Thrombotic Microangiopathies (TTP, HUS, HELLP)

https://doi.org/10.1016/j.emc.2014.04.008Get rights and content

Section snippets

Key points

  • Thrombotic microangiopathies, including thrombotic thrombocytopenic purpura (TTP), HUS and HELLP and its cousins—ITP, HIT, and DIC—are serious conditions that the emergency physician must recognize early to initiate life-saving treatments.

  • The diagnosis of TTP only requires evidence of a microangiopathic hemolytic anemia with thrombocytopenia and no other explanation.

  • A high clinical suspicion for thrombotic microangiopathies should be maintained in any patient presenting with thrombocytopenia or

Thrombotic Thrombocytopenic Purpura

Thrombotic thrombocytopenic purpura (TTP) is a systemic disease characterized by platelet aggregation into widespread platelet thrombi and resulting occlusion of the body’s microvasculature. This disease is closely related to and overlaps with hemolytic uremic syndrome (HUS) within the broader definition of TMAs.5 Historically, TTP has had mortalities as high as 90% when left untreated.6 Prompt recognition and initiation of early therapy have drastically reduced that mortality rate to 10% to

Immune thrombocytopenia purpura (ITP)

ITP is an acquired immune-mediated syndrome characterized by an isolated thrombocytopenia and increased risk of bleeding. It is classified as primary ITP, a diagnosis of exclusion with no inciting cause, or secondary ITP, an underlying condition or medication drives the immunologic response leading to platelet degradation.

HELLP

HELLP is a life-threatening condition that presents in pregnancy after 20 weeks’ gestation. It is regarded as a severe variant of preeclampsia, which may or may not be known at the time of diagnosis. Patients diagnosed with HELLP earlier in pregnancy tend to have significantly worse symptoms and disease course. The unborn fetus can also be affected, particularly with earlier disease manifestations resulting in fetal growth restriction.

Disseminated intravascular coagulation (DIC)

DIC is an acquired, systemic process of overstimulation of the coagulation pathway resulting in thrombosis, followed by consumption of platelets and coagulation factors, and ending in hemorrhage. DIC can be acute and decompensated when the generation of clotting factor cannot match the excessive consumption, or chronic and compensated when the clotting factor consumption is matched by production. Acute DIC has a rapid onset with bleeding seen in more than 64% of cases.65 Bleeding from more than

HIT

HIT occurs after the initiation of heparin and is divided into 2 distinct processes. Type 1 is generally clinically benign, non-immune-mediated, and a direct medication mediated effect. On the other hand, type 2 is a life-threatening and limb-threatening immune-mediated process with the formation of antibodies against the heparin-platelet factor 4 complex (PF4).68 Patients are often asymptomatic with unexplained thrombocytopenia 4 to 10 days after heparin exposure. Spontaneous bleeding is

Summary

TMAs including TTP, HUS, HELLP and the related disease processes, ITP, HIT and DIC, are serious conditions that the EP must recognize early to initiate life-saving treatments. Although it may be challenging to make the diagnosis, a heightened awareness in the thrombocytopenic patient should trigger a diagnostic evaluation and consultation with a hematology specialist to ensure appropriate management and a safe disposition plan (Table 10).

First page preview

First page preview
Click to open first page preview

References (70)

  • P.M. Mannucci

    Thrombotic microangiopathies: the past as prologue

    Eur J Intern Med

    (2013)
  • J.B. Segal et al.

    Prevalence of immune thrombocytopenia: analyses of administrative data

    J Thromb Haemost

    (2006)
  • S. Cortelazzo et al.

    High risk of severe bleeding in aged patients with chronic idiopathic thrombocytopenic purpura

    Blood

    (1991)
  • G. Kistangari et al.

    Immune Thrombocytopenia

    Hematol Oncol Clin North Am

    (2013)
  • F. Rodeghiero et al.

    Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group

    Blood

    (2009)
  • M.G. Mazzucconi et al.

    Therapy with high-dose dexamethasone in previously untreated patients affected by idiopathic thrombocytopenia purpura: a GIMEMA experience

    Blood

    (2007)
  • V. Blanchette et al.

    Childhood immune thrombocytopenic purpura: diagnosis and management

    Hematol Oncol Clin North Am

    (2010)
  • T. Kuhne et al.

    A prospective comparative study of 2540 infants and children with newly diagnosed idiopathic thrombocytopenia purpura (ITP) from the Intercontinental Childhood ITP Study Group

    J Pediatr

    (2003)
  • C.E. Neunert et al.

    Severe hemorrhage in children with newly diagnosed immune thrombocytopenic purpura

    Blood

    (2008)
  • N. Arulkumaran

    Severe pre-eclampsia and hypertensive crises

    Best Pract Res Clin Obstet Gynaecol

    (2013)
  • U. Abildgaard et al.

    Pathogenesis of the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP): a review

    Eur J Obstet Gynecol Reprod Biol

    (2013)
  • L. Weinstein

    Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy

    Am J Obstet Gynecol

    (1982)
  • G. Boregowda

    Gastrointestinal and liver disease in pregnancy

    Best Pract Res Clin Obstet Gynaecol

    (2013)
  • D.M. Townsley

    Hematologic complications of pregnancy

    Semin Hematol

    (2013)
  • T.E. Warkentin et al.

    Heparin-induced thrombocytopenia: recognition, treatment, and prevention: the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy

    Chest

    (2004)
  • R. Diz-Kucukkaya et al.

    Thrombocytopenia

  • D.B. Brubaker et al.

    Intravascular and total body platelet equilibrium in healthy volunteers and in thrombocytopenic patients transfused with single donor platelets

    Am J Hematol

    (1998)
  • J.L. Moake

    Thrombotic microangiopathies

    N Engl J Med

    (2002)
  • G.A. Rock et al.

    Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura

    N Engl J Med

    (1991)
  • S.L. Allford et al.

    Guidelines on the diagnosis and management of the thrombotic microangiopathic hemolytic anemias

    Br J Hematol

    (2003)
  • M. Scully et al.

    Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies

    Br J Hematol

    (2012)
  • M. Michael et al.

    Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura

    Cochrane Database Syst Rev

    (2009)
  • P. Coppo et al.

    Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura

    Presse Med

    (2012)
  • R.L. Ridolfi et al.

    Thrombotic thrombocytopenic purpura. Report of 25 cases and review of the literature

    Medicine

    (1981)
  • H.-M. Tsai et al.

    Antibodies to von-Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura

    N Engl J Med

    (1998)
  • Cited by (0)

    Disclosure Statement: The authors have nothing to disclose.

    View full text