Successful therapeutic plasma exchange in a 3.2-kg body weight neonate with atypical hemolytic uremic syndrome

2011 ◽  
Vol 26 (3) ◽  
pp. 162-165 ◽  
Author(s):  
Sunkyung Jung ◽  
Eun-Suk Kang ◽  
Chang-Seok Ki ◽  
Dae-Won Kim ◽  
Kyung-Hoon Paik ◽  
...  
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 ◽  
...  

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4635-4635
Author(s):  
Ju-Hsien Chao ◽  
Beth C. Riley ◽  
Rebecca Redman

Abstract Abstract 4635 Atypical hemolytic uremic syndrome is a rare but often lethal disease if not diagnosed and treated early. The mainstay of treatment has been plasma exchange, though eculizumab has recently been approved for treatment. We report a 24-year-old Caucasian male whom presented from a rehab facility with weakness. Three months prior, he had sustained polytrauma from a motorcycle accident. At discharge, labs were normal. At admission, he was found to have anemia, thrombocytopenia, and elevated creatinine and bilirubin. Further testing revealed evidence of hemolysis. ADAMTS13 level was greater than 100% to assess for thrombotic thrombocytopenic purpura (TTP). Flow cytometry for CD59 was negative to assess for paroxysmal nocturnal hemoglobinuria (PNH). Shiga toxin was negative. He was diagnosed with atypical hemolytic uremia syndrome (aHUS). Patient was initially treated with steroids then underwent daily plasma exchange for 2 weeks with improvements in lab parameters and no evidence of hemolysis. Exchanges were changed to every other day for 2 weeks with plans to start eculizumab as outpatient therapy. After discharge, one dose of eculizumab 900 mg was given without further exchanges. Three days later, lab check showed decrease in hemoglobin and platelets, concerning for aHUS exacerbation. Patient was admitted for daily plasma exchange with eculizumab 100 mg boost after each procedure for about 2 weeks. Once his lab parameters were consistent with resolution, exchanges were spaced out to every other day for another 1 week then discontinued. Then he was started on monotherapy with eculizumab 900 mg once weekly with surveillance lab monitoring. He had no further exacerbation of his aHUS and was discharged back to rehab with weekly eculizumab and lab surveillance. Early detection and treatment of aHUS is important to minimize the damages from the underlying thrombosis and prevent disease related mortality. Plasma exchange remains the standard treatment for this condition with close monitoring of lab parameters, though no set treatment schedule has been established. The recent approval of eculizumab offers another option in the management of this disease and appears equally efficacious. Disclosures: No relevant conflicts of interest to declare.


2011 ◽  
Vol 26 (10) ◽  
pp. 1915-1916 ◽  
Author(s):  
Jean-Claude Davin ◽  
Jaap Groothoff ◽  
Valentina Gracchi ◽  
Antonia Bouts

2017 ◽  
Vol 1 (1) ◽  
pp. 01-04
Author(s):  
Ahmed M. Essam ElDin Mansour ◽  
Mostafa Abd El-nassier Abd Elgawad

Aim: A retrospective research study revealing clinical features and prognostic parameters of 22 cases of gestational atypical hemolytic uremic syndrome. Methodology: A retrospective research study in an analytical manner of 22 cases of gestational Atypical hemolytic uremic syndrome from the Demerdash Maternity Hospital records using diverse management protocols. Results: 12 cases underwent plasma exchange management protocol with a favourable impact regarding renal response in only 8 cases. On the contrary, 10 cases were managed with high dose plasma infusion with an excellent renal response in 7 cases by regaining renal functional capacity in managed cases. Conclusion: The research performed by our group revealed considerable effectiveness of high dose therapy plasma infusion management of atypical HUS. Although therapeutic plasma exchange is the recommended treatment of HUS, this cumbersome procedure may not be available for all patients in an emergency. In this context, plasma infusion may represent an alternative first-line therapy.


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