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2017 ; 2017
(ä): 5184587
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Evans Syndrome Complicated by Intratubular Hemoglobin Cast Nephropathy
#MMPMID29163997
González I
; Rais R
; Gaut JP
; Dehner LP
Case Rep Pediatr
2017[]; 2017
(ä): 5184587
PMID29163997
show ga
Evans syndrome (ES) is a rare autoimmune disorder whose exact pathophysiology is
unknown. It is characterized by the simultaneous or subsequent development of
autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP).
Intravascular hemolysis, with hemoglobinemia, is known to produce acute kidney
injury; however, the development of intratubular hemoglobin casts (hemoglobin
cast nephropathy) in the setting of acute hemolysis is uncommon. Likewise, the
association of ES and acute renal failure is equally uncommon. We present a case
of a 7-year-old girl with ES who developed acute kidney injury in the setting of
intravascular hemolysis and had widespread intratubular hemoglobin casts.