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A case of atypical hemolytic uremic syndrome successfully treated with eculizumab.

Thajudeen B, Sussman A, Bracamonte E - Case Rep Nephrol Urol (2013)

Bottom Line: We report a case of aHUS successfully treated with eculizumab.Treatment with eculizumab was initiated which resulted in the stabilization of his hemoglobin, platelets, and LDH.Eculizumab is an effective therapeutic agent in the treatment of aHUS.

View Article: PubMed Central - PubMed

Affiliation: Division of Nephrology, University of Arizona, Tucson, Ariz., USA.

ABSTRACT
Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy (TMA) characterized by the triad of hemolytic anemia, thrombocytopenia, and acute renal failure. Eculizumab, a monoclonal complement C5 antibody which prevents the induction of the terminal complement cascade, has recently emerged as a therapeutic option for aHUS. We report a case of aHUS successfully treated with eculizumab. A 51-year-old male was admitted to the hospital following a mechanical fall. His past medical history was significant for rheumatic valve disease and mitral valve replacement; he was on warfarin for anticoagulation. A computed tomography scan of the head revealed a right-sided subdural hematoma due to coagulopathy resulting from a supratherapeutic international normalized ratio (INR). Following treatment with prothrombin complex concentrate to reverse the INR, urine output dropped and his serum creatinine subsequently increased to 247.52 μmol/l from the admission value of 70.72 μmol/l. Laboratory evaluation was remarkable for hemolytic anemia, thrombocytopenia, elevated lactate dehydrogenase (LDH), low haptoglobin, and low complement C3. A renal biopsy was consistent with TMA, favoring a diagnosis of aHUS. Treatment with eculizumab was initiated which resulted in the stabilization of his hemoglobin, platelets, and LDH. Hemodialysis was terminated after 2.5 months due to improvement in urine output and solute clearance. The interaction between thrombin and complement pathway might be responsible for the pathogenesis of aHUS in this case. Eculizumab is an effective therapeutic agent in the treatment of aHUS. Early targeting of the complement system may modify disease progression and thus treat aHUS more effectively.

No MeSH data available.


Related in: MedlinePlus

Electron microscopy demonstrates capillary loops occluded by increased cells and occasional fibrin thrombi (arrow). There was mild swelling of the endothelial cell cytoplasm and patchy effacement of epithelial cell foot processes. No evidence of electron-dense deposits was seen (×2,650).
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Figure 3: Electron microscopy demonstrates capillary loops occluded by increased cells and occasional fibrin thrombi (arrow). There was mild swelling of the endothelial cell cytoplasm and patchy effacement of epithelial cell foot processes. No evidence of electron-dense deposits was seen (×2,650).

Mentions: On day 4, a renal biopsy was performed which conformed TMA. The findings are outlined below (fig. 1, fig. 2, fig. 3).


A case of atypical hemolytic uremic syndrome successfully treated with eculizumab.

Thajudeen B, Sussman A, Bracamonte E - Case Rep Nephrol Urol (2013)

Electron microscopy demonstrates capillary loops occluded by increased cells and occasional fibrin thrombi (arrow). There was mild swelling of the endothelial cell cytoplasm and patchy effacement of epithelial cell foot processes. No evidence of electron-dense deposits was seen (×2,650).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3924711&req=5

Figure 3: Electron microscopy demonstrates capillary loops occluded by increased cells and occasional fibrin thrombi (arrow). There was mild swelling of the endothelial cell cytoplasm and patchy effacement of epithelial cell foot processes. No evidence of electron-dense deposits was seen (×2,650).
Mentions: On day 4, a renal biopsy was performed which conformed TMA. The findings are outlined below (fig. 1, fig. 2, fig. 3).

Bottom Line: We report a case of aHUS successfully treated with eculizumab.Treatment with eculizumab was initiated which resulted in the stabilization of his hemoglobin, platelets, and LDH.Eculizumab is an effective therapeutic agent in the treatment of aHUS.

View Article: PubMed Central - PubMed

Affiliation: Division of Nephrology, University of Arizona, Tucson, Ariz., USA.

ABSTRACT
Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy (TMA) characterized by the triad of hemolytic anemia, thrombocytopenia, and acute renal failure. Eculizumab, a monoclonal complement C5 antibody which prevents the induction of the terminal complement cascade, has recently emerged as a therapeutic option for aHUS. We report a case of aHUS successfully treated with eculizumab. A 51-year-old male was admitted to the hospital following a mechanical fall. His past medical history was significant for rheumatic valve disease and mitral valve replacement; he was on warfarin for anticoagulation. A computed tomography scan of the head revealed a right-sided subdural hematoma due to coagulopathy resulting from a supratherapeutic international normalized ratio (INR). Following treatment with prothrombin complex concentrate to reverse the INR, urine output dropped and his serum creatinine subsequently increased to 247.52 μmol/l from the admission value of 70.72 μmol/l. Laboratory evaluation was remarkable for hemolytic anemia, thrombocytopenia, elevated lactate dehydrogenase (LDH), low haptoglobin, and low complement C3. A renal biopsy was consistent with TMA, favoring a diagnosis of aHUS. Treatment with eculizumab was initiated which resulted in the stabilization of his hemoglobin, platelets, and LDH. Hemodialysis was terminated after 2.5 months due to improvement in urine output and solute clearance. The interaction between thrombin and complement pathway might be responsible for the pathogenesis of aHUS in this case. Eculizumab is an effective therapeutic agent in the treatment of aHUS. Early targeting of the complement system may modify disease progression and thus treat aHUS more effectively.

No MeSH data available.


Related in: MedlinePlus