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Licensed Unlicensed Requires Authentication Published by De Gruyter August 31, 2019

Acquired von Willebrand’s syndrome caused by primary hypothyroidism in a 5-year-old girl

  • Claire Flot , Isabelle Oliver , Philippe Caron , Frédérique Savagner , Maithé Tauber , Ségolène Claeyssens and Thomas Edouard EMAIL logo

Abstract

Background

Acquired von Willebrand’s syndrome (aVWS) associated with hypothyroidism is rare in children and more often diagnosed during the peripubertal period in the context of Hashimoto’s thyroiditis.

Case presentation

A 5-year-old girl was referred to the paediatric haematology unit for rectal bleeding, anaemia and prolonged activated partial thromboplastin time (aPTT). Her developmental and learning skills were normal. The physical examination revealed severe short stature (height SDS: −3.6) with overweight (body mass index SDS: 1.8) and clinical sign of hypothyroidism. Laboratory investigation revealed aVWS type 1 associated with severe primary hypothyroidism. Anti-thyroid antibodies were negative and thyroid ultrasound found thyroid hypoplasia in favour of congenital hypothyroidism. Restoration of euthyroidism was associated with increased growth velocity and normalisation of coagulation parameters.

Conclusion

This report highlights the importance of excluding an underlying pathology (including hypothyroidism) in children with suspected VWS, even in young age.


Corresponding author: Thomas Edouard, MD, PhD, Paediatric Endocrine Unit, Children’s Hospital, Purpan University Hospital, 330 avenue de Grande-Bretagne TSA 70034, 31059 Toulouse Cedex 9, France, Phone: +33 (0) 5 34 55 85 55, Fax: +33 (0) 5 34 55 85 58

  1. Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

  2. Research funding: None declared.

  3. Employment or leadership: None declared.

  4. Honorarium: None declared.

  5. Competing interests: The funding organisation(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.

  6. Conflict of interest: None declared.

References

1. Federici AB, Budde U, Castaman G, Rand JH, Tiede A. Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update. Semin Thromb Hemost 2013;39:191–201.10.1055/s-0033-1334867Search in Google Scholar PubMed

2. Mital A. Acquired von Willebrand syndrome. Adv Clin Exp Med 2016;25:1337–44.10.17219/acem/64942Search in Google Scholar PubMed

3. Federici AB. Acquired von Willebrand syndrome associated with hypothyroidism: a mild bleeding disorder to be further investigated. Semin Thromb Hemost 2011;37:35–40.10.1055/s-0030-1270069Search in Google Scholar PubMed

4. Manfredi E, van Zaane B, Gerdes VE, Brandjes DP, Squizzato A. Hypothyroidism and acquired von Willebrand’s syndrome: a systematic review. Haemophilia 2008;14:423–33.10.1111/j.1365-2516.2007.01642.xSearch in Google Scholar PubMed

5. Federici AB, Rand JH, Bucciarelli P, Budde U, van Genderen PJ, et al. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost 2000;84:345–9.10.1055/s-0037-1614018Search in Google Scholar PubMed

6. Franchini M, Veneri D, Lippi G. Analysis of thyroid hormone status in 131 consecutive individuals with low von Willebrand factor levels. Thromb Haemost 2005;93:392–3.10.1055/s-0037-1616250Search in Google Scholar PubMed

7. Stuijver DJ, Piantanida E, van Zaane B, Galli L, Romualdi E, et al. Acquired von Willebrand syndrome in patients with overt hypothyroidism: a prospective cohort study. Haemophilia 2014;20:326–32.10.1111/hae.12275Search in Google Scholar PubMed

8. Blesing NE, Hambley H, McDonald GA. Acquired von Willebrand’s disease and hypothyroidism: report of a case presenting with menorrhagia. Postgrad Med J 1990;66:474–6.10.1136/pgmj.66.776.474Search in Google Scholar PubMed

9. Bruggers CS, McElligott K, Rallison ML. Acquired von Willebrand disease in twins with autoimmune hypothyroidism: response to desmopressin and L-thyroxine therapy. J Pediatr 1994;125(6 Pt 1):911–3.10.1016/S0022-3476(05)82007-4Search in Google Scholar PubMed

10. Coccia MR, Barnes HV. Hypothyroidism and acquired von Willebrand disease. J Adolesc Health 1991;12:152–4.10.1016/0197-0070(91)90459-YSearch in Google Scholar PubMed

11. Concha R, Borzone MA, Castillo M, Rossle A, Quevedo I. [Acquired von Willebrand disease as an unusual manifestation of primary hypothyroidism: report of two cases]. Rev Med Chil 2005;133:813–6.10.4067/S0034-98872005000700009Search in Google Scholar PubMed

12. Dalton RG, Dewar MS, Savidge GF, Kernoff PB, Matthews KB, et al. Hypothyroidism as a cause of acquired von Willebrand’s disease. Lancet 1987;1:1007–9.10.1016/S0140-6736(87)92272-0Search in Google Scholar PubMed

13. Galli-Tsinopoulou A, Stylianou C, Papaioannou G, Nousia- Arvanitakis S. Acquired von Willebrand’s syndrome resulting from untreated hypothyroidism in two prepubertal girls. Haemophilia 2006;12:687–9.10.1111/j.1365-2516.2006.01355.xSearch in Google Scholar PubMed

14. Setian N, Tanaka CM, Damiani D, Dichtchekenian V, Carneiro JD, et al. Hypopituitarism, deficiency of factors V and VIII and von Willebrand factor: an uncommon association. J Pediatr Endocrinol Metab 2002;15:331–3.10.1515/JPEM.2002.15.3.331Search in Google Scholar PubMed

15. Smith SR, Auger MJ. Hypothyroidism and von Willebrand’s disease. Lancet 1987;1:1314–5.10.1016/S0140-6736(87)90565-4Search in Google Scholar PubMed

16. Viswanath AK, Mayo A, King D, Bevan JS, editors. Willebrand’s disease secondary to autoimmune hypothyroidism. Brighton, UK: 23rd Joint Meeting of the British Endocrine Societies; 2004:22–4.Search in Google Scholar

17. Olukman O, Sahin U, Kavakli T, Kavakli K. Investigation of acquired von Willebrand Syndrome in children with hypothyroidism: reversal after treatment with thyroxine. J Pediatr Endocrinol Metab 2010;23:967–74.10.1515/jpem.2010.155Search in Google Scholar PubMed

18. Szinnai G. Genetics of normal and abnormal thyroid development in humans. Best Pract Res Clin Endocrinol Metab 2014;28:133–50.10.1016/j.beem.2013.08.005Search in Google Scholar PubMed

19. Callaghan MU, Wong TE, Federici AB. Treatment of acquired von Willebrand syndrome in childhood. Blood 2013;122:2019–22.10.1182/blood-2012-10-435719Search in Google Scholar PubMed

20. Marongiu F, Barcellona D, Mameli A, Mariotti S. Thyroid disorders and hypocoagulability. Semin Thromb Hemost 2011;37:11–6.10.1055/s-0030-1270066Search in Google Scholar PubMed


Supplementary Material

The online version of this article offers supplementary material (https://doi.org/10.1515/jpem-2019-0082).


Received: 2019-02-13
Accepted: 2019-07-25
Published Online: 2019-08-31
Published in Print: 2019-11-26

©2019 Walter de Gruyter GmbH, Berlin/Boston

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