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Hypoprothrombinemia Treatment & Management

  • Author: J Nathan Hagstrom, MD; Chief Editor: Robert J Arceci, MD, PhD  more...
 
Updated: Apr 08, 2016
 

Medical Care

Initial treatment of hypoprothrombinemia is aimed at controlling hemorrhage. Numerous products that provide prothrombin are available. Frozen plasma contains about 1 U/mL of prothrombin. It is readily available and contains other factors that may be useful if the hypoprothrombinemia is associated with multiple factor deficiencies. Concentrates of prothrombin complex (eg, Proplex T, Konyne 80, Bebulin VH) are concentrated sources of prothrombin. However, these products also contain other vitamin K–dependent factors in high concentration, and use of these products at high doses has been associated with thromboembolic complications. Prothrombin-complex concentrates may contain activated clotting factors, and their use has been associated with thromboembolic complications. No pure concentrate of prothrombin factor is available.

When lupus anticoagulant-hypoprothrombinemia syndrome (LAHS) is associated with systemic lupus erythematosus, treatment with steroids, intravenous immunoglobulin, fresh-frozen plasma, or azathioprine has been successful in reducing lupus anticoagulant levels, in increasing prothrombin levels, and in controlling bleeding. However, prothrombin levels have decreased in some patients with the drugs were tapered.

In vitamin K deficiency and warfarin overdose, vitamin K is the treatment of choice unless clinically significant bleeding is present and quick correction of the coagulopathy is desired. In these cases, use either frozen plasma or a prothrombin-complex concentrate.

The question of prophylactic treatment in patients with hypoprothrombinemia is controversial. No replacement protocols are standard. Prophylaxis has been reserved for patients who have had severe, recurrent episodes of bleeding. Case reports of patients with severe hypoprothrombinemia who were treated weekly with prothrombin-complex concentrates described a reduction in hemorrhagic episodes and improved quality of life.

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Surgical Care

Surgery could result in clinically significant bleeding in patients with hypoprothrombinemia. Avoid surgery whenever possible. Use concentrates of prothrombin complex in patients with factor II deficiency who require surgery. If an inhibitor is present, attempt to decrease the inhibitor titer before the surgical procedure, if possible.

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Activity

Patients with hypoprothrombinemia must avoid activities and situations that could result in clinically significant trauma, especially head trauma.

Several variables determine a child's risk of bleeding. Among them are the nature and severity of the trauma, the severity of the bleeding disorder, and the speed at which treatment can be administered.

Risk of bleeding during athletic activity increases as the level of competition increases and as the likelihood of collision at top running speed increases.

The National Hemophilia Foundation has published guidelines regarding athletic activity in people with bleeding disorders.

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Contributor Information and Disclosures
Author

J Nathan Hagstrom, MD Division Head and Director, Hematology-Oncology, Connecticut Children's Medical Center; Associate Professor of Pediatrics, University of Connecticut

J Nathan Hagstrom, MD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Coauthor(s)

James L Harper, MD Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Associate Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Society of Pediatric Hematology/Oncology, American Federation for Clinical Research, Council on Medical Student Education in Pediatrics, Hemophilia and Thrombosis Research Society, American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Gary D Crouch, MD Associate Professor, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD Director, Children’s Center for Cancer and Blood Disorders, Department of Hematology/Oncology, Co-Director of the Ron Matricaria Institute of Molecular Medicine, Phoenix Children’s Hospital; Editor-in-Chief, Pediatric Blood and Cancer; Professor, Department of Child Health, University of Arizona College of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Acknowledgements

Gary R Jones, MD Associate Medical Director, Clinical Development, Berlex Laboratories

Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Sara E Tisdale, MD Resident Physician, Creighton University Joint Pediatric Residency Program, Department of Pediatrics, University of Nebraska Medical Center

Disclosure: Nothing to disclose.

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