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Idiopathic Thrombocytopenic Purpura Treatment & Management

  • Author: Michael A Silverman, MD, MD; Chief Editor: Gil Z Shlamovitz, MD, FACEP  more...
 
Updated: Jul 22, 2016
 

Prehospital Care

Prehospital care focuses on the ABCs, which include providing oxygen, controlling severe hemorrhage, and initiating intravenous (IV) fluids to maintain hemodynamic stability. Airway control may be necessary for a large intracranial hemorrhage.

EMS providers should be aware of the potential for serious bleeding complications in patients with idiopathic thrombocytopenic purpura (ITP).

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Emergency Department Care

Life-threatening bleeding requires conventional critical care interventions. In the patient with known ITP, high-dose parenteral glucocorticoids and IV immunoglobulin (IVIg), with or without platelet transfusions, are appropriate.

Platelet transfusion is indicated for controlling severe hemorrhage. Send a blood specimen to the lab for type and screen in case platelet transfusion is necessary. Platelet survival is increased if the platelets are transfused immediately after IVIg infusion. A consultation with a hematologist may be required to make a decision regarding the transfusion of platelets.

Guidelines for transfusion dosage are as follows:

  • 6-8 U of platelet concentrate, or 1 U/10 kg
  • 1 U of platelets to increase count of a 70-kg adult by 5-10,000/mm 3 and an 18-kg child by 20,000/mm 3

Splenectomy is reserved for patients in whom medical therapy fails. Emergent splenectomy is indicated in patients with life-threatening bleeding in whom medical therapy fails.

In patients without life-threatening complications, focus ED care on confirming the diagnosis, if possible, and initiating therapy as needed. Most patients with undiagnosed thrombocytopenia and purpura will need admission for further evaluation and treatment, since ITP is a diagnosis of exclusion.

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Consultations

Consult a hematologist for assistance in confirming the diagnosis or, in the patient with known ITP, arranging disposition and follow-up care, if appropriate.

Consult a neurosurgeon for intracranial hemorrhage. Consultation by other surgical specialists may be required for extensive hemorrhage at other sites.

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Medical Care

Treatment in pediatric patients

American Society of Hematology (ASH) guidelines recommend that in children who have no bleeding, or only mild bleeding (ie, skin manifestations only, such as bruising and petechiae), regardless of the platelet count, management should be with observation alone. For pediatric patients who require treatment, recommended first-line agents are a single dose of intravenous immunoglobulin (IVIg; 0.8-1 g/kg) or a short course of corticosteroids; IVIg provides a more rapid increase in the platelet count. A single dose of anti-D immune globulin can be used in Rh-positive, nonsplenectomized children.[3]

In children and adolescents who have significant ongoing bleeding despite first-line therapy, second-line treatments include rituximab or high-dose dexamethasone. Splenectomy is recommended for those who do not respond to, or cannot tolerate, other treatments and have significant or persistent bleeding, or need a better quality of life. However, ASH guidelines recommend waiting at least 12 months before performing splenectomy, in most cases.[3]

Treatment in adults

A 2010 international consensus report advised that treatment for ITP is rarely indicated in adult patients with platelet counts above 50 × 109/L, in the absence of the following[48] :

  • Bleeding due to platelet dysfunction or another hemostatic defect
  • Trauma
  • Surgery
  • Clearly identified comorbidities for bleeding
  • Mandated anticoagulation therapy
  • Profession or lifestyle that predisposes the patient to trauma

For adults with ITP, ASH guidelines recommend treating newly diagnosed patients whose platelet count is less than 30 × 109/L. Recommendations for first-line treatment include the following[3] :

  • Longer courses of corticosteroids are preferred over shorter courses or treatment with IVIg (1 g/kg, one-time dose)
  • IVIg may be used with corticosteroids when a more rapid increase in platelet count is required
  • Either IVIg or anti-D (in appropriate patients) be used if corticosteroids are contraindicated

Recommended second-line treatments for cases that do not respond to corticosteroids, or recur afterward, are as follows:

  • Splenectomy
  • Thrombopoietin receptor agonists for patients at risk of bleeding who relapse after splenectomy or who have a contraindication to splenectomy and have failed at least one other therapy
  • Rituximab may be considered for patients at risk of bleeding who have failed one line of therapy (eg, corticosteroids, IVIg, splenectomy)

Increasingly, clinicians are trying thrombopoietin receptor agonists before referring patients for splenectomy.  According to published data, 25–30% of patients who are treated with thrombopoietin receptor agonists have a sustained response after stopping the drug. The mechanism for these sustained responses is not known. Spontaneous remission is unlikely, as the patients in reported studies had all received several prior treatments and had severe, prolonged thrombocytopenia.[47]

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Contributor Information and Disclosures
Author

Michael A Silverman, MD, MD Chairman, Department of Emergency Medicine, Virginia Hospital Center; Instructor of Emergency Medicine, Johns Hopkins University School of Medicine

Michael A Silverman, MD, MD is a member of the following medical societies: American College of Emergency Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Jeffrey L Arnold, MD, FACEP Chairman, Department of Emergency Medicine, Santa Clara Valley Medical Center

Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine, American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Gil Z Shlamovitz, MD, FACEP Associate Professor of Clinical Emergency Medicine, Keck School of Medicine of the University of Southern California; Chief Medical Information Officer, Keck Medicine of USC

Gil Z Shlamovitz, MD, FACEP is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association

Disclosure: Nothing to disclose.

Additional Contributors

Edward A Michelson, MD Associate Professor, Program Director, Department of Emergency Medicine, University Hospital Health Systems of Cleveland

Edward A Michelson, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

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