Hypoprothrombinemia Follow-up

  • Author: J Nathan Hagstrom, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Feb 29, 2012
 

Further Outpatient Care

Patients with inherited prothrombin deficiency should receive follow up with a hematologist at a comprehensive center that cares for patients with bleeding disorders. Follow-up should occur on a yearly basis as a minimum.

A hematologist should initially evaluate patients with acquired hypoprothrombinemia. Follow-up care depends on the underlying cause.

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Prognosis

Patients with lupus anticoagulant-hypoprothrombinemia syndrome after a viral infection can be expected to spontaneously recover.

Few patients with systemic lupus erythematosus–associated lupus anticoagulant-hypoprothrombinemia syndrome (most cases) have spontaneously recovered. Immunosuppressive therapy successfully controls bleeding and increases prothrombin levels in most patients, though some have had a recurrence of symptoms when drug therapy was tapered.

The prognosis for patients with inherited prothrombin deficiency varies. The degree of deficiency does not always predict the clinical course, as patients with severe deficiency with only mild bleeding tendencies have been reported. Impairment of the procoagulant and anticoagulant activities of prothrombin are speculated to result in a delicate coagulation balance in patients who have mild or no symptoms.

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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, and 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; Assistant 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 Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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 and American Society of Hematology

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthor Sara E Tisdale, MD, to the development and writing of this article.

References
  1. Meeks SL, Abshire TC. Abnormalities of prothrombin: a review of the pathophysiology, diagnosis, and treatment. Haemophilia. Nov 2008;14(6):1159-63. [Medline].

  2. Xue J, Wu Q, Westfield LA, et al. Incomplete embryonic lethality and fatal neonatal hemorrhage caused by prothrombin deficiency in mice. Proc Natl Acad Sci U S A. Jun 23 1998;95(13):7603-7. [Medline]. [Full Text].

  3. Acharya SS, Coughlin A, Dimichele DM, et al. Rare Bleeding Disorder Registry: deficiencies of factors II, V, VII, X, XIII, fibrinogen and dysfibrinogenemias. J Thromb Haemost. Feb 2004;2(2):248-56. [Medline].

  4. Pasmant E, Dumont B, Lacapere JJ, Dautzenberg MD, Bezeaud A. A severe neonatal presentation of factor II deficiency. Eur J Haematol. Nov 2011;87(5):464-6. [Medline].

  5. Appert-Flory A, Fischer F, Amiral J, Monpoux F. Lupus Anticoagulant-Hypoprothrombinemia syndrome (HLAS): report of one case in a familial infectious context. Thromb Res. Aug 2010;126(2):e139-40. [Medline].

  6. Akhavan S, Luciani M, Lavoretano S, Mannucci PM. Phenotypic and genetic analysis of a compound heterozygote for dys- and hypoprothrombinaemia. Br J Haematol. Jan 2003;120(1):142-4. [Medline].

  7. Baca V, Montiel G, Meillon L, et al. Diagnosis of lupus anticoagulant in the lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature. Am J Hematol. Nov 2002;71(3):200-7. [Medline].

  8. Bhat RV, Deshmukh CT. A study of Vitamin K status in children on prolonged antibiotic therapy. Indian Pediatr. Jan 2003;40(1):36-40. [Medline].

  9. Bolton-Maggs PH, Perry DJ, Chalmers EA, et al. The rare coagulation disorders--review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation. Haemophilia. Sep 2004;10(5):593-628. [Medline].

  10. Eberhard A, Sparling C, Sudbury S, et al. Hypoprothrombinemia in childhood systemic lupus erythematosus. Semin Arthritis Rheum. Aug 1994;24(1):12-8. [Medline].

  11. Erkan D, Bateman H, Lockshin MD. Lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus: report of 2 cases and review of literature. Lupus. 1999;8(7):560-4. [Medline].

  12. Galli M, Barbui T. Antiprothrombin antibodies: detection and clinical significance in the antiphospholipid syndrome. Blood. Apr 1 1999;93(7):2149-57. [Medline]. [Full Text].

  13. Girolami A, Scarano L, Saggiorato G, et al. Congenital deficiencies and abnormalities of prothrombin. Blood Coagul Fibrinolysis. Oct 1998;9(7):557-69. [Medline].

  14. Humphries JE, Acker MN, Pinkston JE, Ruddy S. Transient lupus anticoagulant associated with prothrombin deficiency: unusual cause of bleeding in a 5-year-old girl. Am J Pediatr Hematol Oncol. Nov 1994;16(4):372-6. [Medline].

  15. Lechler E. Use of prothrombin complex concentrates for prophylaxis and treatment of bleeding episodes in patients with hereditary deficiency of prothrombin, factor VII, factor X, protein C protein S, or protein Z. Thromb Res. Aug 15 1999;95(4 Suppl 1):S39-50. [Medline].

  16. Lipsky JJ. Antibiotic-associated hypoprothrombinaemia. J Antimicrob Chemother. Mar 1988;21(3):281-300. [Medline].

  17. Rouy S, Vidaud D, Alessandri JL, et al. Prothrombin Saint-Denis: a natural variant with a point mutation resulting in Asp to Glu substitution at position 552 in prothrombin. Br J Haematol. Mar 2006;132(6):770-3. [Medline].

  18. Schmugge M, Tolle S, Marbet GA, et al. Gingival bleeding, epistaxis and haematoma three days after gastroenteritis: the haemorrhagic lupus anticoagulant syndrome. Eur J Pediatr. Jan 2001;160(1):43-6. [Medline].

  19. Stanchev H, Philips M, Villoutreix BO, et al. Prothrombin deficiency caused by compound heterozygosity for two novel mutations in the prothrombin gene associated with a bleeding tendency. Thromb Haemost. Jan 2006;95(1):195-8. [Medline].

  20. Strijks E, Poort SR, Renier WO, et al. Hereditary prothrombin deficiency presenting as intracranial haematoma in infancy. Neuropediatrics. Dec 1999;30(6):320-4. [Medline].

  21. Vivaldi P, Rossetti G, Galli M, Finazzi G. Severe bleeding due to acquired hypoprothrombinemia-lupus anticoagulant syndrome. Case report and review of literature. Haematologica. May-Jun 1997;82(3):345-7. [Medline].

  22. Yacobovich JR, Uziel Y, Friedman Z, et al. Diffuse muscular haemorrhage as presenting sign of juvenile systemic lupus erythematosus and lupus anticoagulant hypoprothrombinaemia syndrome. Rheumatology (Oxford). May 2001;40(5):585-7. [Medline]. [Full Text].

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