Transfusion and Apheresis Science 52 (2015) 314–316
Contents lists available at ScienceDirect
Transfusion and Apheresis Science j o u r n a l h o m e p a g e : w w w. e l s e v i e r. c o m / l o c a t e / t r a n s c i
Case Report
An effective treatment of atypical hemolytic uremic syndrome with plasma exchange and eculizumab: A case report Nilay Sengul Samanci a,*, Mesut Ayer b, Abdulkadir Ergen a, Savas Ozturk c a
Department of Internal Medicine, Haseki Training and Research Hospital, Istanbul, Turkey Department of Hematology, Haseki Training and Research Hospital, Istanbul, Turkey c Department of Nephrology, Haseki Training and Research Hospital, Istanbul, Turkey b
A R T I C L E
I N F O
Article history: Received 4 January 2015 Accepted 8 January 2015 Keywords: Atypical hemolytic uremic syndrome Eculizumab Plazma exchange
A B S T R A C T
Atypical hemolytic uremic syndrome is a rare thrombotic microangiopathy caused by chronic defective regulation of the complement activation. This activation results in systemic endothelial damage leading to renal failure. Eculizumab, an anti-C5 antibody, is effective in limiting complement activation in patients with aHUS and has recently came out as a therapeutic option for aHUS. Here we present a case showing that first-line eculizumab treatment successfully prevents the induction of the terminal complement cascade and blocked the progression of thrombotic microangiopathy in aHUS. © 2015 Elsevier Ltd. All rights reserved.
1. Introduction Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. aHUS is known as non Shiga-toxin-HUS and represents the majority of HUS in adults [1]. It is a disease of complement dysregulation and has genetic or acquired functional defects in complement factor H (CFH), complement factor I (CFI) and membrane cofactor protein (MCP:CD 46) [2]. It has a poor prognosis that 25% of patients die in the acute phase and 50% of patients require ongoing renal replacement therapy [3].
2. Case report A 50-year-old male patient was admitted to our clinic with the complaint of dyspnea. On physical examination, his blood pressure was 240/140 and diminished breath sounds were present. Wbc: 6700, Hb: 8.44 g/dL, Hct: 25.8,
* Corresponding author. Department of Internal Medicine, Haseki Training and Research Hospital, Istanbul, Turkey. Tel.: +90 2125294400/ 1709; fax: +90 2125294463. E-mail addresss: [email protected] (N. Sengul Samanci). http://dx.doi.org/10.1016/j.transci.2015.01.006 1473-0502/© 2015 Elsevier Ltd. All rights reserved.
Plt: 81 10e9/L, creatinine: 10.38 mg/dl, urea: 198 mg/dl, albumin: 3.43 g/dL, LDH: 1021 U/L (
Contents lists available at ScienceDirect
Transfusion and Apheresis Science j o u r n a l h o m e p a g e : w w w. e l s e v i e r. c o m / l o c a t e / t r a n s c i
Case Report
An effective treatment of atypical hemolytic uremic syndrome with plasma exchange and eculizumab: A case report Nilay Sengul Samanci a,*, Mesut Ayer b, Abdulkadir Ergen a, Savas Ozturk c a
Department of Internal Medicine, Haseki Training and Research Hospital, Istanbul, Turkey Department of Hematology, Haseki Training and Research Hospital, Istanbul, Turkey c Department of Nephrology, Haseki Training and Research Hospital, Istanbul, Turkey b
A R T I C L E
I N F O
Article history: Received 4 January 2015 Accepted 8 January 2015 Keywords: Atypical hemolytic uremic syndrome Eculizumab Plazma exchange
A B S T R A C T
Atypical hemolytic uremic syndrome is a rare thrombotic microangiopathy caused by chronic defective regulation of the complement activation. This activation results in systemic endothelial damage leading to renal failure. Eculizumab, an anti-C5 antibody, is effective in limiting complement activation in patients with aHUS and has recently came out as a therapeutic option for aHUS. Here we present a case showing that first-line eculizumab treatment successfully prevents the induction of the terminal complement cascade and blocked the progression of thrombotic microangiopathy in aHUS. © 2015 Elsevier Ltd. All rights reserved.
1. Introduction Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. aHUS is known as non Shiga-toxin-HUS and represents the majority of HUS in adults [1]. It is a disease of complement dysregulation and has genetic or acquired functional defects in complement factor H (CFH), complement factor I (CFI) and membrane cofactor protein (MCP:CD 46) [2]. It has a poor prognosis that 25% of patients die in the acute phase and 50% of patients require ongoing renal replacement therapy [3].
2. Case report A 50-year-old male patient was admitted to our clinic with the complaint of dyspnea. On physical examination, his blood pressure was 240/140 and diminished breath sounds were present. Wbc: 6700, Hb: 8.44 g/dL, Hct: 25.8,
* Corresponding author. Department of Internal Medicine, Haseki Training and Research Hospital, Istanbul, Turkey. Tel.: +90 2125294400/ 1709; fax: +90 2125294463. E-mail addresss: [email protected] (N. Sengul Samanci). http://dx.doi.org/10.1016/j.transci.2015.01.006 1473-0502/© 2015 Elsevier Ltd. All rights reserved.
Plt: 81 10e9/L, creatinine: 10.38 mg/dl, urea: 198 mg/dl, albumin: 3.43 g/dL, LDH: 1021 U/L (
