Evans Syndrome Complicated by Intratubular Hemoglobin Cast Nephropathy

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  • Iván González
    The Lauren V. Ackerman Laboratory of Surgical Pathology, St. Louis Children’s Hospital, Washington University Medical Center, St. Louis, MO, USA
  • Rehan Rais
    The Lauren V. Ackerman Laboratory of Surgical Pathology, St. Louis Children’s Hospital, Washington University Medical Center, St. Louis, MO, USA
  • Joseph P. Gaut
    The Lauren V. Ackerman Laboratory of Surgical Pathology, St. Louis Children’s Hospital, Washington University Medical Center, St. Louis, MO, USA
  • Louis P. Dehner
    The Lauren V. Ackerman Laboratory of Surgical Pathology, St. Louis Children’s Hospital, Washington University Medical Center, St. Louis, MO, USA

Description

<jats:p>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.</jats:p>

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