Journal of Blood Disorders & Transfusion

A typical hemolytic uremic syndrome, diagnosis and treatment dilemma: A case report and literature review

6^th International Conference on Hematology

October 03-05, 2016 Orlando, USA

Deme Ergete Gurmu, Lisa C Thomas, John E Prior and Hasan Zerti

The Wright Center for Graduate Medical Education, USA

Posters & Accepted Abstracts: J Blood Disord Transfus

Abstract:

Background: Atypical hemolytic uremic syndrome (aHUS) is a rare disorder defined by the concurrent characteristic triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. Primary causes of aHUS are most likely due to complement gene mutations and in a very small percentage of cases from antibodies to complement proteins. Despite aHUS having a distinct pathophysiology, it is still difficult to differentiate from other thrombotic microangiopathies. Case Report: A 71 year old male patient presented with left scapular pain, anuric acute renal failure, hypertensive emergency, hemolysis and thrombocytopenia. Initially he was found to have non ST-segment elevation myocardial infarction (NSTEMI), but further intervention was withheld as the evaluation resulted in another life threatening condition: aHUS versus thrombotic thrombocytopenic purpura (TTP). The patient immediately started with plasmapheresis, as eculizumab was not readily available. Hemodialysis was initiated for anuric acute renal failure. ADAMTS 13 activity study was within normal range. Evaluation of therapeutic response was promising as the patient showed significant improvement within a few days. Conclusions: Understanding the genetics and epidemiology of aHUS is very limited and the overlapping clinical features with other thrombotic microangiopathy (TMA) syndromes often delay diagnosis and initiation of appropriate treatment of this rare disease.

Biography :

Email: demeergete@yahoo.com