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Thrombocytopenia-Associated Multiple Organ Failure Syndrome

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Pediatric Critical Care Medicine

Abstract

Thrombocytopenia-associated multiple organ failure (TAMOF) is a clinical syndrome often managed by critical care physicians. It is characterized by new onset thrombocytopenia in the setting of evolving multiple organ failure. TAMOF is an entity within the family of thrombotic microangiopathies, a spectrum of mixed coagulopathies and thrombotic disorders that include thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS) on one end of the spectrum and disseminated intravascular coagulation (DIC) on the other. Autopsies performed in patients who have succumbed to DIC, TTP and HUS reveal disseminated microvascular thromboses with distinct findings that help to differentiate these three entities. Furthermore, our biologic and molecular understanding of the pathophysiologic processes governing DIC, TTP and HUS have significantly expanded and allow better laboratory delineation among these three entities. Tissue factor plays a pivotal role in the initiation and propagation of DIC. Von Willebrand factors and deficient ADAMTS-13 (a.k.a von Willebrand factor-cleaving proteinase) drive the pathology in TTP. Shiga toxins and the complement pathway drive the pathology in HUS. With better understanding of the biology of TAMOF syndrome, innovative therapies are currently being evaluated with the hope of reversing this destructive pathology.

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Authors and Affiliations

  1. Department of Pediatrics, Texas Children’s Hospital/Baylor College of Medicine, 6621 Fannin St WT 06-006, Houston, TX, 77030, USA

    Trung C. Nguyen MD

  2. Department of Critical Care Medicine, Children’s Mercy Hospital and Clinics, 2401 Gillham Road, Kansas City, MO, 64108, USA

    Yong Y. Han MD

  3. Department of Pediatric Critical Care, Children’s Healthcare of Atlanta, Emory University School of Medicine, 1405 Clifton Road NE, Atlanta, GA, 30322, USA

    James D. Fortenberry MD, MCCM, FAAP

  4. State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Bei Li Shi Lu #167, Xi Cheng District, Beijing, PR, 100037, People’s Republic of China

    Zhou Zhou MD, PhD

  5. Medicine/Cardiovascular Research Section, One Baylor Plaza, N1319, Houston, TX, 77030, USA

    Miguel A. Cruz PhD

  6. Pediatric Intensive Care Unit, Children’s Hospital of Pittsburgh of UPMC, 4401 Forbes Avenue, FP2118, Pittsburgh, PA, 15224, USA

    Joseph A. Carcillo Jr. MD

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  1. Trung C. Nguyen MD
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  2. Yong Y. Han MD
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  3. James D. Fortenberry MD, MCCM, FAAP
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  4. Zhou Zhou MD, PhD
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  5. Miguel A. Cruz PhD
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  6. Joseph A. Carcillo Jr. MD
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Correspondence to Trung C. Nguyen MD .

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Editors and Affiliations

  1. Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA

    Derek S. Wheeler

  2. Div. of Critical Care Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA

    Hector R. Wong

  3. MICHR, University of Michigan Medical Scho, Ann Arbor, Michigan, USA

    Thomas P. Shanley

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Nguyen, T.C., Han, Y.Y., Fortenberry, J.D., Zhou, Z., Cruz, M.A., Carcillo, J.A. (2014). Thrombocytopenia-Associated Multiple Organ Failure Syndrome. In: Wheeler, D., Wong, H., Shanley, T. (eds) Pediatric Critical Care Medicine. Springer, London. https://doi.org/10.1007/978-1-4471-6416-6_31

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