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Severe transplant‐associated thrombotic microangiopathy in patients with hemoglobinopathies
Author(s) -
Abusin Ghada A.,
AbuArja Rolla,
Bajwa Rajinder P.S.,
Horwitz Edwin M.,
Auletta Jeffery J.,
Rangarajan Hemalatha G.
Publication year - 2017
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26503
Subject(s) - thrombotic microangiopathy , medicine , eculizumab , kidney disease , thalassemia , acute kidney injury , microangiopathy , hypertensive emergency , defibrotide , incidence (geometry) , tamponade , gastroenterology , hematopoietic stem cell transplantation , transplantation , disease , diabetes mellitus , immunology , endocrinology , physics , complement system , antibody , optics , blood pressure
Incidence and severity of transplant‐associated thrombotic microangiopathy (TA‐TMA) in patients with hemoglobinopathies receiving hematopoietic cell transplant is unknown. We report the outcomes for two patients with TA‐TMA who received eculizumab. A 2.5‐year‐old male with sickle cell disease developed TA‐TMA‐associated pericardial tamponade, severe hypertension, and acute kidney injury 2 months after transplant. A 7‐year‐old female with β‐thalassemia major developed TA‐TMA‐related acute kidney injury, severe hypertension, and seizures at 6 months after transplant. Both patients progressed to chronic kidney disease (CKD). In patients with hemoglobinopathies, preexisting endothelial dysfunction may place them at a greater risk for TA‐TMA and subsequent CKD.