scholarly journals An adult case of atypical hemolytic uremic syndrome presented with posterior reversible encephalopathy syndrome: Successful response to late-onset eculizumab treatment

2018 ◽  
Vol 10 (3) ◽  
Author(s):  
Serife Solmaz Medeni ◽  
Sinem Namdaroglu ◽  
Tugba Cetintepe ◽  
Can Ozlu ◽  
Funda Tasli ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Plasma Exchange ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
Complement Activation ◽  
Late Onset ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Neurological Involvement ◽  
Posterior Reversible Encephalopathy ◽  
Uremic Syndrome ◽  
Reversible Encephalopathy

Atypical hemolytic uremic syndrome is a rare and progressive disease caused by uncontrolled alternative complement activation. Dysregulatıon of the complement activation results in thrombotic microangiopathy and multiorgan damage. A 29-yearold woman who was admitted with complaints of vomiting and headache was detected to have acute renal failure with microangiopathic hemolytic anemia (MAHA). After the diagnosis of atypical hemolytic uremic syndrome (aHUS), she was treated with plasma exchange (PE) and hemodialysis (HD). She has experienced hypertensionrelated posterior reversible encephalopathy syndrome (PRES) at the second plasma exchange. She was initiated on eculizumab therapy because of no response to PE on the 34th days. Her renal functions progressively improved with eculizumab treatment. Dependence on dialysis was over by the 4th month. Dialysis free-serum Creatinine level was 2.2 mg/dL [glomerular filtration rate (e-GFR): 30 mL/min/1.73 m2] after 24 months. Neurological involvement (PRES, etc.) is the most common extrarenal complication and a major cause of mortality and morbidity from aHUS. More importantly, we showed that renal recovery may be obtained following late-onset eculizumab treatment in patient with aHUS after a long dependence on hemodialysis.

scholarly journals Eculizumab Therapy Leads to Rapid Resolution of Thrombocytopenia in Atypical Hemolytic Uremic Syndrome

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Han-Mou Tsai ◽  
Elizabeth Kuo
Keyword(s):  
Renal Function ◽  
Platelet Count ◽  
Hemolytic Uremic Syndrome ◽  
Plasma Exchange ◽  
Complement Activation ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Steady Increase ◽  
Platelet Counts ◽  
Plasma Exchange Therapy ◽  
Uremic Syndrome

Eculizumab is highly effective in controlling complement activation in patients with the atypical hemolytic uremic syndrome (aHUS). However, the course of responses to the treatment is not well understood. We reviewed the responses to eculizumab therapy for aHUS. The results show that, in patients with aHUS, eculizumab therapy, when not accompanied with concurrent plasma exchange therapy, led to steady increase in the platelet count and improvement in extra-renal complications within 3 days. By day 7, the platelet count was normal in 15 of 17 cases. The resolution of hemolytic anemia and improvement in renal function were less predictable and were not apparent for weeks to months in two patients. The swift response in the platelet counts was only observed in one of five cases who received concurrent plasma exchange therapy and was not observed in a case of TMA due to gemcitabine/carboplatin. In summary, eculizumab leads to rapid increase in the platelet counts and resolution of extrarenal symptoms in patients with aHUS. Concurrent plasma exchange greatly impedes the response of aHUS to eculizumab therapy. Eculizumab is ineffective for gemcitabine/carboplatin associated TMA.

Efficacy of plasma exchange in the therapy of recurrent pregnancy-associated atypical hemolytic-uremic syndrome

2021 ◽  
Vol 6_2021 ◽  
pp. 186-191
Author(s):  
Kirsanova T.V. Kirsanova ◽  
Fedorova T.A. Fedorova ◽  
Gurbanova S.R. Gurbanova ◽  
Pyregov A.V. Pyregov ◽  
Vinogradova M.A. Vinogradova ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Plasma Exchange ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Uremic Syndrome

scholarly journals Desafios Diagnósticos e Terapêuticos na Síndrome Hemolítica Urémica Atípica: A Propósito de Um Caso Clínico

2019 ◽  
Vol 32 (10) ◽  
pp. 673
Author(s):  
Sofia Reis ◽  
Daniela Ramos ◽  
Carolina Cordinhã ◽  
Clara Gomes
Keyword(s):  
Differential Diagnosis ◽  
Hemolytic Uremic Syndrome ◽  
Shiga Toxin ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Specific Treatment ◽  
Neurological Involvement ◽  
Thrombocytopenic Purpura ◽  
Alternate Pathway ◽  
Uremic Syndrome ◽  
Deficient Activity

The atypical hemolytic uremic syndrome comprises a thrombotic microangiopathy resulting from the complement alternate pathway hyperactivation. Its severity requires early diagnosis and treatment. The differential diagnosis includes typical hemolytic uremic syndrome (associated with Shiga toxin) and thrombotic thrombocytopenic purpura (associated with deficient activity of ADAMTS13). The only specific treatment currently available for atypical hemolytic uremic syndrome is eculizumab. We describe the case of a child with atypical hemolytic uremic syndrome diagnosed in the context of bloody diarrhea, complicated by neurological involvement that posed several diagnostic and therapeutic challenges.

scholarly journals Complement Activation and Thrombotic Microangiopathies

2019 ◽  
Vol 14 (12) ◽  
pp. 1719-1732 ◽  
Author(s):  
Marta Palomo ◽  
Miquel Blasco ◽  
Patricia Molina ◽  
Miquel Lozano ◽  
Manuel Praga ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Hemolytic Uremic Syndrome ◽  
Acute Phase ◽  
Complement Activation ◽  
Hellp Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Malignant Hypertension ◽  
Clinical Relapse ◽  
Thrombotic Microangiopathies ◽  
Uremic Syndrome

Background and objectivesAtypical hemolytic uremic syndrome is a form of thrombotic microangiopathy caused by dysregulation of the alternative complement pathway. There is evidence showing complement activation in other thrombotic microangiopathies. The aim of this study was to evaluate complement activation in different thrombotic microangiopathies and to monitor treatment response.Design, setting, participants, & measurementsComplement activation was assessed by exposing endothelial cells to sera or activated-patient plasma—citrated plasma mixed with a control sera pool (1:1)—to analyze C5b-9 deposits by immunofluorescence. Patients with atypical hemolytic uremic syndrome (n=34) at different stages of the disease, HELLP syndrome (a pregnancy complication characterized by hemolysis, elevated liver enzymes, and low platelet count) or severe preeclampsia (n=10), and malignant hypertension (n=5) were included.ResultsAcute phase atypical hemolytic uremic syndrome–activated plasma induced an increased C5b-9 deposition on endothelial cells. Standard and lower doses of eculizumab inhibited C5b-9 deposition in all patients with atypical hemolytic uremic syndrome, except in two who showed partial remission and clinical relapse. Significant fibrin formation was observed together with C5b-9 deposition. Results obtained using activated-plasma samples were more marked and reproducible than those obtained with sera. C5b-9 deposition was also increased with samples from patients with HELLP (all cases) and preeclampsia (90%) at disease onset. This increase was sustained in those with HELLP after 40 days, and levels normalized in patients with both HELLP and preeclampsia after 6–9 months. Complement activation in those with malignant hypertension was at control levels.ConclusionsThe proposed methodology identifies complement overactivation in patients with atypical hemolytic uremic syndrome at acute phase and in other diseases such as HELLP syndrome and preeclampsia. Moreover, it is sensitive enough to individually assess the efficiency of the C5 inhibition treatment.

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