Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome

  • Jodie Bachman, DO, MBS Saint Barnabas Medical Center, Livingston, NJ
  • William Cieplinski, MD, PhD Goshen Medical Associates, Goshen, NY
  • Aliss Markosian, DO, MPH UCLA Health, Los Angeles, CA
  • Jay H. Shubrook, DO, FACOFP Touro University California, Primary Care Department, Vallejo, CA

Abstract

Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome is an acute illness with abnormalities in multiple organ systems. Patients typically present with thrombotic microangiopathy, thrombocytopenia, and microangiopathic hemolytic anemia without another apparent cause. Additional features include fever, neurologic, and renal abnormalities depending on the site of microangiopathic damage. Potential causes include congenital deficiencies, Shiga-Toxin producing bacteria, numerous drugs, factors related to malignancy, allogeneic hematopoietic cell transplant, cardiovascular surgery, and pregnancy. A medical emergency, appropriate treatment needs to be initiated promptly or the disease can be fatal. Plasma exchange is the initial treatment of choice, but plasma infusion can be used until plasma exchange is available. Adjunctive treatment with glucocorticoids may be used in certain scenarios. If left untreated, the syndrome typically progressively worsens. Affected individuals experience irreversible renal failure, progressive neurological deterioration, cardiac ischemia, and death. With a prompt recognition of the disease and treatment initiation, patients have a better prognosis and mortality rate.

Published
2018-04-13
How to Cite
Bachman, DO, MBS, J., W. Cieplinski, MD, PhD, A. Markosian, DO, MPH, and J. H. Shubrook, DO, FACOFP. “Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome”. Osteopathic Family Physician, Vol. 10, no. 2, Apr. 2018, https://ofpjournal.com/index.php/ofp/article/view/557.
Section
Review Articles