rFVIIa. US color doppler renal imaging before and after drug administration should be sufficient as an early warning.

Show MeSH
Related in: MedlinePlus